Hemangioblastoma

Steven H. Lin and Ori Shokek

Background

What is the typical age of presentation for hemangioblastoma?

20–50 yrs is the typical age of presentation for hemangioblastoma (primarily in young adults).

Where do most hemangioblastomas arise anatomically?

Hemangioblastomas arise in the cerebellum. They are the most common primary cerebellar tumor in adults.

What genetic disorder is associated with hemangioblastomas?

Von Hippel-Lindau (VHL; hemangioblastomas, pancreatic/renal cysts, renal cell carcinoma)

Are hemangioblastomas benign/low grade or malignant/high grade?

Benign/low grade (WHO grade 1)

What is the cell of origin or hemangioblastomas, and what is the associated pathology?

Endothelial stem cells; closely packed vascular lesions with a stroma of large oval “foamy” cells

The # of lesions seen in hemangioblastomas correlates with what in terms of etiology?

Single lesion (sporadic, older pts) vs. multiple lesions (familial, younger pts)

What hematologic abnormality is present in pts with hemangioblastomas? Why?

Polycytemia is present because of erythropoietin production by the tumor.

How do hemangioblastomas cause morbidity if not treated?

Local compression and hemorrhage

What are common Sx of hemangioblastoma at presentation?

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Feb 12, 2017 | Posted by admin in ONCOLOGY | Comments Off