pulse issue 13 dec 2015 - srl diagnostics december 2015 issue 13 editorial team president-research...

24
Pulse December 2015 Issue 13 A Technical Newsletter of SRL Pulse

Upload: truongdat

Post on 15-Mar-2018

221 views

Category:

Documents


2 download

TRANSCRIPT

Page 1: Pulse Issue 13 Dec 2015 - SRL Diagnostics December 2015 Issue 13 Editorial Team President-Research & Innovation SRL, Mumbai Director, Department of Medical Oncology Jaslok Hospital

Pulse December 2015 Issue 13

A Technical Newsletter of SRL

Pulse

Page 2: Pulse Issue 13 Dec 2015 - SRL Diagnostics December 2015 Issue 13 Editorial Team President-Research & Innovation SRL, Mumbai Director, Department of Medical Oncology Jaslok Hospital

Pulse December 2015 Issue 13

Editorial Team

President-Research & InnovationSRL, Mumbai

Director, Department of Medical OncologyJaslok Hospital & Research Centre and Raheja Hospital, Mumbai

President-InternationalSRL, Mumbai

Senior GastroenterologistApollo Hospitals, Chennai

HOD & Senior Consultant-Internal MedicineDirector Academics & Research, Fortis Hospital, Jaipur

Senior Gynecologist & IVF Specialist, Mumbai

Founder-Director, Centre for Genetic Health CareCenter of Excellence in Cytogenetics, SRL Diagnostics, Mumbai

HOD, Rehabilitation Medicine and Sports MedicineSir H. N. Reliance Foundation Hospital and Research Centre, Mumbai

Senior Research Scientist, SRL, Mumbai

Director, Clinical Reference Laboratory, SRL, Gurgaon

Chief Pathologist, SRL, Chennai

Senior Histopathologist, SRL, Mumbai

Head-Lab Medicine, Fortis Hospital, Mohali

Consultant Radiologist, SRL Diagnostics L H Hiranandani Hospital, Mumbai

Editor

Advisory Board

Associate Editors

Dr. B. R. Das

Dr. S. H. Advani,

Dr. Amar Das Gupta

Dr. A. T. Mohan

Dr. Rajeev Gupta

Dr. Sanjay Patil

Dr. Hema Purandarey

Dr. Aashish Contractor

Dr. Arnab Roy

Dr. Rajiv Tangri

Dr. Premila Samuel

Dr. K. D. Patole

Dr. Anita Sharma

Dr. Natasha Nanda

Padmashri

Page 3: Pulse Issue 13 Dec 2015 - SRL Diagnostics December 2015 Issue 13 Editorial Team President-Research & Innovation SRL, Mumbai Director, Department of Medical Oncology Jaslok Hospital

Pulse December 2015 Issue 13

In This Issue

PageNo.

In Focus

Case Report

Brain Teasers

Some of Our Recent Activities

Rare Tumor of Uterus - Angiolipoleiomyoma and Its Comparision with RenalAngiomyolipoma 1

1. Ovarian Hemangioma and Cervical Stromal Melanosis - Incidental Detections in a 2Routine Hysterectomy

2. Extremely Rare Case of Advanced Epithelioid Malignant Peripheral Nerve Sheath 4Tumour (Empnst) Presenting as Right Upper Arm Growth, Anterior Chest Wall Massand Thyroid Nodule with Lung Metastasis in a 65 Year Old Female AutoimmuneHepatitis

3. A Case of Stiff Person Syndrome 7

4. Extranodal Anaplastic Large Cell Lymphoma, ALK Positive 9

5. Solid Pseudopapillary Neoplasm of Pancreas in a Male Child: A Rare Presentation 10

6. An Interesting Cause of Brain Abscess 12

7. Syringomatous Adenoma of Nipple 14

8. Myroides Species: A Rare Cause of Endocarditis 15

9. Liposarcoma of Tongue 17

18

• Recent Test Releases 19

• Recent Publications from SRL 19

• Continuing Medical Education on SRL Global Knowledge Forum 20

Page 4: Pulse Issue 13 Dec 2015 - SRL Diagnostics December 2015 Issue 13 Editorial Team President-Research & Innovation SRL, Mumbai Director, Department of Medical Oncology Jaslok Hospital

Message from the Editor

At the stroke of New Year, with great joy and pride, we bring forthto you the thirteenth issue of Pulse.

For this issue of Pulse, the editorial team received anunprecedented number of clinical case reports and had a toughtime reviewing and selecting the ones for publication. This showsthe vibrancy and talent of our scientific staff and the fact that Pulsecontinues to be a platform for us to report novel and rare caseswe encounter in our professional settings.

This issue’s In Focus article is a rare and unique case of extrarenalangiolipoleiomyoma of the uterus. Cases range from varioussections, including rare cases of Myroides species causing

endocarditis and fungal brain abscess from Microbiology; ALK positive extranodalanaplastic large cell lymphoma from Hematology; stiff person syndrome fromBiochemistry; and numerous reports from Histopathology like the extremely rare case ofadvanced epithelioid malignant peripheral nerve sheath tumor, solid pseudopapillaryneoplasm of pancreas, syringomatous adenoma of nipple, liposarcoma of tongue, andthe incidental finding of ovarian hemangioma and cervical stromal melanosis.

Along with case reports, Pulse also encompasses some brain-twisters, R&D activities andContinued Medical Education programs.

We would like to thank all the contributing authors for providing such a rich diversity ofmedical case reports on a diverse range of topics. At the same time, the editorial boardwelcomes case reports, medical quizzes and puzzles, citations of research publication,CME details and pictures to be included in the next issue.

Hope you will find these reports interesting and useful in your professional practice. Welook forward to your invaluable suggestions and feedback.

Happy Reading…Dr. B. R. Das

Pulse December 2015 Issue 13

Page 5: Pulse Issue 13 Dec 2015 - SRL Diagnostics December 2015 Issue 13 Editorial Team President-Research & Innovation SRL, Mumbai Director, Department of Medical Oncology Jaslok Hospital

[1]

Summary

Background

Case Presentation

Investigations and Treatment

Extrarenal angiomyolipomas (AML) have been reportedat various anatomical sites, but are extremely rare inuterus. We describe a case of 62-year-old woman whopresented wi th low abdominal pain andmenomet ro r rhag ia fo r las t 2-3 months .Ultrasonography suggested a fibroid measuring upto 3cm. Patient underwent total hysterectomy with bilaterala d n e x e c t o m y. H i s t o p a t h o l o g i c a l a n dimmunohistochemical examination showed features ofrare benign uterine tumor- angiolipoleiomyoma.

Angiolipoleiomyoma (ALLM) is a benign mesenchymalneoplasm that present with a variable admixture ofadipose tissue, smooth muscle with a well expressedvascular component. They are typically found in thekidneys and its occurrence in a uterus is extremely rare.The reported incidence of angiolipoleiomyomas is0.06% of all benign uterine tumors (2). Case ispresented due to its rarity. On review of literature only17 cases have been reported till date.

• 62-year-old patient presented with low abdominalpain, menometrorrhagia for last 2-3 months.

• There was no significant medical, family andobstetric history.

• Biochemical and haematological examination waswithin normal limit.

• The ultrasonography revealed a fibroid measuringupto 3 cm.

• The patient underwent total abdominalhysterectomy with bilateral adnexectomy.

• Gross examination showed 3x2 cm, wellcircumscribed soft, yellowish, submucosal nodulein the fundus.

• Histopathological examination showed a benigntumor angiolipoleiomyoma composed ofadmixture of three tissue components: smoothmuscle fibers, mature adipose tissue and multiple

thick-walled vessels (fig.1). No mitoses or necrosiswere seen.

• On IHC, Desmin was strongly positive in spindlecells (fig.2) whereas these cells were nonimmunoreactivity to HMB-45 (fig.3).

Differentials considered were:1. Lipoleiomyoma biphasic tumor comprising of

spindle cells, adipose tissue component and lackprominent vascular component.

2. Uerine PEComa show spindle to epithelioid cellwith predilection for perivacular arrangement andare immunoreactive for HMB-45, desmin andactin.

In our case tumor was triphasic with spindle cellsimmunoreactive to desmin and negative for HMB-45.

ALLM was first report by Sieinski in 1989 (1).Intrauterine ALLMs are extremely rare. A review of theliterature reveals 17 cases of ALLMs have been reportedtill date (1). The clinical presentation of uterine ALLMs isvariable, often similar to that of typical leiomyomas –menometrorrhagia, presence of pelvic mass,abdominal pain or even lack ofsymptoms. Age of presentation ranges from 20-62years. Most ALLMs are located in the corpus uteri,

Differential Diagnosis

Discussion

Pulse December 2015 Issue 13

In Focus

Rare Tumor of Uterus - Angiolipoleiomyoma and Its Comparision withRenal Angiomyolipoma

Mallika Dixit*, Seema Rana, Rajiv TangriSRL Clinical Reference Laboratory, Gurgaon, Haryana*Corresponding author ([email protected])

Fig.1 H&E (40X) Fig.2 Desmin (40X)

Fig.3 HMB-45 (40X)

Page 6: Pulse Issue 13 Dec 2015 - SRL Diagnostics December 2015 Issue 13 Editorial Team President-Research & Innovation SRL, Mumbai Director, Department of Medical Oncology Jaslok Hospital

[2]

Pulse December 2015 Issue 13

followed by the cervix and the lower uterine segment assubserosal or intramural growths. The tumor sizeranges from 2 to 16 cm with a median of 8.4 cm.Usually, the tumors are well defined with apseudocapsule and are either soft or firm, as aconsequence of the amount of smooth muscle, adiposetissue, and vascular components. Cut surface of tumorsshow a gray, pink/tan, and variegated appearance.Necrosis and hemorrhage are very rare features similarto those of renal AML. In our case age was 62 years,tumor was present in uterine corpus and size of tumorwas 3 cms.

Currently, there are no criteria about the percentages ofthe 3 components for the diagnosis of ALLM. Thesmooth muscle cells are present in both thin and thickfascicles coursing through adipose and connectivetissue. In one case reported so far, the smooth musclecells showed a focus of atypical smooth muscle cellproliferation with many pleomorphic giant cells with nomitoses or necrosis therefore, the authors called this asALLM with focus of atypical or symplastic leiomyoma(2). Only one out of 17 cases reported showedimmunoreactivity to HMB-45 (5). Renal AML is also atriphasic tumor and is associated with tuberous sclerosis- a hereditary autosomal dominant complex,presenting with multiple hamartomas in various internalorgan (4). On IHC epithelioid cells of renal AML areusually arranged around blood vessels showingimmunoreactive to HMB-45 and smooth muscle cellsshow scattered, weak desmin immunoreactivity (4). Incontrast to renal AML the uterine ALLMs is notassociated with tuberous sclerosis and is HMB-45

negative. Smooth muscle cells of uterine ALLMs showstrong cytoplasmic positivity for smooth muscle actinand desmin.

• Though benign and rare tumor should beconsidered in the differential diagnosis ofleiomyoma and uterine PEComa.

• Combination of actin, desmin and HMB-45 isuseful in differentiating the above entities.

1. Tihomir P. Totev, Svetlana A. Matev et al. Uterineangiomyolipoma: a case report, differentialdiagnosis with PEComa and review of literature, JBiomed Clin Res, 2014, Volume 7 Number 1

2. Ren R, Wu HH. Pathologic quiz case a 40-year-oldArch Pathol Lab Med.; February 2004 128:e31-32

3. Kajo K, Žúbor P, Krivuš Š. Danko J.Angiolipoleiomyoma of the uterus. Case reportand literature review. Ceska Gynekol. 2010;75(1):54-6.

4. Huang PC, Chen JT, Ho WL. Clinicopathologicanalysis of renal and extrarenal angiomyolipomas:report of 44 cases. Chinese Med J. 2000;63(1):3744.

5. Cil AP, Haberal A, Hucumenoglu S, Kovalak EE,Gunes M. Angiomyolipoma of the uterusassociated with tuberous sclerosis: case report andreview of the literature. Gynecol Oncol 2004;94(2):593-6

Learning Points/ Take Home Messages

References

Case Reports

Ovarian Hemangioma and Cervical Stromal Melanosis - IncidentalDetections in a Routine Hysterectomy

Irneet Mundi, Ritu Pankaj, A. K. BanerjeeSRL Fortis Hospital, Mohali, Chandigarh

Summary

We report two interesting incidental findings in ahysterectomy performed on a 59 year old. Totalabdominal hysterectomy with bilateral salpingo-opherectomy (TAH-BSO) was performed for fibroiduterus. One of the ovaries showed an ovarianhemangioma which is a rare tumor at this site. Thecervix showed foci of stromal melanosis.

Background

Incidental findings in histopathology specimens arealways a treat to the pathologist’s eyes. Incidentaldetection of ovarian hemangioma and foci of cervicalstromal melanosis in a routine hysterectomy specimenmakes the present case interesting. Sometimes thesemay pose diagnostic challenge to the pathologists.Hence, it is important to be aware of these entities.

Page 7: Pulse Issue 13 Dec 2015 - SRL Diagnostics December 2015 Issue 13 Editorial Team President-Research & Innovation SRL, Mumbai Director, Department of Medical Oncology Jaslok Hospital

[3]

Pulse December 2015 Issue 13

Case Presentation

Investigations

Differential Diagnosis

• A 59 year old female underwent TAH-BSO forsymptomatic uterine fibroid.

• The right ovary on gross examination measured2.5x1x0.3 cm and showed a haemorrhagic areameasuring 1x0.5x0.3 cm. The other ovary wasunremarkable.

• An intramural fibroid was detected.

• On microscopic examination the right ovarianstroma showed a relatively circumscribed area withmany dilated and congested blood vessels lined byflattened endothelial cells. No atypical featureswere noted and it was diagnosed as cavernoushemangioma (Figure 1).

• Another interesting finding detected incidentallywas the presence of pigmented cells in thesubepithelial stroma of the endocervix (Figure 2).The pigment was positive with Masson Fontana andnegative with Perl’s stain. The features were of focalstromal melanosis of the uterine cervix.

• The intramural leiomyoma was benign in nature.Section from endometrium showed atrophicchanges.

The routine blood investigations were unremarkable.

• The main difficulty is distinguishing a smallhemangioma from proliferation of dilated hilarvessels. In order to be regarded as a truehemangioma, a mass of vascular channels,ranging from small to large size with minimalamount of stroma should form a reasonablycircumscribed lesion distinct from remainder of theovary. Medullary blood vessels may appearparticularly numerous and closely packed inpostmenopausal women and should not bemistaken for a hemangioma. One of the maincharacteristics of these vessels is that they may becalcified or have thickened wall with narrowedlumen due to medial deposition of hyaline, amyloidlike material.

• Presence of red blood cells within the vascularlumen and absence of pale eosinophilic

homogenous material helps distinguishingcavernous hemangioma from lymphangioma (1).

• Another differential diagnosis is ovarian teratomawith a large hemangiomatous component. Thesecan be distinguished from hemangioma byextensive and careful sampling to detect otherteratomatous components.

• Angiosarcomas usually show marked cytologicalatypia, pleomorphism, papillary endothelialtufting, increased mitosis and necrosis (2).

• Stromal melanosis should be distinguished fromother pigmented lesions of the cervix. Melanosis ofcervix reveals pigmented melanocytes in the basallayer of epithelium but do not involve the stroma(3).

• Stromal melanosis should be distinguished frommelanoma in which there is stromal infiltration bymalignant cells. Junctional change is usuallypresent. This may be especially problematic inscanty endocervical curettings, cervical biopsy orcervical cone biopsy (4).

Both the lesions were detected incidentally and did notrequire any further treatment.

The hospital stay of the patient was uneventful and sherecovered well after hysterectomy.

Incidental findings always interest the pathologists. Anumber of lesions are picked up incidentally inhysterectomy specimens varying from benign lesionslike adenomyosis and granulomas to premalignant andmalignant conditions.

Ovarian hemangiomas are usually small andasymptomatic, being diagnosed usually incidentally.Vascular tumors of the female genital tract, especiallythose arising in the ovary are very rare. Some of themcan attain large size and present clinically with pain dueto torsion or abdominal distention due to the mass itself.Few cases have been reported to be associated withmassive ascitis and elevated CA-125 mimickingovarian carcinoma (5).

Although the etiology of the lesion remains unknown,they have been considered either hamartomatousmalformations or true neoplasms in which pregnancy,other hormonal effects or infection have beenimplicated as growth enhancing factors (1).

The uterine cervix is normally devoid of melanocytesand hence melanin containing lesions are rare in thissite. Foci of stromal melanocytes (FSM) have been

Treatment

Outcome and Followup

Discussion

Figure 1: Multiple large dilatedvessels of ovarian hemangioma

Figure 2: Pigmented melanocytesin the endocervical stroma

Page 8: Pulse Issue 13 Dec 2015 - SRL Diagnostics December 2015 Issue 13 Editorial Team President-Research & Innovation SRL, Mumbai Director, Department of Medical Oncology Jaslok Hospital

[4]

Pulse December 2015 Issue 13

known as extra-cutaneous blue nevus. Howevermacroscopic and histological findings suggest that FSMof cervix are analogous to dermal melanocytosis ratherthan to cutaneous blue nevus and lesions are moreappropriately called stromal melanocytosis (6).

These lesions are rare and usually occur as anincidental finding in middle aged women. The mostwidely accepted theory is that they originate frommelanoblasts that aberrantly migrate from the neuralcrest to the cervix during embryogenesis (4).

Incidentally detected ovarian hemangioma and foci ofcervical stromal melanosis are rare entities and maypose a diagnostic challenge. Hence, it is important tobe aware of these conditions to avoid misinterpretation.

1. Mitra B, Sengupta S etal. Ovarian haemangioma:A rare case report. International Journal of Surgerycase reports 2013 (4): 981-84.

Learning Points/ Take Home Messages

References:

2. Balot F, Erkanli S etal. Ovarian Hemangioma:Report of Two Cases and Review of the Literature.Turkish Journal of Pathology 2010; 26 (3):264-66.

3. S Balamurugan, K Rameshrao etal. Blue Nevus ofcervix – A Case Report. The Internet Journal ofGynecology and Obstetrics 2012; 16 (1).

4. Parada D, Karla B Peña etal. Coexisting MalignantMelanoma and Blue Nevus of the Uterine Cervix:An Unusual Combination. Case Reports inPathology 2012 Article ID 986542

5. Uppal S, Heller DS etal. Ovarian hemangioma-Report of three cases and review of literature. ArchGynecol Obstet 2004; 270: 1-5

6. Uehara T, Takayama S, Takemura T, Kasuga T. Fociof stromal melanocytes (so-called blue naevus) ofthe uterine cervix in Japanese women. VirchowsArchiv A Pathol Anat 1991; 418: 327-31.

Corresponding Author: Dr. Irneet MundiSRL Fortis Hospital, Mohali, ChandigarhE-mail: [email protected]

Extremely Rare Case of Advanced Epithelioid Malignant Peripheral NerveSheath Tumour (Empnst) Presenting as Right Upper Arm Growth, AnteriorChest Wall Mass and Thyroid Nodule with Lung Metastasis in a 65 YearOld Female

Kanwaljeet Kaur Miglani, Punitha Shorey, Avneet Boparai, Shaveta VediFortis Escorts Hospital, Amritsar, Punjab

Summary

Malignant peripheral nerve sheath tumour (MPNST) is arare malignant soft tissue tumour. It was also knownover the years as malignant schwannoma, neurogenicsarcoma and neurofibrosarcoma. About half of thesetumours arise de novo and other half from nervesinvolved by neurofibroma as a part of type 1Recklinghausen disease. They represent approx. 10%of all soft tissue malignant tumours.

Epithelioid variants of MPNST are even rarer and areestimated to comprise 5% or fewer of MPNSTs. Theyhave poorer prognosis and rarely involve the head andneck. They usually have predilection for lowerextremities.

We present a case of 65 year old female who presentedwith a right upper arm papilloma like growth, anteriorchest wall mass and thyroid nodular swelling for last 6

months. CT Scan of thorax showed a large soft tissuemass in the postero-superior mediatinum measuring3.2x4.6x3 cms, causing compression of trachea andoesophagus. The mass appeared to extend into thetrachea. Fat planes between it and right lobe of thyroidwere lost, suggestive of infiltration. The mass was alsoindenting left lobe of thyroid and superior mediastinalvessels. Multiple small centrilobular nodules were seenin right middle and both lower lobes of lung, largestmeasuring approximately 1.2x0.9 cms.

A soft tissue mass with necrosis was seen in the rightanterior chest wall, at the level of lower end of sternum,measuring approximately 4.7x3.7 cms. Fine NeedleAspiration Cytology (FNAC) of all three sites includingright upper arm growth, anterior chest wall mass andthyroid nodule showed hypercellular smears composedof similar looking atypical cells arranged as singlydispersed, in papillary clusters and few groupssuggestive of malignant neoplasm.

Page 9: Pulse Issue 13 Dec 2015 - SRL Diagnostics December 2015 Issue 13 Editorial Team President-Research & Innovation SRL, Mumbai Director, Department of Medical Oncology Jaslok Hospital

[5]

Pulse December 2015 Issue 13

Biopsy from right upper arm growth showed a definedlesion composed of poorly differentiated cells in theform of sheets and nests of polygonal cells withepithelioid morphology having prominent nucleoli andeosinophilic cytoplasm. On IHC, tumour cells werepositive for Vimentin, S-100, patchy weak for CK,negative for LCA, Desmin and HMB-45. Tumour cellswere patchy weak for EMA, patchy membranouspositive for CD99 and negative for CD68.Morphological and Immmunohistochemical featuresare in favour of Epithelioid Malignant Peripheral NerveSheath tumour (EMPNST).

• Epithelioid malignant peripheral nerve sheathtumour (EMPNST) is rare and differs fromconventional malignant peripheral nerve sheathtumour by showing diffuse S-100 protein positivity,infrequent association with NF1, and occasionalorigin in a schwannoma.

• Loss of INI1 expression is seen in a subset of tumour

A 65 year old female presented with a right upper armpapilloma like growth, anterior chest wall mass andthyroid nodular swelling for last 6 months. There are nosymptoms of dysphagia, loss of appetite or fever.

Fine Needle Aspiration Cytology (FNAC) of all threesites including right upper arm growth, anterior chestwall mass and thyroid nodule showed hypercellularsmears composed of similar looking atypical cellsarranged as singly dispersed, in papillary clusters andfew groups. The atypical cells showed moderatecytoplasm with central to eccentric nuclei, prominentnucleoli and moderate cytoplasm. Few mitotic figuresand foci of necrosis were also seen. Features weresuggestive of malignant neoplasm.

Histopathological and Immunohistochemical report -Biopsy from right upper arm growth showed a definedlesion composed of poorly differentiated cells in theform of sheets and nests of polygonal cells withepithelioid morphology having prominent nucleoli andeosinophilic cytoplasm.

On IHC, tumour cells were positive for Vimentin, S-100, patchy weak for CK, negative for LCA, Desminand HMB-45. Tumour cells were patchy weak for EMA,patchy membranous positive for CD99 and negativefor CD68.

Morphological and Immmunohistochemical featuresare in favour of Epithelioid Malignant Peripheral NerveSheath tumour (EMPNST).

Background

Case Presentation

Lab Investigations

Differential Diagnosis

Discussion

EMPNST may be confused with many epithelioidneoplasm’s including metastatic melanomas,metastatic carcinomas, large cell lymphoma,myoepithelial carcinomas, epithelioid schwannomas,and other soft tissue sarcomas with epithelioid features(epithelioid sarcoma, epithelioid angiosarcoma, clearcell sarcoma etc) .

EMPNST might be very difficult to differentiate fromamelanotic melanoma, but the presence ofimmunoreactivity for other melanoma markers such astyrosinase, MelanA, and HMB-45 and the presence ofa junctional component are useful in differentiatingmelanoma from epithelioid MPNST.

Malignant peripheral nerve sheath tumour (MPNST) is arare malignant soft tissue tumour. Its variants includerhabdomyoblastic (malignant “Triton tumour”),glandular, melanocytic and epithelioid MPNST [1].Most MPNSTs are generally considered high-gradesarcomas [1]. Approximately 40% of patientsdeveloped local recurrence, and the overall five-yearsurvival rate was 34–43% [6, 7].

On the other hand, epithelioid variants are rare and areestimated to comprise 5% or fewer of MPNSTs [1]. Thus,biologic behaviour and prognosis are unclear.However, the largest series, which included 26 casesand was reported by Laskin et al. [3], showed thatalthough most patients were treated with wide excision,four developed distant metastases and three died of thedisease within three years. High incidence of metastasis(seven of 14 cases) was also reported by Lodding et al.in 1986 [2].

A lobulated grey whitesoft tissue piece measuring

3x2.5x2 cms. Cut sectionis grey white homogenouswith no area of necrosis

or haemorrhage

Poorly differentiated cells in theform of sheets and nests of

polygonal cells with epithelioidmorphology having prominent

nucleoli and eosinophiliccytoplasm (H&E 10X)

Poorly differentiatedcells in the form of sheets

and nests of polygonalcells with epithelioidmorphology having

prominent nucleoli andeosinophilic cytoplasm

(H&E 40X)

IHC showing diffusepositivity for S-100

Page 10: Pulse Issue 13 Dec 2015 - SRL Diagnostics December 2015 Issue 13 Editorial Team President-Research & Innovation SRL, Mumbai Director, Department of Medical Oncology Jaslok Hospital

[6]

Pulse December 2015 Issue 13

The Epithelioid malignant peripheral nerve sheathtumour (EMPNST) is a rare sarcoma originating fromthe supportive non-neuronal components of peripheralnerves [8]. Epithelioid MPNST (EMPNST) is amorphologically distinct variant that most commonlyaffects adults on the lower extremity or trunk, although awide age range and site distribution are seen.

In this variant morphologically, a part or most of thetumour is composed of plump cells with polygonalacidophilic cytoplasm and an epithelioid likeappearance. MPNST EMPNST is rare and differs fromconventional malignant peripheral nerve sheathtumour by showing diffuse S-100 protein positivity,infrequent association with NF1, and occasional originin a schwannoma. Loss of INI1 expression is seen in asubset of tumours. In contrast with MPNST with spindlecell morphologic features, the epithelioid variant oftendisplays more uniform S-100 protein staining [9].

Differential diagnosis of this variant includes malignantmelanoma, metastatic carcinoma and epithelioidsarcoma. Immunohistochemistry helps in diagnosis.MPNST is positive for S-100, Leu 7, and myelin basicprotein. Malignant melanoma is positive for S-100 andHMB 45 [10]. Metastatic carcinoma is positive forcytokeratin and epithelial membrane antigen (EMA).Epithelioid sarcoma shows positivity for cytokeratin,EMA, vimentin, and CD34.

MPNST is a very aggressive tumour which spreads viadirect perineural invasion and the hematogenousroute. The local recurrence rate is about 54% and therate of distant metastases to lung and bone is about65% [11]. Lymph nodal metastasis occurs inconjunction with widespread disease [12]. Prognosis ofhead and neck tumours are poorer compared to thoseof the extremities and the trunk, with five years survivalrates from 15% to 35% [13].

The authors wish to express thanks to Dr. Seema Rana,Dr. Neeraj Prakash and Dr. Jasbir Singh , ConsultantHistopathologists at SRL Reference lab, Gurgaon fortheir help rendered in IHC marker studies required forthis case.

1. W. Weiss and J. R. Goldblum, “Malignant tumors ofthe peripheral nerves,” in Enzinger and Weiss’s SoftTissue Tumors, S. W. Weiss and J. R. Goldblum,Eds., pp. 903–944, Mosby Elsevier, Philadelphia,Pa, USA, 5th edition, 2008.

2. P. Lodding, L. G. Kindblom, and L. Angervall,“Epithelioid malignant schwannoma. A study of 14cases,” Virchows Archiv, vol. 409, no. 4, pp.433–451, 1986.

3. W. B. Laskin, S. W. Weiss, and G. L. Bratthauer,“Epithelioid variant of malignant peripheral nerve

Acknowledgment

References

shea th tumour (mal ignan t ep i the l io idschwannoma),” American Journal of SurgicalPathology, vol. 15, no. 12, pp. 1136–1145, 1991.

4. A. M. Manganoni, C. Farisoglio, A. Lonati, F. Zorzi,G. Tucci, and P. G. C. Pinton, “Cutaneousepithelioid malignant schwannoma: review of theliterature and case report,” Journal of Plastic,Reconstructive and Aesthetic Surgery, vol. 62, no.9, pp. e318–e321, 2009.

5. S. Suster, K. Amazon, L. B. Rosen, and J. M.Ollague, “Malignant epithelioid schwannoma ofthe skin. A low-grade neurotropic malignantm e l a n o m a ? ” A m e r i c a n J o u r n a l o fDermatopathology, vol. 11, no. 4, pp. 338–344,1989.

6. B. S. Ducatman, B. W. Scheithauer, and D. G.Piepgras, “Malignant peripheral nerve sheathtumors. A clinicopathologic study of 120 cases,”Cancer, vol. 57, no. 10, pp. 2006–2021, 1986.

7. J. E. Wanebo, J. M. Malik, S. R. VandenBerg, H. J.Wanebo, N. Driesen, and J. A. Persing, “Malignantp e r i p h e r a l n e r v e s h e a t h t u m o r s : aclinicopathologic study of 28 cases,” Cancer, vol.71, no. 4, pp. 1247–1253, 1993.

8. Matsuda Y, Saoo K, Hosokawa K, Yamakawa K,Yokohira M, Zeng Y, Takeuchi H, Iwai J, Shirai T,Obika K, Imaida K, “Epithelioid malignantperipheral nerve sheath tumour , Report of a casewith inflammatory infiltration,” Pathol Res Pract.2005;201(4):355-60

9. Rosai and Ackerman Surgical Pathology, Volume 2, “Malignant peripheral nerve sheath tumour “ Softtissues, pp. 2137-2139, Mosby Elsevier , St. Louis,Missouri, 10th edition, 2011

10. Rekhi B, Ingle A, Kumar R, DeSouza M, Dikshit R,Jambhekar N. Malignant peripheral nerve sheathtumors: Clinicopathological profile of 63 casesdiagnosed at a tertiary cancer referral centre inMumbai, India. Indian J Pathol Microbiol. 2010;53(4):611–618. [PubMed]

11. Ueda R, Saito R, Horiguchi T, Nakamura Y, IchikizaiK. Malignant peripheral nerve sheath tumour in thean t e r i o r s ku l l ba s e a s soc i a t ed w i t hneurofibromatosis type 1. Neurol Med Chir (Tokyo)2004;44(1):38–42.[PubMed]

12. Gwendolyn JG, Hanan F. Lymph node metastasis ofmalignant peripheral nerve sheath tumour in theabsence of widespread disease five years afterdiagnosis: a rare finding. Int J Clin ExpPathol.2010; 3 (8):812–814. [PMC free article][PubMed]

13. Pfeiffer J, Arapakis L, Boedeker CC, Ridder GJ.Malignant peripheral nerve sheath tumour of theparanasal sinuses and the anterior skull base. JCraniomaxillofac Surg. 2008; 36 (5):293–299.

Corresponding Author: Dr. Kanwaljeet Kaur Miglani,Fortis Escorts Hospital, Amritsar, PunjabE-mail: [email protected]

Page 11: Pulse Issue 13 Dec 2015 - SRL Diagnostics December 2015 Issue 13 Editorial Team President-Research & Innovation SRL, Mumbai Director, Department of Medical Oncology Jaslok Hospital

[7]

Pulse December 2015 Issue 13

A Case of Stiff Person Syndrome

Leena Dash*, Leena Chatterjee#, Simi Bhatia@, Harirama Acharya*,Rajakumar DV~*Fortis Hospital, BG Rd., Bangalore; #SRL, New Delhi; @ SRL Mumbai Reference Lab; ~SRL Gurgaon,Haryana

Summary

Background

Case Presentation

Stiff person syndrome (SPS) is a rare neurologicalcondition with an intriguing pathophysiology and mayresult in severe functional disability. This was a case of a49 year old male who presented with back pain since 3years. He had progressively debilitating symptoms ofleg stiffness and inability to walk without support. Hehad severe spastic paraperesis in all limbs. During stayin the hospital, he further developed severe paraspinalmuscle spasm, severe spasm of abdominal musclesalong with stiffness of lower limbs and gait ataxia.

Various investigations were done systematically toestablish diagnosis and among this most notably thelevels of Anti GAD antibodies which were high, finallyhelped to establish the diagnosis of stiff personsyndrome. Treatment with IV Immunoglobulin and IVBaclofen improved his symptoms and he wasdischarged.

Stiff person syndrome (SPS) is a rare neurologicalcondition with an intriguing pathophysiology and mayresult in severe functional disability. SPS is estimated tooccur in 1 per 1 million individuals.SPS is currentlyviewed as an autoimmune disease and may beassociated with other autoimmune conditions such astype 1 diabetes mellitus, Hashimoto’s thyroiditis,vitil igo, pernicious anemia, systemic lupuserythematosus (SLE) and coeliac disease.SPS was first described in 1956 as a previouslyunknown neurological disorder characterized by‘progressive fluctuating muscular rigidity and spasm’. In1998, Solimena et al were the first to associate SPS withantibodies against the enzyme glutamic aciddecarboxylase (GAD). The elevated levels of GAD helpto clinch the diagnosis of Stiff person syndrome alongwith typical clinical features.

This case was selected as it is very rare. This syndrome isoften mistaken for chronic backache and /or psychiatricillness. Early diagnosis and treatment helps to preventlong term disability. A high level of the biochemicalmarker, Anti GAD antibody, along with relevantdiagnostic findings helps to confirm the diagnosis .

A 49 year old male of African origin, presented with

C/C of back pain since 3 years. Associated with stiffnessof both lower limbs.• Since 1 year he has been unable to walk without

support.• Walking with stiff legs unable to bend at knee joint.

Had visited a few hospitals where he was treated forchronic backache.

• H/O sudden onset of SDH, underwent burr holeevacuation in 2013.

• No H/O pain in neck, weakness of limbs/boweland bladder disturbances /fever/trauma

• Hypertension, Burr hole evacuation of SDH on lefttemporo parietal region.

No pallor/cyanosis/pedaledema• Temp: 98.6 deg F Pulse: 80/min• BP: 120/80 , RR :22/min• CVS: S1 S2+ No murmur• RS: NVBS,B/L air entry equal• PA : soft , no tenderness, no organomegaly,BS+

• Conscious, alert, oriented, higher mental functionsnormal.

• Cranial nerves: normal• Straight leg rising test: bilateral negative• Motor: no motor deficits, spasticity in all four limbs• No cerebellar or meningeal signs• Severe muscle spasm intermittent in abdominal

muscles, likely from spinal myoclonus• Spastic paraparesis with significant lumbar lordosis• Progressive paraparesis with significant gait

abnormality with no sensory signs and symptoms• No bladder/bowel symptoms

Progressively, patient developed severe paraspinalmuscle spasm, severe spasm of abdominal musclesalong with stiffness of lower limbs and gait ataxia. Hewas sensitive to stimuli of sudden sounds.

Spastic Paraparesis/ StiffPerson Syndrome

• MRI Spine• Serum B12,Serum Copper, TFT, Serum Calcium• CSF analysis, Meningoencephalitis panel• CSF routine

Past history

Physical examination:

On neurological examination:

Differential diagnosis:

Investigations

Page 12: Pulse Issue 13 Dec 2015 - SRL Diagnostics December 2015 Issue 13 Editorial Team President-Research & Innovation SRL, Mumbai Director, Department of Medical Oncology Jaslok Hospital

[8]

Pulse December 2015 Issue 13

• Blood for anti GAD Abs• ANA profile blot• Serum IgA• Needle EMG• Nerve conduction study of right upper limb and

lower limbs

• B12 : 858 pg/ml (normal)• Serum copper: 140 micro gm/dl (normal)• CSF: Lymphocytes 100. No organisms, No AFB• Glutamic acid decarboxylase IgG >500 U/ml

Negative: <30 Positive >30o ANA Profile Blot: U1 nRNP detectedo Needle EMG : continuous (low frequency)

motor unit activity (CMUA) simultaneouslyoccurring in agonist and antagonist muscles

• IV Immunoglobulin• Clonazepam• Baclofen

Treatment continued with IV Immunoglobulin, stiffnessimproved symptomatically and spasticity decreased.Further stay in hospital uneventful.

SPS is currently viewed as an autoimmune disease andmay be associated with other autoimmune conditionssuch as type 1 diabetes mellitus, Hashimoto’sthyroiditis, vitiligo, pernicious anemia, systemic lupuserythematosus (SLE) and celiac disease. Epilepsy maybe associated with SPS and is present in 10 - 20% ofcases. More than 60% of patients diagnosed with SPShave at least one psychiatric diagnosis. In 1998,Solimena et al were the first to associate SPS withantibodies against the enzyme glutamic aciddecarboxylase (GAD).

Glutamic acid decarboxylase (GAD) is a neuronalenzyme involved in the synthesis of the neurotransmitter

Investigation results:

Treatment

Outcome/ Followup

Discussion

gamma-aminobutyric acid (GABA). This enzymecatalyses the decarboxylation of L-glutamate intogamma-amino butyric acid (GABA). GABA functionsin GABA-ergic neurons, which are the main inhibitoryneurotransmitter in the brain and spinal cord. Auto-antibodies directed against GAD, lower the levels ofGABA and raise levels of glutamic acid.

This relative GABA deficiency has been proposed as thebasis for the pathophysiology of SPS.

GAD is also synthesized in non-neuronal tissues,including the beta cells of pancreatic islets, testes andoviducts. The enzyme has two isoforms, namely amembrane-associated 65 kDa form, GAD 65, and thesoluble 67 kDa form, GAD67. These isoforms differ instructure and function. In cases of SPS, auto-antibodiesdirected against GAD 65 are present in the sera of up to80%of patients. In 75% of patients, GAD 65 antibodiesare present in the cerebrospinal fluid (CSF). Auto-antibodies to GAD67 are found in 50% or fewer of SPSpatients, although at notably lower titres. The CSF titresare 50 times lower than in serum, although the rate ofantibody synthesis is 10 times higher in CSF.

The lack of GABA results in cerebellar ataxia which is ahallmark of SPS.

1. Stiff-man Syndrome: Case Report Walter F. Kuhn,MD, Peter J. Light, MD, Sharon C. Kuhn, MDAcademic Emergency MedicineVolume 2, Issue 8

2. Stiff-Person Syndrome: A Case Report and Reviewof the Literature Steve Egwuonwu, MD; FernandoChedebeau, MD J Natl Med Assoc.2010;102:1261-1263

References

Corresponding Author: Dr. Leena Dash, Lab Headand Regional Head (Karnataka and Tamil Nadu), SRL-Fortis Hospitals, Bannerghata Road, Bangalore,KarnatakaE-mail: [email protected]

Page 13: Pulse Issue 13 Dec 2015 - SRL Diagnostics December 2015 Issue 13 Editorial Team President-Research & Innovation SRL, Mumbai Director, Department of Medical Oncology Jaslok Hospital

[9]

Pulse December 2015 Issue 13

Extranodal Anaplastic Large Cell Lymphoma, ALK Positive

Ritu Chadha, Priti Jain, Sunita AlhawatSRL, Fortis Memorial Research Institute, Gurgaon, Haryana

Biopsy from a progressively increasing soft tissueswelling from right lower thigh of an 11 year old childrevealed poorly differentiated tumour. Using stepwisepanel of anti-body markers by immunohistochemistry, adiagnosis of Anaplastic Large cell Lym-phoma (ALCL),ALK Positive was made. ALCL ALK+ accounts for 3% ofadult Non-Hodgkin Lymphoma and 10-20% ofchildhood lymphomas. It is considered as a sepa-rateentity in 2008 WHO classification of hematolymphoidmalignancies among Pe-ripheral T cell neoplasms dueto its favourable prognosis compared with ALCL, ALK-and recently developed ALK inhibitors for treatment.

Among mature T cell neoplasms, currently two types ofsystemic ALCL are described: ALK+ ALCL and ALK-ALCL, based on translocation involving anaplasticlymphoma kinase (ALK) gene on chromosome 2 andnucleophosmin (NPM) gene on chromosome 5 or avariant translocations. ALK protein expression identifiedby tissue IHC along with CD30 helps in diagnosisamong wide morphological spectrum of ALCL.

11 year old male patient presented with pain andswelling involving right side lower thigh for 6 months.Initially he was treated for suspected osteomyelitis. Latera Tru-Cut biopsy done elsewhere was reported aschronic non-specific inflammation and pa-tient was puton ATT for 2 months. Since there was progressiveincrease in swelling patient was referred to ourHospital. Patient reported weight loss; however, therewas no history of trauma. MRI of right femur showedlarge soft tissue lesion measuring 9 x 5 cm, involvinglower thigh on right side. Also, underlying corticalirregularity in dia, meta and epiphysis of femur wasnoted. MRI findings were suggestive of a neoplasticetiology and biopsy was done under GA and sent forhistopathology.

Routine investigations CBC, LFT, KFT, PT and APTT werewithin normal limits. Culture from necrotic materialfrom soft tissue and bone were sterile after 5 days andAFB was negative.

Histopathology of biopsy sections showed widespreadinfiltration by poorly differenti-ated tumour comprisinglarge cells with oval to irregular kidney shaped nuclei,finely dispersed nuclear chromatin, inconspicuous tooccasional small nucleoli and abundant cytoplasm. On

Background

Case Presentation

Investigations

Immunohistochemistry, tumour cells were positive forLCA, ALK, CD30, CD43, CD99, CD56 and BCL2(patchy). Ki67 proliferation index was ~ 70%. FewCD68 positive singly scattered histiocytic cells were alsoobserved. List of negative markers comprised T cellmarkers (CD2, CD3, CD4, CD8, CD5, CD7 & Tdt), Bcell markers (CD20, CD79a, PAX-5) and CD15,CD30, EMA, Synaptophysin, CK & Chromogranin. Lossof pan T-cell antigens points to a null phenotype ofALCL ALK+; however, T/null ALCL ALK+ is considereda single entity as both show evidence of T cell lineage atge-netic level. TCR gene rearrangement and ALKmutation analysis were recommended in this case.

ALK positive ALCL must be distinguished from primarycutaneous ALCL, ALCL ALK-, ALK positive diffuse largeB cell Lymphoma and some non- hematopoieticneoplasms such as rhabdomyosarcoma andinflammatory myofibroblastic tumours.

Patient was planned for BFM 90 protocol for paediatriclymphoma.

ALK+ ALCL shows a broad morphologic spectrum, butall cases contain a variable pro-portion of cells witheccentric, horseshoe or kidney-shaped nuclei often withan eosi-nophilic region near the nucleus (“hallmarkcells”). Various morphologic patterns can berecognised such as common, lymphohistiocytic, smallcell, Hodgkin-like, signet ring cell patterns etc.

Differential Diagnosis

Treatment

Discussion

Page 14: Pulse Issue 13 Dec 2015 - SRL Diagnostics December 2015 Issue 13 Editorial Team President-Research & Innovation SRL, Mumbai Director, Department of Medical Oncology Jaslok Hospital

[10]

Pulse December 2015 Issue 13

Composite patterns may also be seen in a single biopsy.Diagnosis is based on expression of CD30 and ALKprotein by tissue IHC. Our case showed a clas-sic largepleomorphic cell pattern and Null phenotype ALCLALK+.

A review of literature highlights ALCL in pediatric agegroups, which mimics non-lymphomatous tumors. Inthe report by Gustafson et al., a 6-year-old girl hadALK-positive ALCL that arose in soft tissue of the neck. Inanother report by Rekhi B et al two patients aged 9 and16 years, respectively, who presented with soft tissueand axillary nodes and were differentially diagnosed asrhabdomyosarcoma and a lym-phoma; however, IHCwas conclusive in diagnosing ALCL. Pant et al, in theirstudy of 12 cases, concluded that the pleomorphiccytomorphology of ALCL leads to confusion with thebone and soft-tissue sarcoma.

High level of suspicion and systematic use of IHCmarkers are required to arrive at a diagnosis of thisentity.

References

1. Gustafson S, Medeiros LJ, et al. Anaplastic largecell lymphoma: Another entity in the differentialdiagnosis of small round blue cell tumors. AnnDiagn Pathol. 2009; 13:413–27

2. Pant V, Jambhekar NA, et al. Anaplastic large celllymphoma (ALCL) presenting as primary bone andsoft tissue sarcoma-a study of 12 cases. Indian JPathol Microbiol. 2007; 50:303–7

3. Lowe EJ, Gross TG. Anaplastic large cell lymphomain children and adolescents. Pe-diatr HematolOncol. 2013;30:509–19.

4. Toby A. Eyre, Dalia Khan, et al. Anaplasticlymphoma kinase-positive anaplastic large celllymphoma: current and future prospective in adultand paediatric disease. Eur J Haematology. 2014;93:455-468

5. Falini B, Martelli MP. Anaplastic large celllymphoma: changes in the World HealthOrganization classification and perspectives fortargeted therapy. Hematologica. 2009; 94:897-900

Corresponding Author: Dr Ritu Chadha, SRL, FortisMemorial Research Institute, Gurgaon, HaryanaE-mail: [email protected]

Solid Pseudopapillary Neoplasm of Pancreas in a Male Child: A RarePresentation

Seema Rana, Mallika Dixit, Anu Chawla, Rajiv TangriSRL, Clinical Reference Laboratory, Gurgaon, Haryana

Summary

Background

A thirteen year old boy, previously in good healthpresented with vague pain in right hypochondrium andupper abdomen since two months. An ultrasoundperformed initially revealed a mass/cystic lesion inhead of pancreas and CT scan was advised.CT scanshowed an inhomogenous well circumscribed mass inhead of pancreas measuring up to 5 cm. There wereno enlarged abdominal lymph nodes .Patientunderwent complete excision of tumor. Grossexamination revealed a solid brown tumor.Histopathology and IHC features were of solidpeudopapillary neoplasm of pancreas.

Solid pseudopapillary neoplasm, an uncommon tumor,usually involves tail/body of pancreas in young females(2nd to 4th decade). It is rare in males and in children.We present a case of Solid pseudopapillary neoplasminvolving head of pancreas in a male child.

Case PresentationMale child presented with pain in right upper abdomen.Past/medical/Social history was not contributory.

• CT scan showed well circumscribed heterogenousmass in head of pancreas.

• Gross examination revealed a solid grey brownwell circumscribed mass measuring up to 5 cm.

• Microscopic examination revealed a tumorcomposed of relatively monomorphic polygonalcells with granular eosinophilic cytoplasm, focallyarranged around peudopapillary fibrous cores.N u c l e i s h o w e d s u b t l e g r o o v e s a n dindentation.Nucloeli were inconspicuous. Mitoticfigures were not seen. No necrosis /haemorrhagewere seen.

• On IHC, tumor cells showed patchy positivity forPANCK and were negative for synaptophysin andchromogranin. Tumor cells were diffusely positive

Investigations

Page 15: Pulse Issue 13 Dec 2015 - SRL Diagnostics December 2015 Issue 13 Editorial Team President-Research & Innovation SRL, Mumbai Director, Department of Medical Oncology Jaslok Hospital

[11]

Pulse December 2015 Issue 13

for vimentin, CD 10 and Progesterone receptor. Ki67 index was less than 1%. On the basis of aboveradiologic, histologic and IHC findings, patient wasdiagnosed with Solid pseudo papillary neoplasm ofpancreas.

Differentials considered were pancreatoblastoma,acinic cell carcinoma and neuroendocrine tumours.On histology, presence of characteristic nuclearfeatures, granular eosinophilic cytoplasm along withfocal peudopapillary arrangement of cells pointedtoward solid peudopapillary neoplasm. Thesehistopathological features coupled with IHC pattern ofdiffuse vimentin, PR and CD 10 positivity and negativestaining for synaptophysin and chromogranin in a wellcircumscribed heterogenous tumor on CT scan led us tothe diagnosis of solid pseudo papillary neoplasm ofpancreas.

Complete excision of tumor by pylorus sparingpancreatoduodenectomy was performed.

One year follow up of patient did not show anyrecurrence or metastasis.

Figure 1 Figure 2

Fig 1) H&E (40 X):- Tumor with pseudopapillary arangement of

cells,nuclear indentation and granular cytoplasm.Inset(Tumor

cells do not express synaptophysin and chromogranin on IHC).

Fig 2) IHC :- Tumor cells showing patchy positivity for PanCK,

diffuse positivity for Vimentin, CD 10 and PR.

Differential Diagnosis

Treatment

Outcome and Followup

Discussion

Learning Points/ Take Home Messages

References

Solid peudopapillary neoplasm, an uncommon tumorwith strong predilection for young females is rare inmale children. Review of the literature revealed abouttwenty five cases of solid peudopapillary neoplasm ofpancreas in male children with an average age ofpresentation being 10.5 years (1, 2).

CT and MRI of this tumor reveal a well definedheterogeneous mass involving head/body/tail ofpancreas (2). Careful nuclear features (nuclear grovesand nuclear indentation) can aid in diagnosis wherepapillary architecture is not present (2). There are nosignificant differences between male and femalepatients regarding surgical strategies and prognosis(3).

• Solid peudopapillary neoplasm should always beconsidered in differential diagnosis of pancreatictumours irrespective of age profile and sex of thepatient.

• Careful examination of radiological findings,histological features along with IHC help us toarrive at a diagnosis.

1. Parelkar V, Oak N et al, Solid peudopapillary tumorof the pancreas: An unusual tumor in children,2013, J Indian Assoc Paediatric Surg, 18(1):38-40.

2. Roganovic J, Matijasic N et al. Paediatric SolidPeudopapillary Neoplasm of the pancreas –casereport and literature review, 2015, Annals ofClinical Pathology3 (1):1044.

3. Cai Yun –Qiang, Xie Si –Ming etal. Solidpeudopapillary neoplasm of the pancreas in malepatients: Report of 16 cases. World JGastroenterol.2014, Jun 14;20(22)

Corresponding Author: Dr. Seema Rana, ChiefHistopathologist, SRL, Clinical Reference Laboratory,Gurgaon, HaryanaE-mail: [email protected]

Page 16: Pulse Issue 13 Dec 2015 - SRL Diagnostics December 2015 Issue 13 Editorial Team President-Research & Innovation SRL, Mumbai Director, Department of Medical Oncology Jaslok Hospital

[12]

Pulse December 2015 Issue 13

An Interesting Cause of Brain Abscess

Shiva Priya Eswaran, Pramod Nath, Nitin Dumeer, Kamlesh Kumar, AbhaSabhikhiSRL Fortis Hospital Vasant Kunj, New Delhi

Summary

Background

Case Presentation

This is an interesting case report of a 53 year male aknown diabetic, hypertensive and a case of chronicrenal failure on haemodialysis who presented withheadache, drowsiness and left hemipareisis. Patientunderwent right frontal craniotomy and total excision offrontal lobe abscess along with lobectomy. Direct Gramstained preparation of pus and 10% Potassiumhydroxide (KOH) preparation revealed pus cells, but nobacteria and presence of broad hyphal elements. Thepatient being a diabetic a provisional diagnosis ofmucor mycosis was entertained. However no fungalgrowth was obtained in 48 hours of incubation. After 96hours of incubation, the blood agar showed growth ofcolonies with an olive gray velvety appearanceconsistent with Cladophialophora bantiana. He wastreated with Amphotericin B and Flucytosine andhaemodialysis. Post operatively he made good recoverywith improvement in sensorium and hemiplegia. Ondischarge, patient was conscious alert with minimal leftsided weakness.

Phaeohypomycosis is a less common cause of brainabscess and aetiological agent Cladophialophorabantiana was isolated in culture. Timely diagnosishelped in targeted antifungal therapy with the patientshowing good response and neurological recovery.Case reports from India are very few.

• This is an interesting case report of a 53 year male,a known diabetic, hypertensive and a case ofchronic renal failure on haemodialysis whopresented with complaints of headache,drowsiness and left hemipareisis of 3 days duration.There was no history of seizure or trauma.

• Physical examination revealed an extremely drowsypatient, opening eyes to repeated calling, obeyingsimple commands and left hemiplegia with 0/5power. Radiological evaluation CT head doneshowed a ring enhancing mass in the right frontalregion with severe diffuse oedema.

• Patient underwent right frontal craniotomy and totalexcision of frontal lobe abscess along withlobectomy under general anaesthesia. Care wastaken not to open the ventricles.

• Direct Gram stained preparation of pus revealedpus cells, but no bacteria and presence of broad

hyphal elements.(Fig.1) The smear stained by ZiehlNeelsen method was also negative for acid fastbacilli. Pus for bacterial culture and sensitivity wassterile. 10% Potassium hydroxide (KOH)preparation of pus showed numerous broadhyphae with near right angle branching. Thepatient being a diabetic a provisional diagnosis ofmucor mycosis was entertained.

• However no fungal growth was obtained in 48hours of incubation. After 96 hours of incubation,the blood agar showed growth of colonies with anolive gray velvety appearance (Fig.2) which on LCBmount showed dark walled septate hyphae withsingle celled oval conidia in long branched chains(Fig. 3). Hyphae were septate and darklypigmented with many conidia attached to the sidesand lying free. Conidia were one-celled, palebrown, smooth-walled, and ellipsoid in shape. Theconidiophores showed an acropetous type ofbranching (Fig. 4). The isolate could be grown at42°C and was also urease positive, features whichdi f ferent ia tes C. bant iana from othermorphologically similar saprophytic fungi.

• Histopathological examination also showed agranulomatous inflammation along with numerousseptate fungal hyphae compatible withCladophialophora (Fig.5 and Fig.6).The fungalpresence was confirmed by special stains (PAS)(Fig.7).

Fig.1 Gram stain of pus Fig.2 Dematiaceous fungalcolonies on Blood Agar

Fig.3 LCB mount(Low power 10x)

Fig.4 LCB mount(High power 40x)

Page 17: Pulse Issue 13 Dec 2015 - SRL Diagnostics December 2015 Issue 13 Editorial Team President-Research & Innovation SRL, Mumbai Director, Department of Medical Oncology Jaslok Hospital

[13]

Pulse December 2015 Issue 13

Discussion

Learning Points/ Take Home Messages

References

Phaeohyphomycosis is a term used to describeinfections caused by dematiaceous fungi, i.e., fungiwhich contain melanin in their cell wall.[1]Cladophialophora bantiana has been implicated tocause brain abscess in immunocompromised patientsincluding solid organ transplant recipients and thosewith congenital or acquired immunodeficiency.[2-6]

• Timely reporting of the presence of dematiaceousfungus from such samples can serve as a usefuladjunct to clinical management.

• Less common cause of brain abscess and should beconsidered as a differential diagnosis and shouldbe identified.

1. Revankar SG, Sutton DA, Rinaldi MG. Primarycentral nervous system phaeohyphomycosis: Areview of 101 cases. Clin Infect Dis 2004;38:206-16.

2. Levin TP, Baty DE, Fekete T, Truant AL, Suh B.Cladophialophora bantiana brain abscess in asolid-organ transplant recipient: Case report andreview of the literature. J Clin Microbiol2004;42:4374-8.

3. Gupta SK, Manjunath-Prasad KS, Sharma BS,Khosla VK, Kak VK, Minz M, et al. Brain abscess inrenal transplant recipients: Report of three cases.Surg Neurol 1997;48:284-7.

4. Sood P, Dogra V, Thakur A, Mishra B, Mandal A,Sinha S. Brain abscess due to Xylohypha bantiana.Scand J Infect Dis 2000;32:708-9.

5. Goel A, Satoskar A, Desai AP, Pandya SK. Brainabscess caused by Cladosporium trichoides. Br JNeurosurg 1992;6:591-3.

6. Banerjee U, Mohapatra AK, Sarkar C, ChaudheryR. Cladosporiosis (cerebral phaeohyphomycosis)of brain: A case report. Mycopathologia1989;105:163-6.

Corresponding Author: Dr. Shiva Priya EswaranSRL Fortis Hospital Vasant Kunj, New Delhi-110070E-mail: [email protected]

• The pus culture and histopathological examinationwere both suggestive of brain abscess caused byCladophialophora bantiana.

• He was treated with Amphotericin B(5mg/kg for 28days) and Flucytosine (100mg per day for 6 weeks)and haemodialysis. Post operatively he made goodrecovery with improvement in sensorium andhemiplegia. On discharge, patient was consciousalert with minimal left sided weakness.

Radiological evaluation CT head done showed a ringenhancing mass in the right frontal region with severediffuse oedema.

Mucormycosis

He was treated with Amphotericin B (5mg/kg for 28days) and Flucytosine (100mg per day for 6 weeks) andhaemodialysis.

Post operatively he made good recovery withimprovement in sensorium and hemiplegia. Ondischarge, patient was conscious alert with minimal leftsided weakness. Patient is on regular neurosurgicalfollow up with improvement in weakness.

Investigations

Differential Diagnosis

Treatment

Outcome and Followup

Fig.5 H/E stain showinggranulomatous inflammation

Fig.6 GMS stain

Fig.7 PAS stain

Page 18: Pulse Issue 13 Dec 2015 - SRL Diagnostics December 2015 Issue 13 Editorial Team President-Research & Innovation SRL, Mumbai Director, Department of Medical Oncology Jaslok Hospital

[14]

Pulse December 2015 Issue 13

Syringomatous Adenoma of Nipple

Shobha Khanduri, Shabnam Izhar, Sneha TiwariSRL Ltd, Lucknow, Uttar Pradesh

Summary

Background

Case Presentation

Syringomatous adenoma of the nipple is a rare disease.We present a case of a 33 years old woman withSyringomatous adenoma of the nipple, in whom localexcision of the tumour was performed. Histologically,the tumour consisted of tubules, ductules and epithelialcell strands and some of the proliferating ducts showedcharacteristic tear drop and comma shapedappearance. Careful monitoring to detect the localrecurrence is considered necessary, becauseSyringomatous adenoma of the nipple, while benign, isa locally invasive tumor but does not metastasize.

Syringomatous adenoma of the nipple is a very rarebenign tumor that was first reported by Rosen in 1983[1]. To the best of our knowledge, only 38 cases havebeen reported in the literature [2].

A 33 years old lady presented with firmness of her rightnipple for 1 year. On examination nipple was retractedand firm to hard. No other lesion was palpable. Noregional lymph node involved. Cone biopsy of nipplewas taken with deeper tissue and sent forhistopathological examination.

Grossly we received two skin covered soft tissue pieces.Cut section of larger tissue piece was grayish white andshow tiny cystic spaces.

On microscopy the lesion located mainly in nipple. Thelining of keratinized stratified squamous epitheliumnotice with underlying fibrous stroma showinghaphazard proliferation of small oval ductules andtubules composed of small basophilic cells and fewbilayered epithelial tubular structures. Ducts arecomma shaped at places. Many keratinizingsquamous cysts and solid islands of cells also seen. Noatypia or mitotic activity was seen.

Discussion

Learning Points/ Take Home Messages

Syringomatous adenoma of the nipple is an extremelyrare benign tumor. To the best of our knowledge, only38 cases have been reported in the English literature[2], [4]–[5], including 36 female and 2 male cases. Theage of these patients ranged from 11 to 87 years, with amean age at presentation of 46.1 years.

Syringomatous adenoma of the nipple commonlymanifest as solitary firm masses in the subareolar ornipple region of the unilateral breast, and may alsooccur within the breast parenchyma. They may beclinically asymptomatic, tender and painful onpalpation, and/or present with itching and ulceration[6]. Nipple inversion or discharge is noted on occasion[3].

Syringomatous adenoma of the nipple is a rare tumor,and diagnosis can be difficult. Based on histologicalclassification of mammary gland tumors, differentialdiagnosis include nipple adenomas and low-gradeadenosquamous carcinomas. Nipple adenomas arepapillary or solid adenomas developing within thenipple where proliferation of papillary epithelium isremarkable [1]. Low-grade adenosquamouscarcinomas that derive from the salivary gland ductshow an adenoma-like structure. Because theyfrequently develop in the large and small salivaryglands, these carcinomas can be differentiated fromtheir sites of origin [7]

Syringomatous adenoma of the nipple forms comma-shaped cell nests or small glandular cavities with singleor multiple layers of small homogeneous epithelial cellsin a background of dense stromal cells. It proliferatesand can infiltrate tissue from the nipple to as far as thesubareolar stroma. Some of them differentiate tosquamous epithelium.

Based on morphological features, we diagnosed thetumor in this case as Syringomatous adenoma of thenipple. The tumors are likely to have originated from theeccrine sweat ducts remaining in the breast.

• Syringomatous adenoma of the nipple is a rarebenign but locally aggressive tumour; thus, cautionis required. It should also be considered as one ofthe differential diagnosis when any nodule in nippleor retractionof nipple.Fig 1a (4x), b (10x) Photomicrograph shows haphazard proliferation

of small oval ductules and tubules composed of small basophilic cells.Coma shaped ducts at places. Keratinising squamous cyst also seen.

Page 19: Pulse Issue 13 Dec 2015 - SRL Diagnostics December 2015 Issue 13 Editorial Team President-Research & Innovation SRL, Mumbai Director, Department of Medical Oncology Jaslok Hospital

[15]

Pulse December 2015 Issue 13

mound resection and oncoplastic reconstruction.Breast J. 2008;14(1):102–105.

4. Sarma DP, Stevens T. Infiltrating syringomatouseccrine adenoma of the nipple: a case report.Cases J. 2009; 2:9118.

5. Mrklic I, Bezic J, Pogorelic Z, Ilic N, Tadic T, BuljevicV et al. Synchronous bilateral infiltratingsyringomatous adenoma of the breast. Scott Med J.2012; 57:121.

6. Carter E, Dyess DL. Infiltrating syringomatousadenoma of the nipple: a case report and 20-yearretrospective review. Breast J. 2004; 10:443-7.

7. Rosen PP: Syringomatous adenoma of the nipple;in Rosen PP (ed): Rosen’s Breast Pathology, ed 2.Philadelphia, Lippincott Williams and Wilkins,2001, pp 111–114.

Corresponding Author: Dr Shabnam IzharSRL Ltd, Lucknow, Uttar PradeshE-mail: [email protected]

• Complete excision is necessary for the treatment ofSyringomatous adenoma of the nipple in order toavoid recurrence of the tumor and confirmation ofhistologically negative margins.

• Clinicians and pathologists should be aware of thepossibility of diagnosing this extremely rare tumorto avoid unnecessary mastectomy and axillarylymph node dissections.

1. Rosen PP. Syringomatous adenoma of the nipple.Am J Surg Pathol. 1983; 7:739-45.

2. Ichinokawa Y, Ohtuki A, Hattori M, Sadamasa H,Hiruma M, Matumoto T. A case of syringomatousadenoma of the nipple. Case Rep Dermatol. 2012;4:98-103.

3. Oliva VL, Little JV, Carlson GW. Syringomatousadenoma of the nipple— treatment by central

References

Myroides Species: A Rare Cause of Endocarditis

Shweta Sharma, Anu Gupta, Deepa RaoSRL Ltd, Fortis Escorts Heart Institute, Okhla, New Delhi

Summary

Background

We present a case 32 years old female, known case ofrheumatic heart disease that underwent valvereplacement around 17 years back. She was admittedwith complaints of breathlessness since 3 days inemergency for evaluation. She underwent redo MVR.Intraoperatively vegetations were present on the mitralvalve. Subsequently her blood culture grew Myroidesspp. She responded well to treatment and discharged.Myroides spp are not part of human flora and arecommonly found in soil and water. They are consideredas low grade pathogens. Genera Myroides aremembers of the family Flavobacteriaceae and compriseof a group of yellow-pigmented, oxidase positive, nonmotile, non fermentative Gram negative bacilli.However now a days they have been identified ascausative agents in urinary tract infections, pneumonia,men ing i t i s , f a sc i i t i s and ven t r i cu l i t i s i nimmunocompromised patients and a few life-threatening infections have been reported inimmunocompetent individuals also.

Myroides spp previously known as Flavobacteriumodoratum are rare source of human infections. Naturalhabitat includes soil, fresh and marine waters, foods

and sewage treatment plants. This case is presented toshow the increasing incidence of uncommon isolatescausing endocarditis. Isolation of such organism is ofclinical significance due to its high resistance to thecommonly used antibiotics.

• Patient is a 32 old female non diabetic, known caseof rheumatic heart disease undergone mitral andaortic valve replacement about 17 years back. Shepresented with complaints of breathlessness onwalking and lying position in emergency. She wasinvestigated and found to have stuck valve.

• After proper investigation and work up sheunderwent mitral valve replacement Vegetationswere present on the mitral valve.

• Post operatively patient was put on inotropicsupport.

• She developed high TLC, fever for which bloodculture, Urine culture were send. Empirically shewas put on Inj Meropenem and Inj teicoplanin. Herblood culture grew Myroides spp. Then targocidwas disccontinued and meropenem was continuedfor 21 days as per the sensitivity report. She

Case Presentation

responded well. Subsequent blood cultures werenegative.

Page 20: Pulse Issue 13 Dec 2015 - SRL Diagnostics December 2015 Issue 13 Editorial Team President-Research & Innovation SRL, Mumbai Director, Department of Medical Oncology Jaslok Hospital

[16]

Pulse December 2015 Issue 13

flexirubin pigment and produce a fruity odour. M.odoratus did not grow on MacConkey agar.

• Although Myroides spp. are rare clinical isolates, ithave been found to be associated with variousclinical infections ranging from recurrent cellulitis,post-operative wound infections and necrotizingfasciitis to severe septicaemia.

• Cases of endocarditis and ventriculitis have alsobeen reported by Maraki et al.

• Myroides odoratimimus bacteremia was reportedby Tiana R et al in a diabetic patient who wasresponded to Inj MeropenemMulti-drug resistant nature makes identificationand antibiotic susceptibility testing very important.Resistance to -lactams mainly due to theproduction of chromosomally encoded metallo- -lactamases (TUS-1 and MUS-1) has been reportedby Mammeri et al. Clinical isolates of Myroides hadbeen found to be susceptible to quinolones andtrimethoprim-sulfamethoxazole and clinical curewas attained in few studies.

Myroides spp. infections are being increasinglyreported in both immunocompromised andimmunocompetent hosts. This case highlights the factthat a rare pathogen like Myroides spp. should beconsidered in differential diagnosis of endocarditis andclinicians must be aware of its pathogenic role.

1. Tiana R. Endicott-Y, et al. Myroides odoratimimusbacteremia in a diabetic patient. Proc (Bayl UnivMed Cent) 2015; 28(3):342–343

2. Motwani B, Krezolek D et al. Myroides odoratumCellulitis and Bacteremia: A Case Report. (InfectDis Clin Pract 2004;12:343–344)

3. Elantamilan D, Valarie W et al. Septicaemia causedby Myroides spp.: a case report. JMM Case Reports(2015) DOI 10.1099/jmmcr.0.000097

4. Prateek s, Gupta p Fatal Case of PericardialEffusion Due to Myroides Odoratus:A Rare CaseReport Journal of Clinical and DiagnosticResearch. 2015 Nov, Vol-9(11): DD01-DD02.

5. Maraki S, Sarchianaki E, Barbagadakis S. Myroidesodoratimimus soft tissue infection in animmunocompetent child following a pig bite: casereport and literature review. The Braz J Infect Dis.2012;16(4):390-92.

6. Ferrer C, Jakob E, Pastorino G, Juncos LI. Right-sided bacterial endocarditis due [6] toFlavobacteriun odoratum in a patient on chronichaemodialysis. Am J Nephrol. 1995;15:82–84

Learning Points/ Take Home Messages

References

Corresponding Author: Dr. Shweta Sharma,Microbiologist, SRL Ltd, Fortis Escorts Heart Institute,Okhla, New DelhiE-mail: [email protected]

Investigation:

Outcome and Followup

Discussion

Routine investigation: Normal except high TLC count,Procalcitonoin = 12.21.Echocardiography: Stuck Mitral valve. Mean PG =42mm Hg. Good bolus across aortic valve. No clot,vegetation.Blood Culture: 2 sets of blood culture were send.Positive signal came after 12 hours of aerobicincubation in BacTAlert system. Gram stain showedgram negative bacilli.Macroscopic Features: On blood agar plate, colonieswere round, non hemolytic yellow-pigmented about1–2 mm in size. (Fig. 1), It gives characteristic fruityodour. No growth on Macconkey agar.Identification of species: The organism was a catalase-and oxidase-positive. Further identification was doneusing VITEK 2 compact, BioMe´rieux; which identified itas Myroides sp. (Excellent Identification).Antibiotic SusceptibilityVitek 2compact does not gave the sensitivity. Soantimicrobial susceptibility testing was done onMueller–Hinton agar by the Kirby–Bauer disc diffusionmethod and interpreted following Clinical LaboratoryStandards Institute standards (CLSI, 2014), using thezone diameters for Pseudomonas aeruginosa (as thereare no established standards for Myroides spp).

Patient condition at the time of discharge was stable.

• Initially the genus Myroides comprises of twospecies M odoratus and M odoratimimus. Recently,three novel species (M pelagicus, Ms profundi andM marinus) were isolated from seawater. Myroidesodoratus was first isolated by Stutzer et al.

• It grows on most media and produce lightly yellowpigmented colonies due to the presence of

Fig 1: Blood agar showing growth of Myroides spp

ANTIBIOTIC

AmikacinGentamicin

CiprofloxacinPiper+tazobactam

CeftazidimeCefoperazone sulbactum

CefepimeImipenem

MeropenamAztreonam

INTERPRETATION

ResistantResistantSensitiveSensitiveResistantResistantResistantResistantSensitiveResistant

Page 21: Pulse Issue 13 Dec 2015 - SRL Diagnostics December 2015 Issue 13 Editorial Team President-Research & Innovation SRL, Mumbai Director, Department of Medical Oncology Jaslok Hospital

[17]

Pulse December 2015 Issue 13

Liposarcoma of Tongue

Subhra Dhar, Piyali Biswas, Kundan Nawale, Chandreyi BanerjeeSRL Ltd, Kolkata Reference Lab, Kolkata, West Bengal

Summary

Background

Case Presentation

Liposarcoma as are the commonest soft tissuemalignancies but liposarcomas of the head and neckare rare, representing 5 - 6 % of all sarcomas in thisregion. Of the intraoral liposarcomas, the tonguerepresents the most common site. The subset ofintraoral liposarcomas is also more interesting becausethey tend to have a better prognosis in this region (1).However accurate clinical and histopathologicdiagnosis of this lesion is difficult. The majority of casesrun a benign course but have a high propensity to recur(2). Clinically they usually present as painless polypoidmasses in the tongue but can also be multinodular (3).Sometimes they can be underdiagnosed as benignlipomas or misdiagnosed because of low mitotic activityand relative paucity of atypical cells (3). Histologicallymost are well differentiated type and respond well towide surgical excision (4). We present here a case ofwell differentiated liposarcoma of tongue because of a)its extreme rarity b) unusual clinical presentation. Ourcase presented as a lingual ulcer on the left lateralborder and extensive literature review revealed just asingle case of liposarcoma of tongue presenting asulcer.

Given the rarity of liposarcoma in oral mucosa, it isimportant to keep in mind this entity when dealing withgrowth in this region. Presentation as a chronic lingualulcer also adds to the pecularity of this case. Carefulattention to histopathologic features whenever dealingwith a lipomatous lesion can lead to correct diagnosisand prevent mis or under diagnosis.

An eighty four year old female presented with anulcerated mass on right lateral border of tongue of twoyears duration. The ulcerated mass was excised andsent to our lab for histopathology examination.• Gross examination showed an irregular greyish

yellow tissue piece with ulcer measuring 1.5x1.1 x 0.8 cm. The tissue was firm in consistency.

• On light microscopic examination an ulceratedbed was seen with hyperplastic stratified squamousepithelium on either side of the ulcer. The ulcer bedwas infiltrated by many neutrophils, lymphocytesand eosinophils. (Fig 1). Another striking featurewas the presence of lobules of lipocytes whichshowed marked variation in size. Carefulexamination revealed skeletal muscle infiltration bythe lipocytes (Fig 2).. The lipocytes reached right

upto the base and also extended into the lateralmargins. A thorough search for lipoblastic cellsyielded sparse presence of mostly late stagelipoblasts. These had hyperchromatic eccentricallyplaced nuclei in a large vacuolated cell (Fig 3). Nonecrosis or mitosis were seen. The stroma alsoshowed broad bands of collagen and bloodvessels, the walls of which harboured spindloidcells ( Fig 4). Based upon the above findings adiagnosis of Atypical lipomatous tumour (ALT)/ welldifferentiated liposarcoma (WDLS ) of tongue wasmade.

Liposarcoma of the tongue is a tumour of adult and oldage with no gender predeliction. Mean age is sixty twoyears with a peak incidence in the 7th and 8th decades.Clinically it is an indolent lesion with a long history andpresents usually as a polypoid or nodular massmeasuring 3-4cm in average diameter (5). Afterextensive search of literature we came across only onecase report in a twenty one years old male whopresented as an ulcerated nodule on the tongue withtelangiectasia and a firm consistency6. Our case toopresented as an ulcerated mass and this could havebeen caused by mechanical trauma.The most commonhistopathologic type is that of ALT/ WDLS (75%). In thetongue they have often been categorised mostly aslipoma like Liposarcoma. However spindle cell typeand sclerosing type may also be found. In our case too,typical lipoma like areas were seen, however on carefulobservation under high power (40x) the lipoblast cellwas identified. Lipoblast once considered essential todiagnose liposarcoma may be very sparse or altogetherabsent, however thick bands of collagen and muscleveins harboring spindle cells can also help in arriving at

Discussion

Fig 1 Fig 2

Fig 1 Fig 2

Page 22: Pulse Issue 13 Dec 2015 - SRL Diagnostics December 2015 Issue 13 Editorial Team President-Research & Innovation SRL, Mumbai Director, Department of Medical Oncology Jaslok Hospital

[18]

Pulse December 2015 Issue 13

a correct diagnosis (7).

ALT/WDLS is usually well circumscribed butunencapsulated. Muscle infiltration can mimic anintramuscle lipoma, which can lead to potentialunderdiagnosis. Immunohistochemistry is not of muchuse in the diagnosis of ALT/WDLS. Lipoblasts are S-100positive, but as they are very sparse9 in ATL/WDLSvariant, the use of IHC gets limited. Wide excision withclear margin is the treatment of choice for tongueliposarcoma. Tumor negative margins are associatedwith long disease free survival (8).

When a lipomatous lesion is encountered in the tongue,Liposarcoma should certainly be considered as apossible diagnosis. WD LS can be easily misdiagnosedas a benign lipoma. Though a malignant tumour,achieving clear tumour negative margins renders afavourable prognosis to the patient.

1. Adelson RT , De Fatta RJ, Verret DJ, Shen Y,Liposarcoma of the tongue : case report andreview of literature; Ear Nose Throat J 2006 Nov;85 (II) : 749 - 51.

2. Orita Y, Nishizaki K, Ogewara T et al; Liposarcomaof the tongue: case report and literature update.Ann Otol Rhinol Laryngol. 2000 Jul; 109(7):683-6

3. Saddik M, Olding DJ, Moured WA, Liposarcoma ofthe base of tongue and tonsillar fossa: A possibly

Learning Points/ Take Home Messages

References

under diagnosed neoplasm, Arch Pathol Lab Med,1996 Mar; 120 (3): 292 - 5.

4. Fanburg – Smith JC, Furlong MA, Childers EL,Liposarcoma of the oral and salivary gland region;a clinicopathologic study of eighteen cases withemphasis on specific site morphologic subtypesand clinical outcome; Mod Pathol 2002 Oct; 15(10): 1020-31

5. Irit A , Marilena V, Dan D; Liposarcoma of thetongue: clinico-pathologic correlations of aposs ib le underd iagnosed ent i t y ; DOI:http://dx.doi.org/10.1016/j.oraloncology.2005.01.006

6. Liposarcoma of the tongue: case report; DOI:http://dx.doi.org/10.1016/j.0000.2012.08.025

7. Ayman FA, Mahammad T M; A case report ofatypical lipomatous tumour of the tongue andliterature review, Sudan Med J 2012 December;48(3)

8. Evangelia P, Konstantinus IT, Nikolas GN et al; Well- Differentiated Liposarcoma/ Atypical lipomatoustumour of the Oral cavity: Report of three cases andreview of the literature, Head Neck Pathol 2012Sep; 6(3): 354-363

Corresponding Author: Dr. Subhra DharSRL Ltd, Kolkata Reference Lab, P.S. Srijan Tech Park,DN 52, Saltlake, Sector V, Kolkata,West Bengal - 700091E-mail: [email protected]

Brain Teasers

1. Diagnose the FollowingBone marrow aspirate of a 12 yr old child(resident of Bihar); presenting with PUO,anaemia and huge hepatosplenomegaly.Contributed by: Dr. Arpita Roy Dam, Clinical ReferenceLaboratory, SRL Ltd, Gurgaon, Haryana

2. Diagnose the Following 3. Identify the organism and specificfeature shown in the pic

49 year old female, 6 months post liver transplantfor HCV Cirrhosis, now presenting with cholestasis.Contributed by: Dr Nalini Bansal and Dr Shaloo KapoorSRL Ltd, Fortis Hospital, Okhla, New Delhi

A 65 yr female presented withB/L nasal sinusitis and necroticmaterial in middle turbinates.Contributed by: SRL Lab Team, Fortis Shalimar Bagh, New Delhi

Page 23: Pulse Issue 13 Dec 2015 - SRL Diagnostics December 2015 Issue 13 Editorial Team President-Research & Innovation SRL, Mumbai Director, Department of Medical Oncology Jaslok Hospital

[19]

Pulse December 2015 Issue 13

SRL ActivitiesRecent Test Releases

Next Generation SequencingBased Hereditary Cancer Panels :HCP-Next and HCP Expanded

Fibrometer – NAFLD & FibrometerALD

Phospholipase A2 receptors(PLA2R) Antibodies

PAX-5

DMD – PNDT

Adenovirus Quantitative DNA PCR

EBV Quantitative DNA PCR

IFOBT (immunochemical-basedfecal occult blood testing)

Red Kidney Beans Allergen

Papaya Allergen

Pumpkin Allergen

Myotonic Dystrophy Type 1 (DM1)

Oncology

Gastroenterology

Autoimmune Disorders

Oncology

Genetic Disorders

Infectious Diseases

Infectious Diseases

Oncology

Allergy

Allergy

Allergy

Genetic Disorders

1. Bhanushali AA, Patra PK, Nair D, Verma H, Das BR.Genetic variant in the BCL11A (rs1427407), but notHBS1-MYB (rs6934903) loci associate with fetal hemoglobin levels in Indian sickle cell disease patients.Blood Cells Mol Dis. 2015 Jan; 54(1):4-8.

2. Vadwai V, Mandsaurwala A, Mukherjee S, Vora S, Das BR. CCR5 Genotyping and Chemokine Receptorusage among HIV-1 Treatment - Naive and Experienced Patients in India. Int J AIDS Res. 2015;02(5), 43-45.

Recent Publications

1. Hereditary Cancer Panel Next targets 32 genes related to7 major cancer types along with 21 associated syndromes.2. Hereditary Cancer Panel Expanded targets 48 genesrelated to 10 cancer types along with 29 associatedsyndromes.

Fibrometer NAFLD combines nine variables (age, weight,fasting glucose, AST, ALT, Ferritin, Platelet count, Prothrombintime and Hyaluronic acid). FibroMeter ALD combines sixvariables (Age, Gender, Alpha-2-macroglobulin, HyaluronicAcid, Prothrombin index and Platelets).

Used to differentiate between anti-PLA2R positive andidiopathic cases. Predicts disease outcome, therapyrequirements and the risk of recurrence after kidneytransplantation.

Aids in diagnosis of undifferentiated lymphoid neoplasm’s.

Prenatal diagnosis of DMD.

Detects adenovirus in immunocompromised patients.

Used to investigate primary or reactivated EBV infection inimmunocompromised patients.

Detects CRC in asymptomatic population; CRC screening inaverage-risk adults (>50 yrs).

Detects allergic reactions to red kidney beans.

Detects allergic reactions to red kidney beans.

Detects allergic reactions to red kidney beans.

Confirmation of DM1

Answer for Last Issues Brain TeaserIdentify the disease (Issue 12, June 2015) -Nevus comedonicus

4. Diagnose the Following45 yrs/ F, Gyane Pap smear. Contributedby: Dr. Neeraj Garg, SRL Limited, FortisMemorial Research Institute, Gurgaon,Haryana

Page 24: Pulse Issue 13 Dec 2015 - SRL Diagnostics December 2015 Issue 13 Editorial Team President-Research & Innovation SRL, Mumbai Director, Department of Medical Oncology Jaslok Hospital

Prime Square, Plot No 1, S. V. Road, Goregaon (W), Mumbai – 400 062, India

For more information please visit us at www.srlworld.com

Pulse December 2015 Issue 13

Continuing Medical Education on SRL Global Knowledge Forum

Under the aegis of SRL organized during July 2015 toNovember 2015.

The CME seminars covered various medical disciplines e.g. gastroenterology, metabolic disorders, hormonaldisorders, hematology, neonatology, infectious diseases, genetic disorders, autoimmunity and allergy, oncology,cardiology, gynecology, neurology, nephrology etc.

Here are some of the notable programmes we organized:

SRL Global Knowledge Forum, 151 CME programs

CME on ‘Preanalytical errors in Diagnostics’

CME on ‘Tuberculosis in Bone and Joint’

CME on ‘Ethical Practices in Pathology’

CME on ‘Blood Based Biomarkers in NAFLDDiagnostics’

CME on ‘Newborn Screening’

Date: July 01, 2015Venue: IMA Institute, Bhubaneswar

Date: August 13 & 19, 2015Venue: Assam Medical College, Dibrugarh

Date: September 08, 2015Venue: Sisir Mancha, Kolkata

Date: September 21, 2015Venue: GB Pant Hospital, Delhi

Date: November 16, 2015Venue: BG Road, Bangalore

CME on ‘Recent Advances in TuberculosisDiagnosis’

CME on ‘Diagnosis of Allergy in Children’

CME on ‘Recent Advances in Cervical Cancer

CME on ‘Advancement of Diagnosis in GITAilments’

CME on ‘Breast Cancer - BRCA1/2 GeneticTesting on NGS’

Date: July 04, 2015Venue: Agra

Date: August 22, 2015Venue: Fortis Hospital, Bangalore

Screening and Infertility’Date: September 12, 2015Venue: NMCH, EMOC Hall, Patna

Date: October 03, 2015Venue: SGPGI, Lucknow

Date: November 24, 2015Venue: Nagpur