an uncommon case of burkitts lymphoma causing ileo-cecal ... · on investigating, her complete...

Article ID: WMCPLS00398 ISSN 2051-0799

An Uncommon Case Of Burkitts LymphomaCausing Ileo-cecal Intussusception In A Child.Corresponding Author:Dr. Dnyanesh Belekar,Associate Professor & Unit Head - Department of General Surgery, K. J. Somaiya Medical College & ResearchCentre,Sion,Mumbai, Ayurvihar, Sion, Mumbai, 400022 - India

Submitting Author:Dr. Dnyanesh Belekar,Associate Professor & Unit Head, K J Somaiya Medical College & Research Centre, Ayurvihar, Sion, Mumbai,400022 - India

Submitted on:22-Sep-2014, 03:58:40 AM GMT

Accepted on:24-Sep-2014, 05:21:28 AM GMT

Article ID: WMCPLS00398

Article Type: Case series

Article URL: http://webmedcentralplus.com/article_view/398

Subject Categories:GASTROINTESTINAL SURGERY

Keywords:Burkittas lymphoma, ileo-cecal intussusception.

How to cite the article:

Belekar D, Sailukar M, Bansal S, Thakur S, Hajirnis K, Patki A, Agarwal A.An Uncommon Case Of BurkittsLymphoma Causing Ileo-cecal Intussusception In A Child.. WebmedCentral plus GASTROINTESTINALSURGERY 1970;-39(1):WMCPLS00398

Source(s) of Funding:

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Competing Interests:

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An Uncommon Case Of Burkitts LymphomaCausing Ileo-cecal Intussusception In A Child.Author(s): Belekar D, Sailukar M, Bansal S, Thakur S, Hajirnis K, Patki A, Agarwal A

Abstract

Burkitt’s Lymphoma (BL) is an uncommon type ofNon-Hodgkin’s Lymphoma. It is mainly seen inchildren in African and Algerian region. In India it is arare disease. It mainly affects B lymphocytes. Thecommonly involved sites are either jaw or abdomen inIndian scenario. BL affecting the ileo-cecal junction isagain observed uncommonly. We would like to presenta rare case where a young female child has sufferedfrom acute abdomen with features of intussusceptionand underwent resection of the Ileo caecal Junctionmass along with ileo-ascending anastomosis, thehistopathology of which showed BL.

Key words: Burkitt ’s lymphoma, i leo-cecalintussusception.

CaseHistory

Five years old Muslim female child presented inpaediatric OPD with chief complaints of on and offabdominal pain since one month & abdominaldistension since 2 days. On enquiry, there was historyof (H/O) mild peri-umbilical colicky pain, loss ofappetite and loss of weight since one month .Therewas no H/O similar complaints in the past. There wasno H/O Tuberculosis or contact with tuberculosis. Herimmunization was complete till the age. There were nobowel or bladder disturbances.

On physical examination, she was afebrile, with pulserate of 100/min, respiratory rate was 32/min and bloodpressure was 120/60 mm of Hg. Her abdomen wasdistended with hyperperistaltic bowel sounds andthere was tenderness all over the abdomen. As theabdomen was distended and tender and the child wasnon cooperative, no mass could be appreciated onpalpation. Per rectal examination was within normallimits.

On investigating, her complete hemogram showedHb-11.7 gm%, White Blood Cell count -16700/cu mmwi th 78% neu t roph i l coun ts & P la te le tCounts-156000/cu mm.

Her X-ray abdomen was taken in erect position whichshowed multiple air fluid levels.

On Ultrasonography of abdomen and pelvis it showedmainly mesenteric lymphadenopathy and a suspiciousmass in right iliac fossa.

Patient was initially treated with conservatively withRyle’s tube aspirations, IV antibiotics, analgesics, IVfluids with vital parameters, abdominal girth,intake-output monitoring.

Since patients symptoms were not relieved, ComputedTomogram (CT) scan of abdomen was done.

Figure 1: CT scan abdomen-pelvis showingileo-ceacal intussusception

CT report showed features suggestive of ileo-ceacalintussusception with bowel within bowel appearance.As there was clinical dilemma for large ileal mass, nohydrostatic reduction was attempted and a decision toperform an exploratory laparotomy was taken.

On exploratory laparotomy, after reduction ofintussusception there was evidence of IC mass;multiple enlarged mesenteric lymph nodes. Theresection of the affected area of Ileo-caecal junctionalong with the mass was done with ilio-ascending Endto End anastomosis which was done in 2 layers withabsorbable suture material. Also, mesenteric lymphnode biopsies were taken.


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Figure 2: Intra-operative photograph showingileo-caecal intussusceptions

On gross and cut opened specimen showed a largepolypoidal mass in caecum with dilated proximal ileumand narrow ascending colon. The mass was hard andblack coloured with areas of haemorrhage andnecrosis.

Figure 3: Cut opened specimen of ileo-ascendingresection showing large polypoidal mass inileo-ceacal junction with grossly dilated proximalileum & narrowed distal ascending colon.

Figure 4: Lymphoma cells infiltrating muscularispropria [H&E X 100]

Her histo-pathology report showed High grade NonHodgkin’s Lymphoma.

An Immunohistochemistry (IHC) at Tata memorialcentre (TMC) confirmed the diagnosis of Burkitt’slymphoma. As the IHC was done at different centre,we could not get the IHC image as per their protocol.

In post operative period, patient recovered well. HerRyle’s tube was removed on 4th post operative dayand sips started. Abdominal drain was removed on 8thpostoperative day.

With HPR and IHC reports, a paediatric oncologist’sopinion was taken where she was started on IVhydration, Tab. Allopurinol and Tab. Prednisolone for aperiod of 15 days.

She was treated with pre-phase COP regime followedby MCP842 regimen.

Ki67, a marker of cellular proliferation, was 97% thusdistinguishing it from diffuse large B cell.

MCP 842 protocol includes alternating cycle A andcycle B. The drugs are adriamycin , cyclophosphsmide, cytarabine and ifosphamide with intrathecal ITMethotrexate.

As St . Jude (Murphy staging) it was stage III.Complete staging workup includes Bone marrow andCerebro-Spinal Fluid [CSF] study was done beforestarting the chemotherapy.

Patient tolerated the chemotherapy drugs well. Now,at 2 years follow up patient is doing well.






Discussion

Burkitt’s lymphoma is an uncommon type ofNon-Hodgkin Lymphoma (NHL). Burkitt’s lymphomacommonly affects children. Dennis Burkitt firstdescribed this entity in 1956 in equatorial Africa. It is ahighly aggressive type of B-cell lymphoma that oftenstarts and involves body parts other than lymph nodes.In spite of its fast-growing nature, Burkitt’s lymphomais often curable with modern intensive therapies.

In an Indian series of solid malignant tumours inchildren, Pramanik et al., in 1997, studied 263 casesover a 10-year period and found only two cases(0.76%) of BL [1,2]. Many etiologic theories have beenpostulated. The role of Epstein Barr Virus [EBV] in BLis not well understood. The virus may be a primeetiologic agent or a co-carcinogen. This viruspreferentially infects B cells via the C3d complementreceptor, CD 21. Other co-factors may includechromosomal abnormalities, immune defects, andprotein energy deficits.

Burkitt's lymphoma cells contain a reciprocalchromosomal translocation, the most frequent of whichis an 8q24; 14q32 translocation. Three subtypes of BLhave been identified [4]:

1. Endemic Burkitt's lymphoma (EBL): It is observed inAfrica. It has a peak incidence between 3-8 years ofage and the male to female ratio is 2:1. Thecommonest site of disease presentation in EBL is theface with multiple facial bone involvement. It usuallyinvolves the maxilla and mandible [3]. 2. Sporadic Burkitt's lymphoma (SBL): It is observed inwestern countries like America. The sporadic formaffects older individuals, with a mean age of 11 yearsand has no gender predilection. It is more likely tohave leukemic or bone marrow involvement and lesslikely to have jaw involvement. In SBL, the mostcommon site of presentation is the abdomen. 3. Burkitt's lymphoma in immunocompromised patientswith HIV: Burkitt's lymphoma is also known to beassociated with HIV infection. Most patients are adultswith marked immunosuppression. There is tumourpresentation both in lymph nodes and at extra nodalsites particularly in CNS, bone marrow andgastrointestinal tract.

Levine et al (1982) classified the cases of theAmerican BL as follows:

Stage I: single tumour mass (extra-abdominal 1A orabdominal 2A).

Stage II: two separate tumour masses on the sameside of the diaphragm.

Stage III: involvement of more than two separatemasses or disease on both the sides of the diaphragm.

Stage IV: pleural effusion, ascitis or involvement of thecentral nervous system (malignant cells in thecerebrospinal fluid) or bone marrow.Burkitt's lymphoma can be distinguished histologicallyand cytologically from other forms of malignantlymphomas [5,6]. Histologic sections show anundifferentiated type of B-cell lymphoma. Immaturecells 10-25 mm in diameter proliferate and haveseveral prominent nucleoli within rounded nuclei. Thesheets of tumor cells are interspersed with large palemacrophages, providing the "starry-sky" appearance,which is typical of but not unique to BL.

Immunohistochemical stains Ki-67, CD-19, CD-20,CD-22, CD-79a protein may be useful in diagnosis.

Several laboratory findings are consistently abnormalin BL patients. Serum lactate dehydrogenase (LDH) iselevated to a level corresponding to the extent of thetumour dissemination. Increased activity of alanineaminotransferase, serum alkaline phosphatase, andimmunoglobulins has been reported. Anemia andleukocytosis are common. In addition, the ESR andblood urea nitrogen (BUN) may be elevated.

The treatment strategy consists of surgery followed bychemotherapy [7]. The result of chemotherapy andimmunotherapy are excellent. Surgical debulking oflarge localized jaw or abdominal tumours is beneficialprior to chemotherapy. However there are no clear cutguidelines regarding the operation strategy and theminimum resection margins regarding our casescenario [8]. More over when situation complicateswith features of intussusception again no specificsurgery study is available like right hemi-colectomyversus ileo-ascending anastomosis as we performedin our case. Das DK et al emphasize the importance ofFNAC for intestinal lymphoma diagnosis [9].Importantis margins of resection should be clear from tumourinvolvement which can be confirmed on frozen sectionstudy. Cyclophosphamide 40 mg/kg in a singleintravenous administration and repeated about 2weeks later has given good results. Vincristine andmethotrexate are also successful in some cases. Arecent report suggests that a combination of



cyclophosphamide, vincristine, and methotrexate givebetter results than any drug used alone [10].

Radiotherapy produces excellent results but becauseof the adverse effects & complexities of immunesystem, it is largely not used. Good prognostic factorsare younger age at diagnosis, minimal tumor burdenand rapid initiation of chemotherapy, emphasizing theimportance of early diagnosis and prompt treatment.Remissions occur in more than 90% of patients withhigh-dose alkylating agent therapy as seen in thiscase. Relapse occurs in two thirds of cases, mostoften in patients with advanced stages. Withcombination chemotherapy, the overall 2-year survivalrate is 55% with a range of 80% for low-stage diseaseand 40% for the advanced stage disease.

Our case showed a complex fea tu re o fintussusception which is not observed otherwiseelsewhere in patients with Burkitt lymphoma.Considering a large mass situated in ileo-caecaljunction associated with intussusception we decided togo ahead with exploration rather than attempting ahydrostatic reduction for it or any other conservativemeasures. It was a right decision in end as the tumourmass would have never reduced to conservativereductions methods. Entire abdominal cavity was alsoexplored and findings were confirmed.

We have given our child chemotherapy after checkingher Complete Haemogram regularly to which she hasresponded quite well. We would like to report such arare case of Burkitt lymphoma presenting withileo-caecal intussusception in a small female child atour institute for which excision of tumour mass alongwith resection and end to end ileo-ascendinganastomosis was done followed by chemotherapybecause of which now the child is disease free.

References

1. Choudary VP, Agarwal RK, Marwah RK.Characteristics of Burkitt's lymphoma in India. Indian JPediatr 1987;54:859-62 2. Pramanik R, Paral CC, Ghosh A. Pattern of solidmalignant tumours in children - a ten year study. J.Indian Med. Assoc 1997;95(4);115:107-8.3. Regezi JA, Scuibba JJ, Jordan RC, editors. Oralpathology. Clinical pathological correlations. ElsevierScience: Missouri; 2003.pp. 331-3 4. Ferry JA. Burkitt's lymphoma: Clinicopathologicalfeature and differential diagnosis. Oncologist2006;11:375-83

5. Patton LL, McMillan CW, Webster WP. AmericanBurkitt's lymphoma: A 10-year review and case study.Oral Surg Oral Med Oral Pathol 1990;69:307-16 6. Lymphomas other than Hodgkin's disease. In :Wintrobe MM, Lee RE, Boggs DR, Bitell T, Athens JW,Forester J, et al . Clinical hematology. 8 th edn. Leaand Febiger; Philadelphia: 1981. pp. 1706-9 7. Bernstein JI, Coleman CN, Strickler JG et al.Combined modality therapy for adults with smallnoncleaved cell lymphoma (Burkitt's and Burkitt-liketype). J Clin Oncol1986; 4: 847–858.8. Raab N, Heller T, Kröger J, Freund M, Nizze H,Rolfs A, et al. Intestinal lymphoma. A long diagnosticpath. Med Klin (Munich) 1999;94:345–52.9. Das DK, Gupta SK, Pathak IC, Sharma SC, DattaBN. Burkitt-type lymphoma. Diagnosis by fine needleaspiration cytology. Acta Cytol. 1987;31:1–7.10. El saghir NS, Jessen K, Mass RE, et al.Combination chemotherapy for primary small intestinallymphoma in the Middle East. Eur J Cancer Oncol1989;25:851.


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