Hemangioblastoma

KEY FACTS

TERMINOLOGY

• Hemangioblastoma (HGBL): Highly vascular tumor of adults

most commonly found in posterior fossa (PF), spinal cord

○ 25-40% of HGBLs in von Hippel-Lindau disease (VHL)

IMAGING

• Best diagnostic clue: Adult with cerebellar mass with cyst

and enhancing mural nodule

• General features

○ 50-60% cyst + "mural" nodule; 40% solid enhancing mass

○ 90-95% in posterior fossa

○ 5-10% supratentorial (around optic pathways,

hemispheres; usually in VHL)

TOP DIFFERENTIAL DIAGNOSES

• Metastasis (most common posterior fossa parenchymal

mass in adults)

• Pilocytic astrocytoma (most common neoplasm with cyst +

nodule in child)

• Cavernous malformation

• Hereditary hemorrhagic telangiectasia

PATHOLOGY

• WHO grade I

• Red or yellowish, well-circumscribed, unencapsulated,

highly vascular mass that abuts leptomeninges

• Histology shows stromal cells, innumerable small vessels

CLINICAL ISSUES

• Headache is most common presenting symptom

• Primary therapy = surgical resection

• Begin MR screening of patients from VHL families after 10

years of age

DIAGNOSTIC CHECKLIST

• Most common posterior fossa intraaxial mass in middleaged/older adult is metastasis, not HGBL

(Left) Coronal graphic shows a

classic cerebellar

hemangioblastoma as largely

cystic mass ﬈ with a vascular

tumor nodule ﬇ that abuts

the pial surface. These lowgrade tumors are the most

common primary posterior

fossa tumor in adults. (Right)

Coronal T1 C+ MR shows a

large cyst and mural nodule

ſt in a VHL patient with

multiple hemangioblastomas.

Note the lack of enhancement

﬈ of the cyst wall, which

consists of compressed but

normal cerebellum. The other

hemangioblastomas ﬇ have a

solid enhancing appearance.

(Left) Coronal T2 MR in a 60-

year-old woman with

headaches shows a

heterogeneous cerebellar

mass ſt with prominent flow

voids ﬊ and surrounding

edema. (Right) Coronal T1 C+

MR in the same patient shows

marked enhancement of the

mass ﬇ and a prominent

adjacent vessel ſt.

Hemangioblastoma was found

at surgical resection. The main

imaging differential diagnosis

in this adult was metastatic

disease. These WHO grade I

tumors have an excellent

prognosis in sporadic cases.

Brain: Pathology-Based Diagnoses: Neoplasms,

Cysts, and Disorders

146