gms | German Medical Science

Objective: Hemangioblastomas are rare posterior fossa tumors which can occur sporadically or as a component tumor of von Hippel-Lindau (VHL) disease. The limited awareness of several pitfalls in the therapy of these rare tumors results in delayed or suboptimal treatment for many of these patients, especially in those with VHL disease.

Methods: We have reviewed the literature and our hemangioblastoma patient series (n= 214) for typical pitfalls and avoidable complications.

Results: The data will be presented as illustrative case reports. We observed several patients with tumor-associated cysts, who have been treated ineffectively by shunting or simply opening of the cyst. Furthermore we have frequently observed hemorrhagic complications in large solid tumors. In some patients treatment was delayed due to ineffective attempts of stereotactic radiosurgery. The delayed diagnosis of VHL in hemangioblastoma patients can result in complications by other tumors of the CNS or other organ systems.

Conclusions: Hemangioblastom-associated cysts should be treated by surgical removal of the solid tumor component. Embolization should be considered for large solid tumors and these tumors should be removed en-bloc via a wide approach. Radiosurgery is not effective for the treatment of hemangioblastomas. Screening for VHL disease should be performed in all hemangioblastoma patients. Interdisciplinary patient care and a consequent annual screening program is the key for adequate treatment of VHL patients.