Hemangioblastoma in the pineal region : Case report

• Published in: Neurosurgery Vol. 61; no. 2; pp. E423; discussion E423 - E423
• Main Authors: ISOLAN, Gustavo R, KRAYENBÜHL, Niklaus, MAHMOUD, Mohamed, AL-MEFTY, Ossama, BRICOLO, Albino, DE TRIBOLET, Nicolas, BRUCE, Jeffrey N
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD Lippincott Williams & Wilkins 01.08.2007; Wolters Kluwer Health, Inc
• Subjects: Biological and medical sciences; Biopsy; Female; Hemangioblastoma - pathology; Hemangioblastoma - surgery; Humans; Magnetic Resonance Imaging; Medical sciences; Middle Aged; Nervous system; Neurosurgery; Neurosurgical Procedures; Pinealoma - pathology; Pinealoma - surgery; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Tumors; Nervous system diseases; Phacomatosis; Angioblastoma; von Hippel-Lindau disease; Cardiovascular disease; Hemangioblastoma; Genetic disease; Vascular disease; Case study; Pineal region; Eye disease; Surgery; Central nervous system disease; Tumor; Von Hippel Lindau disease
• ISSN: 0148-396X; 1524-4040
• DOI: 10.1227/01.NEU.0000255516.12085.BB

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. 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. 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. 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.