Case 5 15-year-old male

Transcription

1 Case 5 15-year-old male Present illness: Six months ago, abnormality of ECG was incidentally detected by annual health check. His blood level of γ-gtp, HbA1c and norepinephrine were elevated; however, definite diagnosis was not obtained by MIBG scintigraphy. Four months ago, surface irregularity of the liver and moderate amount of ascites were detected by US in another hospital. Abdominal dynamic CT and detailed blood tests did not reveal a definite diagnosis. One month ago, he visited an emergency room with complaints of a cough and back pain. Under a diagnosis of pneumonia, antibacterial therapy was performed. However, his symptoms were not improved, resulting in an admission to our hospital.

2 Initial findings BP 108/60 P 88 RR18 BT 36.8 Blood test WBC Hb 15.8 Hct 47.1 Plt 22.7 TP 7.1 Alb 3.0 BUN 10 CRE 0.49 T-Bil 3.8 D- Bil 2.0 AST 22 ALT 12 ALP 225 LDH 275 HbA1c 6.0 PT% 36.4 APTT 32 D-dimer 11.5 FDP 42 Fibrinogen 269 ESR 31 CRP 6.13 sil-2r 471 HBV(-) HCV(-) AFP 1 PIVKA-Ⅱ17 IgG 2068

3 Presented Images : Initial CT (6 months ago) : Follow-up CT (1 month ago) : Follow-up CT and FDG-PET/CT (at an admission to our hospital)

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10 Heart: Imaging findings Diffuse hypertrophy of the pericardium. Pericardial effusion. Disability of cardiac expansion. Aneurysmal protrusion of the right ventricle. Vein: Distention of the SVC and IVC. Counter current of contrast media to the right hepatic vein. Thrombi in the left internal jugular and brachial cephalic veins.

11 Lung and lymph nodes: Bilateral peripheral lung nodules/ consolidations. Mediastinal lymphadenopathy. Pleural effusion (w/ massive ascites). Liver: Surface irregularity and swelling. Multiple mottled enhancement in the liver. FDG-PET/CT: Strong FDG uptakes (pericardial mass and mediastinal LNs).

12 Speculated pathophysiology Constrictive pericarditis. Diastolic dysfunction due to diffuse hypertrophied mass of the pericardium. Massive venous congestion. Venous thromboses. Congestive liver / cirrhosis. Lung infarctions (probable).

13 Differential diagnosis Pericardial mesothelioma Pericardial lymphoma Tuberculous pericarditis Surgical biopsy of the pericardium was performed.

14 Pathological findings H&E. Biphasic tumor growth was shown as epithelioid and sarcomatoid patterns.

15 WT1 calretinin D2-40 Positive for all three mesothelial markers in immunohistochemical stainings.

16 AE1/AE3 CAM5.2 Positive for epithelial markers also in the sarcomatoid lesions.

17 Pathological diagnosis Malignant pericardial mesothelioma Subtype: biphasic type

18 Malignant pericardial mesothelioma Etiology: 0.002% in autopsy. 1%> in all mesotheliomas (pleura 88.8%, peritoneum 9.6%, pericardium 0.7%, tunica vaginalis 0.2%). Extremely rare but most common in pericardial malignancies years (mean 48 y, range 2-76 y), Dominant in male. Risk Factor: Exposure of asbestos, radiation and thorotrast. Infection of Tb and SV40 virus. No clear correlation b/w asbestos exposure and pericardial mesothelioma.

19 Clinical Presentation: Cardiac enlargement, recurrent pericardial effusion, conduction disturbance, and non-specific symptoms related to constrictive pericarditis. Diagnosis: Only 20% of the cases revealed by pericardiocentesis. Pathological Subtype: epithelial 54%, sarcomatoid 17%, biphasic 29% Prognosis: 6 week-15 month survival after onset.

20 Imaging Findings diffuse pericardial hypertrophy pericardial effusion pulmonary embolism(14%) metastasis: L/Ns(50%), liver(13%) congestive hepatomegaly, cirrhosis* venous thrombosis* lung infarction* Differential Diagnosis pericardial lymphoma tuberculous pericarditis sarcoma (angiosarcoma, synovial sarcoma, et al.) * Seen only in the current case.

21 Differential diagnosis of constrictive pericarditis In developed countries Idiopathic Secondary to cardiac surgery In developing countries Tuberculosis Miscellaneous Connective tissue disorder Sarcoidosis Malignancy Asbestosis Medication Uremic pericarditis

22 Tuberculous pericarditis Only accounts for 0.5% of extra-pulmonary tuberculosis. Risk factors: cardiovascular surgery and chest radiations, HIV. Mechanism: usually develop by retrograde lymphatic spread from peritracheal and peribronchial nodes. Manifestation: pericardial effusion, constrictive pericarditis. Initially, involves an effusive state, acute pericardial effusion. The majority patients recover spontaneously. Some patients go on to a chronically constrictive state with pericardial thickening and calcification.

23 Primary cardiac lymphoma Rare subset arising from a heart or pericardium. *distinct from primary effusional lymphoma, associated with HHV-8 and HIV. Frequent in RA ( sometimes invade to LV), ranged from limited invasion of the myocardium with intrapericardial extension, bulk intrapericardial mass, regional L/N swellings. Tumor size (median 47mm, ) Manifestation: Cardiac tamponade, arrhythmia and SVC syndrome. Favorable prognosis.

24 Final diagnosis Malignant pericardial mesothelioma complicated with venous thrombosis, lung infarction and congestive liver, cirrhosis

25 References 1. Fernandes R, et al. BMJ Case Rep doi: / bcr Patel J, et al. Cardiovascular Pathology. 2011;20: Nilsson A, et al. Case Rep Oncol. 2009;2(2): Restrepo CS, et al. Radiographics. 2013;33(6): Oc M, et al. Bratisl Lek Listy. 2012;113(10): Bendek M, et al. Cardiovasc Pathol. 2010;19(6): Sardar MR, et al. Tex Heart Inst J. 2012;39(2): Soussan M, et al. Insights Imaging. 2012;3: Carras S, et al. Hematol Oncol doi: /hon.2301.