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Presented by Fred Askin, M.D. and prepared by Natasha Rekhtman, M.D., Ph.D.
Case 3: The patient is a 58 year-old male with ataxia and cerebellar lesion.
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1. Question
Week 231: Case 3
The patient is a 58 year-old male with ataxia and cerebellar lesion. The patient has a previous history of renal cell carcinoma.images/7 15 05 case 3 1.jpg
images/7 15 05 case 3 2.jpg
images/7 15 05 case 3 3.jpg
images/7 15 05 case 3 4.jpgCorrect
Answer: Cerebellar hemangioblastoma
Histology: Capillary hemangioblastoma is characterized by large vacuolated stromal cells and a very rich capillary network. The vacuolated stromal cells represent the neoplastic tumor component of the tumor and the vacuoles are composed of lipid which can be demonstrated by Oil-Red-O (ORO) stain on frozen sections.
Discussion: This case has at least two important points: The problem in separating cerebellar hemangioblastoma from metastatic renal cell carcinoma (EMA and cytokeratin immunostains facilitate this differential diagnosis) and secondly, the recognition of von Hippel-Lindau Disease (VHL). It is inherited in an autosomal dominant fashion and is related to germ-line mutations of the VHL tumor suppressor gene located on chromosome 3p 25-26. Other extracranial manifestations of VHL Disease include Feochromocytoma, Islet Cell tumors in the pancreas, Endolymphatic Sac Tumors of the inner ear, and a variety of cysts in different organs.
Reference(s):
– Kleihues P, Cavenee WK, Editors, Pathology and Genetics of Tumors of the Nervous System, IARC Press, Lyon, France, 2000.Incorrect
Answer: Cerebellar hemangioblastoma
Histology: Capillary hemangioblastoma is characterized by large vacuolated stromal cells and a very rich capillary network. The vacuolated stromal cells represent the neoplastic tumor component of the tumor and the vacuoles are composed of lipid which can be demonstrated by Oil-Red-O (ORO) stain on frozen sections.
Discussion: This case has at least two important points: The problem in separating cerebellar hemangioblastoma from metastatic renal cell carcinoma (EMA and cytokeratin immunostains facilitate this differential diagnosis) and secondly, the recognition of von Hippel-Lindau Disease (VHL). It is inherited in an autosomal dominant fashion and is related to germ-line mutations of the VHL tumor suppressor gene located on chromosome 3p 25-26. Other extracranial manifestations of VHL Disease include Feochromocytoma, Islet Cell tumors in the pancreas, Endolymphatic Sac Tumors of the inner ear, and a variety of cysts in different organs.
Reference(s):
– Kleihues P, Cavenee WK, Editors, Pathology and Genetics of Tumors of the Nervous System, IARC Press, Lyon, France, 2000.
