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Kidney tumor - adult malignancies

Other neoplasms – adult or adult/child

Hemangioblastoma

Reviewers: Sean Williamson, M.D. (see Reviewers page)
Revised: 30 June 2012, last major update June 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● Very rare in kidney (Am J Surg Pathol 2007;31:1545, Am J Surg Pathol 2010;34:1695)
● Sporadic or associated with von Hippel-Lindau disease
● Both renal cell carcinoma and hemangioblastoma are caused by loss of function of VHL tumor suppressor protein
● Usually occurs in CNS

Case reports
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● 52 year old woman with VHL syndrome and metastatic renal cell carcinoma to CNS hemangioblastoma (Arch Pathol Lab Med 2007;131:641)
● Sporadic occurrences (Am J Surg Pathol 2010;34:1695, Am J Surg Pathol 2011;35:623)

Micro description
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● Highly vascular tumor identical to CNS tumor
● Variable stromal cellularity
● May have cystic spaces
● Stromal cells have clear, foamy, lipid-containing cytoplasm or eosinophilic cytoplasm
● Variable nuclear atypia

Micro images
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CNS tumor


Site unknown


CD10-, inhibin alpha+


CD31 stains vessels

Positive stains
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● Inhibin-alpha (inhibin A) and aquaporin1 (Am J Surg Pathol 2008;32:1051), Leu7/CD57

Negative stains
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● AE1-AE3, CD10 (Mod Pathol 2005;18:788)
● PAX2, PAX8 (Am J Surg Pathol 2011;35:623)

Differential diagnosis
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● Renal cell carcinoma: AE1/AE3+, CD10+, PAX2 or PAX8 +, negative for inhibin alpha and aquaporin1

End of Kidney tumor - adult malignancies > Adult renal cell carcinoma > Hemangioblastoma

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