Transcript Tutorial for Oncology Brain tumor

Inter-hospital Conference
20 March 2012
Hematology/Oncology Department of Pediatric
Queen Sirikit National Institute of Child Health Hospital
ผู้ป่วยเด็กชายไทย อายุ 8 ปี
ภูมลิ าเนา จ.ปทุมธานี
หายใจเหนื่อยมากขึน้
2 วัน ก่ อนมา รพ.
Present illness
4 วัน ก่ อนมา รพ. สังเกตุว่าเหนื่อยง่ าย ไม่ มีไข้
ไอแห้ งๆ นอนราบได้
2 วัน ก่ อนมา รพ. หายใจเร็วมากขึน้ ดูเหนื่อย บางครั ง้
มีเสียงหายใจดังเหมือนนกหวีด
ไอแห้ งๆ ท้ องอืดมากขึน้ ไม่ มีไข้
จึงมา รพ.
Past History
2 เดือน ก่ อนมา รพ. เหนื่อยง่ ายขึน้ เดินแล้ วต้ องนั่งพัก
ไม่ ไอ ไม่ มีไข้ นั่งเรียนแล้ วหมดสติไป
พามาตรวจที่ รพ.เด็ก ตรวจร่ างกาย subcostal
retraction,pulsus paradoxus 20 mmHg,
distant heart sound, wheezing both
lungs, liver 2 cm. below RCM
CXR
• Enlargement of cardiac
shadow
• CT ratio = 0.65
• No pulmonary
infiltration is seen
Past History
• 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:
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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
Physical examination
• 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
Problem lists
• Previous treatment for TB pericarditis
• Progressive dyspnea
• Cardiac tamponade
investigation
CBC
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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
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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
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Total protein
Albumin
Globulin
Total bilirubin
Direct Bilirubin
Indirect bilirubim
AST / .ALT
ALP
6.18 g/dl
3.8 g/dl
2.38 g/dl
0.51 mg/dl
0.24 mg/dl
0.27 mg/dl
57 / 36 U/L
95 U/L
(5.7-8.0)
(2.9-4.2)
(1.8-3.2)
(< 1.00)
(<0.10)
(0-0.5)
(10-30)
(170-420)
Blood Chemistry
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BUN 8.05 mg/dl
Cr 0.46 mg/dl
Na 135 mmol/L
K 4.53 mmol/L
Cl 101 mmol/L
CO2 21.8 mmol/L
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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
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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
CT
Abdomen
CT
Abdomen
CT
Abdomen
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
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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
•Patho: small lymphocytes infiltrattion
of RV and LV, stained positively for
CD45, CD3, CD8 and TdT
•Dx: T-cell Lymphoblastic Lymphoma
Patel J, et al, Annual of Oncology 2010: 21; 1041-1045
10-year-old boy presented with
progressive dyspnea and abdominal pain
• CXR
• Echo: massive
pericardial effusion,
LV decompensation
• Pericardial tapping
• Pleural tapping
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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: 1546-1555
Patel J, et al, Cardiovascular Pathology, 2010;19:343-352
Patel J, et al, Annual of Oncology 2010: 21; 1041-1045