Qap (Anatomical Pathology) 

QAP Peer Review

AP206
Case History: M/58. Right kidney tumor, polycythemia
Intended diagnosis: Hemangioblastoma. Contributed by:MST
Code Diagnosis Comment Score
anonymous Perivascular epithelioid cell tumour, PEComa, (70%); Juxtaglomerular cell tumour (20%); Renal cell carcinoma (10%) Perform immunstains for HMB45, melan A, renin, cytokeratin. PEComa is positive for HMB-45 and melan A, negative for renin and cytokeratin. Juxtaglomerular cell tumour is positive for renin, but negative for HMB-45, melan A and cytokeratin. Renal cell carcinoma is positive for cytokeratin.  50
anonymous Kidney: Epithelioid hemangioendothelioma nil  50
anonymous Paraganglioma (50%) / Haemangioblastoma (50%) -see comment. Immunohistochemistry S100, Chromogranin & syaptophysin etc. for confirmation of paraganglioma. Consider capillary haemangioblastoma if these immunohistochemistry negative and in setting of von Hippel-Lindau disease.  80
anonymous Hemangioblastoma nil  100
anonymous Capillary hemangioblastoma 100% Renal cell carcinoma can be excluded by negativity for cytokeratin. Capillary hemangioblastoma is positive for inhibin. It's worthwhile to screen for von Hippel-Lindau disease clinically.  100
anonymous Hemangioblastoma. (100%) nil  100
anonymous 1. Kidney - Capillary hemangioblastoma 90% 2. Kidney - Epithelioid angiomyolipoma 10% Examine more sections from thorough sampling, need to exclude renal cell CA (with hemangioblastoma-like area?). Do HMB-45 immunostain to rule out AML. Correlate with clinical history of patient - any von Hippel-Lindau syndrome?  100
anonymous Haemangioblastoma(70%) Paraganglioma (30%) Perform immunostaining for S-100 for sustenticular cell for paraganglioma and neuroendocrine markers including synaptophysin and chromogranin.  100
anonymous Haemangioblastoma 70% Angiomyolipoma 30% Need to perform HMB-45 for the diagnosis of angiomyolipoma.  100
anonymous Vascular lesion with area containing epithelioid cells, that in conjunction with clinical data of Polycythemia and specimen (kidney), would favor A) Vasculr lesion mixed with area of renal cell carcinoma; B) Epithelioid vascular neoplasm. RCC marker and CD31 are most important.  50
anonymous Right KIDNEY - clear cell RENAL CELL CARCINOMA (Fuhrman grade 2) (100%) nil  50
anonymous Hemangiomatous tumor (benign) Differential diagnoses are hemangioblastoma, hemangioma and angiomyolipoma, suggest immunohistochemistry for definitive diagnosis (CD34, CD31 and HMB45)  80
anonymous Haemangioblastoma, would perform HMB45 to exclude epithelioid angiomyolipoma 100% nil  100
anonymous Differential diagnoses: Epithelioid haemangioendothelioma (70%) Epithelioid angiomyolipoma (30%) Perform CD31 and Melan-A for confirmation. The former is positive for CD31 and the latter is positive for Melan-A.  50
anonymous Angiomyolipoma (50%), hemangioblastoma (50%) Order immunostain for HMB45, if negative favour hemangioblastoma. Hemangioblastoma of kidney is very rare. Only one case mentioned we could find. Turi S, Visy M, Vissy A, Jaszai V, Czirbesz Z, Haszon I, Szelid Z, Ferkis I. Long-term follow-up of patients with persistent/recurrent, isolated haematuria: a Hungarian multicentre study. Pediatr Nephrol. 1989  80