Management of Infratentorial Hemangioblastomas in Children

Initial Management at Presentation

• Surgery preferred for single lesions: Surgery is the best treatment for an isolated hemangioblastoma since it is curative due to the benign behavior of the tumor.

• Surgery indicated for symptomatic or growing lesion: Surgery is indicated for treatment of symptomatic and/or enlarging isolated hemangioblastomas, or even for incidentally discovered hemangioblastoma to prevent tumor growth and/or occurrence of symptoms. In patients with VHL, surgical treatment is proposed in cases of large, evolving hemangioblastomas. Indeed, these patients usually harbor multiple hemangioblastomas that are prone to recur and/or to develop at different sites. Clinical and radiological monitoring is adopted in these instances.

• Surgery can be needed for hydrocephalus or hemorrhage: Surgery is required to manage associated hydrocephalus or possible tumor complications (e.g., tumoral hemorrhage).

Adjunctive Therapies

• Radiotherapy: Conventional radiation and stereotactic radiation both have been used as treatment alternatives when surgery is deemed not possible (2, 4, 34).

• Bevacizumab: There is a case report describing the use of antiangiogenesis therapy to halt the growth of inoperable hemangioblastomas (31).

Follow-up

• Screening for VHL: All patients with diagnosed/treated hemangioblastomas should be screened for VHL.

• Patients with isolated hemangioblastomas: Patients with isolated hemangioblastomas are followed by serial clinical evaluations and MRI (usually 3 times/year for the first year after surgery, then yearly for at least 5 years).

• Patients with VHL and multiple hemangioblastomas: Patients with VHL require the same postoperative follow-up as do patients with isolated hemangioblastomas but for the duration of their life.  They have an additional need to be followed for their risk for occurrence of other types of neoplasms associated with the disease.