Prof. Dr. Magesh kumar UnitM 1 unit

Dr. Priya Kubendiran

Case History

H/o loss of weight/ appetite +No h/o chest pain , palpitation , syncopeNo H/o skin rash , joint pains, joint swelling

Past history :No h/o similar complaints in the past No H/o TB or contact with TB patientNo h/o jaundice in pastNot a k/c/o SHT , DM , TB , IHD ,

Personal History :Married with 2 childrenNo H/o bad obstetric historyMixed diet

Family History - nil significant

Treatment history –Had been receiving treatment from a local practitioner in the form of oral medications for the above complaints

Got admitted at Meenakshi Medical College and was started on ATT and was then referred to GSH as she didn’t show response to treatment.

O/E :She is conscious, oriented thin built , fairly hydrated , dyspneicPale , no cyanosis , clubbing,icterus,B/L pitting pedal oedema +Facial puffiness presentNo lymph node palpableNeck veins distended JVP – elevated upto 8 cm. ( CVP – 13 cm of blood

column )

Vitals : T – 99F , PR – 120 /min RR – 26 / min BP – 100/70 mm of Hg

RS – trachea shift to Rightuse of accessory muscles presentspine , shoulder ,scapulae – normaldull note on percussionreduced VF / VRabsent breath sound

b/l fine crepitations present

CVS – apical impulse not visualised/ localised muffled heart sounds

In left infra scapular,infra axillary , infra mammary region. And right infra scapular regions as compared to the rest of the lung areas.

P/A distended , umbilicus – normalflank fullness present

Liver – palpable 4 cm below right costal margin, firm , tender ,

Spleen – tip just palpable Shifting dullness present

CNS:- no fnd

ProblemsFever – low grade 15 daysB/L pleural effusion more on Left sidePericardial EffusionAscitesHepatomegaly On ATT.

Provisional diagnosis

Poly serositis for evaluation

Pericardial effusion causing right heart failure and then secondary ascites and pleural effusion

InvestigationsHb – 10.4 gm %TC – 9500 cellsDC – P68 , L30, E-

1,Platelet – 2.5 lacPCV – 31 %ESR – 42 mm / hr

RBS – 70 mg %Urea- 22 mg %Cr- 0.8 mg %

Urine examination: Sugar – nil Protein – + Pus cell – nil RBC – nil Epi cel – 2-3 / hpf

24 hrs urine protein – 150 mg/day

Urine C/S – E. coli growth

ECG

Sinus tachycardia Normal axis Low voltage complexes No electrical alternans No ST –T changes

Radiography

CXR AP VIEW

Cardiologist opinionLarge pericardial effusionThickened pericardiumFew calcific spots seenNo pericardial strands seenNo e/o diastolic collapse of RV / RANo tamponadeNormal LV systolic functionNo regional wall abnormalityTrivial TRNo PHT.

No current need for pericardiocentesisPatient to be managed conservatively

Serum levels Right Pleural Effusion

Left Pleural Effusion

Ascites

Physical - Clear straw coloured

Blood tinged Clear straw coloured

Sugar – 100 mg%

81 96 101

Protein - 5.5 gm/dl

2.4 gm/dl 5.0 gm/dl 2.9 gm/dl

Albumin – 3.5 gm --- ---

1.7 gm

Grams stain AFB

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Cell count Few lymphocytes seenNo atypical cells

Few lymphocytes seenNo atyical cells

Acellular smear

Impression Transudative Exudative Transudative

Investigations …….

LFT :Bilirubin (T) – 0.8mg %SGOT – 34 IU/LSGPT – 26 IU/LProtein – 5.5 gm/LAlbumin 3.5 gm/L

HIV – non reactiveHBs Ag - negHCV – neg

CRP – 57.14 mg/L

TSH – 0.33 mic/dlTotal T3 – 96 mic/dlTotal T4 – 7.8 mic/dl

ANA – negativeRA – negative

Pleural fluid ADA – 10 U/L

Sputum AFB – negMantoux - neg

USG Abdomen:

Liver enlarged – 17 cm , altered echotexture ,GB , Pancreas , Spleen – normal studyKidney - both kidney are of normal size , echoes

and CMD maintained with PCS - normalUterus , Ovaries ,Bladder - normal studyAscites presentPara aortic area – obscuredB/L pleural effusion present

Impression : Hepatomegaly with serous effusionto be co-related clinically.

Course in ward

As the patient had a pericardial effusion with thickened pericardium, a provisional diagnosis of subeffusive constrictive pericarditis probably tuberculous etiology was entertained

Treatment given :ATT Cat 1Oral Predisolone

Accordingly , CTS opinion was sought for favour of surgical management

CTS opinion : To get anaesthesia fitness for

pericardiotomy/pericardectomy (to avoid future tamponade/ constriction)

Anaesthesia fitness was obtained and consent taken

Surgery was done on 13/12/10

Operative notes summaryLeft anterio-lateral thoracotomy500 ml of blood stained fluid was evacuatedLarge bosselated tumor was present at the

root of the main pulmonary trunkMass was adherent to great vesselsIn view of inoperability of the tumor , incisional

biopsy was taken Pericardium anterior to the left phrenic nerve was

excisedLeft pleural space drainedPericardial drain was inserted

Intraop photograph

POST OP CXR

Postop CT Scan Chest

POST OP DIAGNOSIS

MALIGNANT PERICARDIAL EFFUSION

Biopsy reportSection studied shows a well circumscribed lesion

composed of Spindles cells, arranged in intervening fascicles and bundles and small blood vessels of the size of capillaries.

Areas of Haemorrhage and haemosederin laden macrophages seen. Focal ares of hyalinisation seen.

No giant cells, mitosis, necrosis or inflammatory infiltrate seen

IMP : f/s/o Haemangiopericytoma DD - Benign fibrous histiocytomaSuggested IHC

Immuno histo chemistry results :

AWAITED

FINAL DIAGNOSIS

PRIMARY MEDIASTINAL HAEMANGIOPERICYTOMA

With Local invasion & Malignant pericardial effusion

Further Course….

Patient improved symptomatically after the procedure

She was referred to Medical Oncology department

Advised to review with IHC reports

A 53-year-old woman complained of cough and dyspnea for months before consultation. The chest X-rays and computedtomography revealed a well-defined mass in the left middle mediastinum with necrotic changes and calcification withinit. The patient underwent left thoracotomy with tumor resection, and a final diagnosis of primary mediastinal hemangiopericytoma was made.

HEMANGIOPERICYTOMAHemangiopericytoma is one type of soft tissue

sarcoma arising from the pericytes of Zimmerman in the walls of capillaries.

Intrathoracic hemangiopericytoma usually arises from pericytes that surround the basement membrane of capillaries and small venules within the lung parenchyma.

Most common sites : limbs, pelvis, head and neck

Primary mediastinal hemangiopericytoma represents 6% of primary mediastinum tumors.

TYPES : Infantile or Adult type; Benign or malignant, low grade or high grade

Histologically : tightly packed spindle cells around ramifying thin walled and endothelial lined vascular channels ranging from small capillaries to large gaping sinusoidal spaces.

Malignant hemangiopericytoma is recognized by its increased mitotic rate, tumor size and foci of hemorrhage and necrosis

Hemangiopericytoma has no uniform clinical or radiographic features, usually affects older individuals, and mostly presents as an asymptomatic, non-calcified solitary mass on chest X-ray.

Immunohistochemically, hemangiopericytomas are known to show a positive response to antibodies against vimentin and type IV collagen and a negative response to VIII-related antigen, S-100 protein, neuron specific enolase, carcinoembryonic antigen, desmins, laminin and cytokeratins [9].

Differential diagnosis: solitary fibrous tumor

mesenchymal chondrosarcoma

synovial sarcoma fibrous histiocytoma.

Treatment : surgery, chemotherapy (vincristin, cyclophosphamide, doxorubicin, dactinomycen, methotrexate and

metoxantrone)

Spontaneous bleeding into pleura/pericardium leads to haemorragic pleural/pericardial effusion

Infiltration of major vessels makes the tumor inoperable-(complete curable resection made impossible)

Attempt to partially resect this highly vascular tumor will cause heavy blood loss

Prognosis of malignant disease is poor. Recurrence rate is high in the first 2 years. Hence , careful follow up is advised.

We thank Dept of Cardio-Thoracic

SurgeryDept of CardiologyDept of Pathology GSH , Chennai.