Inter-hospital Conference20 March 2012

Hematology/Oncology Department of PediatricQueen Sirikit National Institute of Child Health Hospital

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Present illness

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พามิาตุรวจท� รพ.เด็ก ตุรวจร%างกาย subcostal retraction,pulsus paradoxus 20 mmHg, distant heart sound, wheezing both lungs, liver 2 cm. below RCM

Past History

• Enlargement of cardiac shadow

• CT ratio = 0.65 • No pulmonary

infiltration is seen

CXR

• CBC: Hb 14.1 g/dl, Hct 42.1%, Platelet 477,000/mm3 WBC 10,800/mm3 (N-65, L-21, E-1, Ba-1, Mo-8, ATL-4%) MCV 86.5 fl, MCH 29.5 pg/cell, MCHC 34.1 g/dl, RDW 12%

• Echocardiogram: massive pericardial effusion

Past History

• Pericardial tapping: – straw color with fibrin, WBC 850 (Mono 100%), RBC 365– Pericardial fluid Protein 2.44 g/dl, Serum Protein 6.1 g/dl – Pericardial fluid sugar 84 mg/dl, Blood Sugar 111 mg/dl– Pericardial fluid LDH 351 U/L, serum LDH 849 U/L– Pericardial fluid ADA 106, serum ADA 19 U/L– Pericardial fluid Culture: no growth, PCR for TB: negative

• Tuberculin Skin Test : negative 0 mm. • Sputum for AFB x 3days: negative

Past History

• Treat as TB pericarditis: – IRZS + Dexamethasone

• F/U Echocardiogram (1 week after treatment): – no pericardial effusion

• Continue IRZS

Past History

• Vital signs: BT 37oC, RR 28/min., PR 130/min,

BP 120/70 mmHg, Pulsus paradoxus

• BW 29 Kg.(P50-75) Ht 123 cm.(P10-25) • General Appearance: A Thai boy, good

consciousness, not pale, no jaundice, no neck vein engorged

• Heart: no active precordium, no distant heart sound, normal S1,S2, no murmur

Physical examination

• Lungs: expiratory wheezing both lungs

• Abdomen : no distention, active bowel sound, soft, liver 1 cm. below RCM, spleen was not palpable

• Extremities: no edema

Physical examination

• Previous treatment for TB pericarditis

• Progressive dyspnea• Cardiac tamponade

Problem lists

investigation

CBC• Hb 14 g/dl• Hct 40.8%• WBC 16,140/ mm3 (N-94%, L-

5%%, M-1%)• Platelet 358,000/µL• MCV 81.3 fl, MCH 28.9 pg,

MCHC 35.4 g/dl• RDW 13.5%

U/A• Sp.gr 1.005• pH 7.0• Urobilinogen : negative• Bilirubin : negative• Protein negative• Epithelial cell 0-1/HPF• WBC 1-2/HPF• No RBC

Liver Function Test• Total protein 6.18 g/dl (5.7-8.0)• Albumin 3.8 g/dl (2.9-4.2)• Globulin 2.38 g/dl (1.8-3.2)• Total bilirubin 0.51 mg/dl (< 1.00)• Direct Bilirubin 0.24 mg/dl

(<0.10)• Indirect bilirubim 0.27 mg/dl (0-

0.5)• AST / .ALT 57 / 36 U/L (10-30)• ALP 95 U/L (170-420)

Blood Chemistry

• BUN 8.05 mg/dl • Cr 0.46 mg/dl• Na 135

mmol/L• K 4.53

mmol/L• Cl 101mmol/L

• CO2 21.8 mmol/L

• Calcium 8.2 mg/dL

• Magnesium 0.83 mmol/L

• Phosphorus 6.0 mg/dl

• LDH 860 U/L• Uric acid 10.85

mg/dl

CXR• Enlargement of cardiac

shadow • Progression of BLL

infiltration, combined congestion cannot exclude

EKG

EKG• Low voltage in lead I, aVR, aVL and V1• HR 120/min• RAE, LAE, no chamber hypertrophy• Axis 90o - 120o

Bone Marrow Aspiration

Bone Marrow Aspiration

Bone Marrow Aspiration

Bone Marrow Aspiration• Clotted specimen• M : E : L = 61 : 12 : 18• Histiocyte 3%, not increased hemophagocytic

activity• Tumor cell 5%

Bone Scan

No evidence of bony metastasis

CT-Chest

CT-Chest

CT-Chest

CT-Chest • Hypodensity infiltrative mass extending from lower

neck, superior-anterior mediastinum, subcarina and hili, posterior aspected of the heart down to diaphram , encasing and compressing mediastinal structures

• Invasion into LA chamber

CTAbdomen

CTAbdomen

CTAbdomen

CT-Abdomen

• Multiple soft tissue densities in abdomen are DDx unopacified bowel loops , but cannot R/O mesenteric mass/node

Echocardiogram• RAE, LAE• Pulmonary vein obstruction due to hypertrophy of

Pulmonary vein and extracardiac mass.• PV PG 20 mmHg• Multiple mass in LA chamber, AV groove• Hyperechoic pericardium, no pericardial effusion.• LVEF 70%• Right pleural effusion 18 mm

Pathology• Pericadiectomy: Pericardium

Pathology– Suspected Malignant lymphoma– Immunohistochemistry study

• Positively react with CD3, CD5, CD7 and weekly CD4• CD10, Bcl-2, TdT are positive• MPO, CD20, CD34, CD8, CD117, PAX-5 and AE1/AE3 are

negative

T lymphoblastic lymphoma is diagnosed

Progression• Start Dexamethasone 0.6mg/kg/day• Set OR for Pericardiectomy• Patho: T lymphoblastic lymphoma stage IV• Treatment: TPOG-ALL-02-05• F/U Echocardiogram 1 mo after treatment

– No mass in cardiac chamber– Good LV function– No pericardial effusion

Approach to cardiac mass

Clinical Features• Determined by location of tumor rather than

its histological type– Rapidly progressive heart failure– Arrhythmia– Chest pain– Cardiac tamponade– Superior vena cava syndrome

Bruce C J, Heart 2011;97:151-160

Differential Diagnosis

• Primary cardiac neoplasm

• Secondary cardiac neoplasm

Bruce C J, Heart 2011;97:151-160

J Am Soc Echocardiogr, 2000;13: 1080-3

Primary cardiac neoplasm

– Assessment of the specific location• Endocardium : cardiac myxoma• Myocardium : myofibroblastic sarcoma,

fibroma, Rhabdomyoma• Pericardium: teratoma, mesothelioma,

hemangioma, Lymphoma

( Right side heart, multifocal)

Grebenc M L, et al, RSNA 2000;20: 1073-1103

Cardiac Lymphoma

RA

Secondary cardiac neoplasm

– Most common malignancies that metastasize to the heart are• Carcinomas of lung and Breast• Lymphoma• Leukemia

– Pericardium is the most commonly affected site

Grebenc M L, et al, RSNA 2000;20: 1073-1103

10-year-old boy presented with progressive breathlessness

• CXR: marked cardiomegaly• Echo:

– large pericardial effusion– Compromising function of the heart

• Bradycardia after insertion of pericardial drain, cardiac arrest and died

Patel J, et al, Annual of Oncology 2010: 21; 1041-1045

10-year-old boy presented with progressive breathlessness

Patel J, et al, Annual of Oncology 2010: 21; 1041-1045

•Patho: small lymphocytes infiltrattion of RV and LV, stained positively for CD45, CD3, CD8 and TdT

•Dx: T-cell Lymphoblastic Lymphoma

10-year-old boy presented with progressive dyspnea and abdominal pain

• CXR• Echo: massive pericardial

effusion, LV decompensation

• Pericardial tapping• Pleural tapping

– Straw-color fiuld– P/S protien ratio: 0.39– P/S LDH ratio : 0.8– Culture: nogrowth– AFB: negative

Schraader E B, et al, SAMJ 1987: 72; 878-881

10-year-old boy presented with progressive dyspnea and abdominal pain

• Start IRZS+ Prednisolone• 2 wk after treatment

Clinical improved, D/C• Readmitted 25 days after

D/C, progressive dyspnea• Pleural and pericardial

effusion – P/S protien ratio: 0.52– P/S LDH ratio : 0.48– ADA : 11.5 U/L

Schraader E B, et al, SAMJ 1987: 72; 878-881

10-year-old boy presented with progressive dyspnea and abdominal pain

• Cytology:– Numerous primitive

Lymphocytes

• CT: medistinal mass• Pericardial biopsy

– Tissue infiltration suggestive of lymphoma

Schraader E B, et al, SAMJ 1987: 72; 878-881

Conclusion• Primary cardiac lymphoma is very rare.• Both B-cell and T-cell lymphoma have been

reported• RA and RV are the most common sites• 20% of NHL presented with pleural effusion• High ADA level may be present in pleural effusion

cause by TB, SLE, Lymphoma and Leukemia

Michael G. Alexandrakis, et al, CHEST 2004;125: 1546-1555Patel J, et al, Cardiovascular Pathology, 2010;19:343-352

Patel J, et al, Annual of Oncology 2010: 21; 1041-1045