A HEMANGIOBLASTOMA IN THE PINEAL REGION

Author:

Isolan Gustavo R.1,Krayenbühl Niklaus1,Mahmoud Mohamed1,Al-Mefty Ossama1

Affiliation:

1. Department of Neurosurgery, University of Arkansas for Medical Sciences, Little Rock, Arkansas

Abstract

Abstract OBJECTIVE Although reported to occur throughout the central nervous system, hemangioblastomas are principally found in the cerebellum and spinal cord. Pineal region tumors comprise approximately 1% of central nervous system neoplasms. A wide variety of tumors can affect this region, the most common being germ cell tumors, gliomas, and pineal cell tumors. In the literature, we found only one case of hemangioblastoma in the pineal region in association with von Hippel-Lindau disease. CLINICAL PRESENTATION We describe the case of a patient with a symptomatic hemangioblastoma in the pineal region with no clinical criteria for von Hippel-Lindau disease. The patient had a 1-month history of short-term memory loss, headache, difficulty concentrating and writing, disturbed balance, and loss of bladder function. At the time of physical examination, she was awake, alert, and oriented. An ophthalmoscopic examination revealed nystagmus with conjugate upward gaze and papilledema. Radiological images showed a mass in the pineal region with obstructive hydrocephalus. INTERVENTION A lateral suboccipital infratentorial supracerebellar approach was used to remove the tumor, which was attached to the quadrigeminal plate. Histological examination showed the lesion to be a hemangioblastoma. The clinical findings for von Hippel-Lindau disease were negative. CONCLUSION The patient's neurological deficits were reversed after surgery. This case emphasizes the importance of the differential diagnosis of hemangioblastomas located in this region. These tumors can be safely removed through surgery.

Publisher

Ovid Technologies (Wolters Kluwer Health)

Subject

Clinical Neurology,Surgery

Reference13 articles.

1. Hemangioblastomas of the central nervous system: Molecular genetic analysis and clinical management;Catapano;Neurosurgery,2005

2. Hemangioblastomas of the central nervous system in von Hippel-Lindau syndrome and sporadic disease;Conway;Neurosurgery,2001

3. Risk of hemorrhage in hemangioblastomas of the central nervous system;Gläsker;Neurosurgery,2005

4. Cryptic von Hippel-Lindau disease: Germline mutation in patient with hemangioblastoma only;Hes;J Med Genet,2000

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