Hemangioblastoma (WHO Grade 1)
Last edit by Alaric Steinmetz on
Synonyms: Hemangioblastoma
ICD-10: D33.0, D33.1, D33.2, D33.4, D33.7, D33.9
Hemangioblastomas are vascular neoplasms of the central nervous system and occur primarily in young adults. They are benign tumors located predominantly in the Cerebellum and are classified as Grade 1 tumors according to the WHO classification.
WHO Classification
According to the 2021 WHO classification, hemangioblastomas are classified as WHO Grade 1 tumors[^1].
Epidemiology
Hemangioblastomas are the most common primary brain tumors in the posterior cranial fossa in adults[^2]. Hemangioblastomas account for approximately 1.5 % to 2.5 % of all intracranial tumors and 7 - 8 % of all tumors of the posterior cranial fossa[^5].
Symptoms
The most common symptoms of hemangioblastomas of the posterior cranial fossa are attributable to increased intracranial pressure, followed by cerebellar symptoms[^5].
Imaging
The gold standard for imaging hemangioblastomas is MRI imaging. digital subtraction angiography frequently demonstrates a strongly vascularized nodule with feeding vessels, which is suggestive of a hemangioblastoma.
Associated Diseases
In 25-40 % of cases, hemangioblastomas are diagnosed in patients with von Hippel-Lindau disease[^3]. In patients with von Hippel-Lindau disease, multiple hemangioblastomas may occur both cranially and spinally.
Pregnancy
In pregnant patients, there is a risk of rapid progression in hemangioblastoma size due to pregnancy[^4].
Treatment
Complete extirpation of the mural tumor nodule is critical in hemangioblastomas. Resection of the cyst wall is generally not necessary, as it usually consists of compressed brain tissue without tumor cells.
