A Pediatric Radiology Textbook and Pediatric Radiology Digital Library
Pediatric Hemangioblastoma
Hemangioblastoma Posterior Fossa
Etiology:
Imaging MRI: — Location is 83% in cerebellum with rest in spinal cord — Location in cerebellum is 80% cerebellar hemisphere and 15% vermis and 5% medulla — Classic is cyst with mural nodule (55%) but can be solid or cystic — Nodule or solid part has marked enhancement — May have flow void — Hemorrhage occasionally into cyst — Nidus abuts pia mater with prominent associated vessels
DDX: Juvenile pilocytic astrocytoma — Age: juvenile pilocytic astrocytoma is 5-15 years old, hemangioblastoma is 30-40 years old — Pial attachment suggests hemangioblastoma — Tiny nodule and huge cyst suggests hemangioblastoma — Angiographically: hypervascular nodule in hemangioblastoma versus hypovascular juvenile pilocytic astrocytoma — Multiplicity with other findings of Von Hippel Lindau disease suggests hemangioblastoma
Complications:
Treatment:
Clinical: — Isolated or associated with Von Hippel Lindau disease — Most common posterior fossa primary neoplasm in adults — Given hemangioblastoma the incidence of Von Hippel Lindau disease is 10-20% — Given Von Hippel Lindau disease the incidence of hemangioblastoma is 35-60%
Hemangioblastoma Spinal Cord
Etiology:
Imaging MRI: — Location – 75% are intramedullary but can be intradural or extradural — Can be multiple — Can be small or fairly large — Can be associated with syrinx — Can have large feeding or draining vessels — See flow voids or adjacent cysts or hemorrhage — T1WI: Low signal intensity — T1WI post contrast: Enhances brightly — T2WI: High signal intensity
DDX:
Complications:
Treatment:
Clinical: Sporadic or associated with Von Hippel Lindau disease
Radiology Cases of Hemangioblastoma Posterior Fossa
Axial (above), sagittal (below left) and coronal (below right) T1 MRI with contrast of the brain shows a round, solid, avidly enhancing lesion in the superior aspect of the left cerebellar hemisphere.
