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Presented by George Netto, M.D. and prepared by Carla Ellis, M.D.
Case 3: A 44 year old woman complaining of headaches was found to have a posterior fossa mass measuring 1.2 cm.
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1. Question
Week 441: Case 3
A 44 year old woman complaining of headaches was found to have a posterior fossa mass measuring 1.2 cm.images/1Alex/07042010case3image3.jpg
images/1Alex/07042010case3image4.jpgCorrect
Answer: Hemangioblastoma
Histology: The tumor is characterized by proliferation of capillaries with variable sized, closely packed, thin walled vessels and large neoplastic stromal cells with pink to clear foamy cytoplasm with fine vacuoles containing PAS+ lipid. Nuclei are hyperchromatic and there is usually no atypia, no fibrillar cells, no necrosis and no/rare mitotic figures.
Discussion: Hemangioblastomas accounts for up to 2.5% of all intracranial tumors and may occur sporadically or as a part of the multi-system genetic syndrome Von Hippel-Lindau disease (VHL).
Approximately 25% of hemangioblastomas occur as a part of VHL, which is caused by the inherited mutation of the VHL gene on chromosome 3p25-26. Patients with VHL are also at increased risk of developing clear cell renal cell carcinoma (cRCC).
Distinguishing hemangioblastomas from metastatic cRCC to the central nervous system (CNS) can be challenging on routine hematoxylin and eosin (H&E) stained sections. We recently demonstrated the utility of a binary immunohistochemistry panel combining PAX2 and inhibin as a helpful approach to distinguishing the two lesions. PAX2(-)/inhibin(+) profile is exhibited by hemangioblastomas while Metastatic cRCC to CNS generally demonstrate a PAX2(+)/inhibin(-) immunoprofile. A subset of hemangioblastoma can be PAX2(-)/inhibin(-).
Reference(s):
– Banerjee P, Albadine R, Sharma R, Burger P, Netto GJ. Pax-2(-)/inhibin(+) immunoprofile in hemangioblastoma: a helpful combination in the differential diagnosis with metastatic clear cell renal cell carcinoma to CNS: Mod Pathol 2009; 22(S1):327A.Incorrect
Answer: Hemangioblastoma
Histology: The tumor is characterized by proliferation of capillaries with variable sized, closely packed, thin walled vessels and large neoplastic stromal cells with pink to clear foamy cytoplasm with fine vacuoles containing PAS+ lipid. Nuclei are hyperchromatic and there is usually no atypia, no fibrillar cells, no necrosis and no/rare mitotic figures.
Discussion: Hemangioblastomas accounts for up to 2.5% of all intracranial tumors and may occur sporadically or as a part of the multi-system genetic syndrome Von Hippel-Lindau disease (VHL).
Approximately 25% of hemangioblastomas occur as a part of VHL, which is caused by the inherited mutation of the VHL gene on chromosome 3p25-26. Patients with VHL are also at increased risk of developing clear cell renal cell carcinoma (cRCC).
Distinguishing hemangioblastomas from metastatic cRCC to the central nervous system (CNS) can be challenging on routine hematoxylin and eosin (H&E) stained sections. We recently demonstrated the utility of a binary immunohistochemistry panel combining PAX2 and inhibin as a helpful approach to distinguishing the two lesions. PAX2(-)/inhibin(+) profile is exhibited by hemangioblastomas while Metastatic cRCC to CNS generally demonstrate a PAX2(+)/inhibin(-) immunoprofile. A subset of hemangioblastoma can be PAX2(-)/inhibin(-).
Reference(s):
– Banerjee P, Albadine R, Sharma R, Burger P, Netto GJ. Pax-2(-)/inhibin(+) immunoprofile in hemangioblastoma: a helpful combination in the differential diagnosis with metastatic clear cell renal cell carcinoma to CNS: Mod Pathol 2009; 22(S1):327A.
