Solid hemangioblastoma-posterior fossa

Discussion:

The patient went to urgent surgery for the excision of the mass via the suboccipital craniectomy approach. The diagnosis of hemangioblastoma was made by histopathology. Following surgery, the patient had an uneventful recovery with improvement in her neurological symptoms. She delivered a healthy baby at 38 weeks of gestation by cesarian section. The patient had a CT scan of the neck, chest, abdomen, and pelvis, which were unremarkable. Negative family history of Von-Hipple disease. Genetic testing showed an absent mutation in the VHL tumor suppressor gene.

Cerebellar hemangioblastomas are benign WHO-grade I tumors of vascular origin. Approximately 80% of hemangioblastomas occur sporadically, with the remainder (20%) developing in patients with von Hippel-Lindau disease. Sporadic lesions are usually single and large, while VHL-associated hemangioblastomas are usually small and multiple. Hemangioblastomas may be cystic, solid, or mixed. Solid hemangioblastomas could mimic posterior fossa neoplasms, especially metastasis.

The incidence of hemangioblastoma of the posterior fossa in pregnancy is extremely low 1. Hemangioblastomas may demonstrate a rapid increase in size during pregnancy, and patients may present with features of increased intracranial pressure caused by obstructive hydrocephalus in addition to cerebellar and brainstem compressive features; hence, they may require urgent surgical therapy. Various hypotheses were proposed to explain the rapid neurological deterioration of hemangioblastoma patients during pregnancy, including rapid expansion or engorgement of the vascular bed secondary to an increase in blood volume occurring during pregnancy 2. The other hypotheses are direct hormonal effects on tumor growth rate, mediated by hormonal receptors 3.

Typical radiological features include a high T2 signal, facilitated diffusion, vivid enhancement, small intra-tumoral cysts, and surrounding prominent vessels.

DDx: solid hemangioblastomas can mimic meningiomas, metastasis, or medulloblastomas.

    Create a new playlist
Loading...