Hemangioblastomas of the posterior cranial fossa in adults: demographics, clinical, morphological, pathological, surgical features and outcomes. A systematic review.

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Hemangioblastomas of the posterior cranial fossa in adults: demographics, clinical, morphological, pathological, surgical features and outcomes. A systematic review.

World Neurosurg. 2017 Dec 08;:

Authors: Kuharic M, Jankovic D, Splavski B, Boop FA, Arnautovic KI

Abstract
BACKGROUND: Posterior cranial fossa (PCF) hemangioblastomas are benign, highly vascularized, and well-differentiated tumors with well-described histopathologic features. Although relatively rare, this tumor is the most prevalent primary tumor of the cerebellum in adults.
OBJECTIVE: Since the demographics of patients with such a tumor-as well as the clinical, morphological, pathological, surgical features, and outcomes-are not fully understood, we systematized characteristic patient and tumor features.
METHODS: We undertook a systematic review of the English-language literature in PubMed for PCF hemangioblastomas in adults published in the past 31 years. We analyzed geographic distribution and year of articles' publication; demographic data of patients; presenting symptoms and clinical signs; tumor location and morphology; histopathological features, extent of tumor resection, perioperative blood loss, and postoperative complications; length of hospital stay; and outcomes.
RESULTS: We reviewed 207 articles describing 1,759 infratentorial hemangioblastomas in a cohort of 1,515 adult patients. We found female predominance in patients with Von Hippel-Lindau disease (VHLD) compared with male predominance in the general patient group. Symptoms of intracranial hypertension were more common in the VHLD group compared with the general group of patients. The cerebellar location was more common in the VHLD group while solid (parenchymatous) tumor was the most common type. The vast majority of patients underwent total resection but rate of resection did not differ between the general and VHLD groups. Most patients had a favorable outcome.
CONCLUSIONS: The literature of adult PCF hemangioblastomas is limited and general surgical experience with such tumors is scarce due to their rarity. Rates of postoperative complications and mortality still remain higher than expected. In spite of that, prognosis and surgical outcomes are generally favorable. Nevertheless, surgery of adult PCF hemangioblastomas is a demanding and challenging task.

PMID: 29229339 [PubMed - as supplied by publisher]

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