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Show PHOTO ESSAY Suprasellar Hemangioblastoma Shiro Miyata, MD, Takeshi Mikami, MD, Yoshihiro Minamida, MD, Yukinori Akiyama, MD, and Kiyohiro Houkin, MD FIG. 1. Postcontrast T1 coronal (A) and sagittal (B) MRI reveals a markedly enhancing suprasellar mass with multiple cysts. A lateral left carotid angiogram in the capillary phase (C) reveals a hypervascular lesion with a persistent blush that is supplied by the left superior hypophyseal artery. Intraoperative photographs (D) from the infra-chiasmal space show a vessel-rich red globular mass. Abstract: A 59-year-old woman presented with disturbance of consciousness and decreased visual acuity caused by a suprasellar mass identified on MRI. A bifrontal interhemispheric approach allowed removal of the top and lateral sides of the tumor from the wall of the third ventricle. The hypothalamus appeared to be the origin of the mass, which proved to be hemangioblastoma, a rare tumor in this location. (J Neuro-Ophthalmol 2008;28:325-326) Department of Neurosurgery, Sapporo Medical University, Sapporo, Japan. Address correspondence to Takeshi Mikami, MD, Department of Neurosurgery, Oji General Hospital, 4-8 Wakakusacho 3-chome, Tomakomai, Hokkaido 053-8506, Japan; E-mail: tmikami@ojihosp.or.jp A59-year-old woman complained of general fatigue, loss of volition, and decreased vision in July 2004 when she was admitted to our neurosurgical department. She was somnolent, and her recent memory was impaired. Karnofsky performance status was 50%. Best-corrected visual acuity was 20/300 in both eyes. MRI revealed a 3.5-cm globular lesion, with small cystic structures, that was isointense to brain parenchyma on precontrast T1 and hyperintense on T2 MRI. The tumor was located in the third ventricle and displaced the left cerebral peduncle dorsally. Postcontrast T1 MRI showed marked enhancement and the fact that the mass had a discrete border (Fig. 1A-B). Cerebral angiography demonstrated a tumor stain supplied by both superior hypophyseal arteries (Fig. 1C). Surgery was performed via the bifrontal interhemi-spheric approach. The lamina terminalis was incised above J Neuro-Ophthalmol, Vol. 28, No. 4, 2008 325 J Neuro-Ophthalmol, Vol. 28, No. 4, 2008 Miyata et al FIG. 2. Histopathology of the resected specimen shows numerous thin-walled, fairly closely packed blood vessels lined by endothelial cells, together with stromal cells containing clear cytoplasmic vacuoles (hematoxylin and eosin; original magnification: 3200). the optic chiasm, and then an easily bleeding reddish mass with a smooth surface and clear border with the surroundings was found in the third ventricle (Fig. 1D). The top and lateral sides of the tumor were easily peeled from the wall of the third ventricle. Accordingly, the hypothal-amus at the base of the third ventricle was considered the tumor origin. With repetitive coagulation, the tumor was removed piece by piece. Histopathologically, the tumor had numerous capil-lary vessels, each composed of a single layer of endothelial cells without nuclear heteromorphism (Fig. 2). The tumor had two main components of large vacuolated stromal cells and a rich capillary network. Immunostaining revealed that the tumor had endothelium-specific markers CD31 and CD34. The histologic diagnosis was hemangioblastoma. Postoperatively, the patient had no deterioration of neurologic deficits other than panhypopituitarism. MRI during the follow-up period demonstrated total removal of the tumor and no recurrence for at least the 3 years of follow-up. Hemangioblastoma is a benign tumor of uncertain histogenesis and accounts for 2.6% of all brain tumors. It is likely to occur predominantly in the cerebellar hemisphere, spinal cord, and medulla oblongata of adults 30-40 years of age (1). Hemangioblastoma arising from supratentorial locations is rare, and hemangioblastoma arising from the suprasellar region is even rarer. The most frequent symptoms of patients with suprasellar hemangioblastomas are visual and hormonal disturbances (2-9). MRI is the diagnostic study of choice (8), although angiographic study is also useful in revealing a highly vascularized lesion with a persistent blush (2). It may be difficult to histopathologically differentiate hemangioblastoma from angioblastic meningioma and metastatic renal cell carcinoma. The histologic features of these tumors are similar (2-4). Angioblastic meningioma is usually supratentorial, solid, and attached to the dura, whereas hemangioblastoma is usually cystic with a mural nodule, lacks dural attachment, and is exceedingly rare in the supratentorial region. The difficulty in distinguishing hemangioblastoma from metastatic renal cell carcinoma arises because the arrangement of its stromal cells appears epithelioid and resembles the clear cell type of renal cell carcinoma. REFERENCES 1. Bohling T, Plate KH, Haltia MJ, et al. Von Hippel-Lindau disease and capillary haemangioblastoma. In: Kleihues P, Cavanee WK, eds. Pathology and genetics. In: Tumors of the Nervous System: World Health Organization Classification of Tumors. Lyon, France: IARC Press; 2000:223-6. 2. Goto T, Nishi T, Kunitoku N, et al. Suprasellar hemangioblastoma in a patient with Von Hippel-Lindau disease confirmed by germline mutation study: case report and review of the literature. Surg Neurol 2001;56:22-6. 3. Grisoli F, Gambarelli D, Raybaud C, et al. Suprasellar hemangio-blastoma. Surg Neurol 1984;22:257-62. 4. Ikeda M, Asada M, Yamashita H, et al. A case of suprasellar hemangioblastoma with thoracic meningioma (in Japanese). No Shinkei Geka 2001;29:679-83. 5. Kouri JG, Chen MY, Watson JC, et al. Resection of suprasellar tumors by using a modified transsphenoidal approach: report of four cases. J Neurosurg 2000;92:1028-35. 6. Neumann HPH, Eggert HR, Weigel K, et al. Hemangioblastomas of the central nervous system: a 10-year study with special reference to von Hippel-Lindau syndrome. J Neurosurg 1989;70:24-30. 7. Niemela M, Lim YJ, Soderman M, et al. Gamma knife radiosurgery in 11 hemangioblastomas. J Neurosurg 1996;85:591-6. 8. Peker S, Kurtkaya Yapicier O, Sun I, et al. Suprasellar hemangio-blastoma: report of two cases and review of the literature. J Clin Neurosci 2005;12:85-9. 9. Wasenko JJ, Rodziewicz GS. Suprasellar hemangioblastoma in Von Hippel-Lindau disease: a case report. Clin Imaging 2003;27: 18-22. 326 q 2008 Lippincott Williams & Wilkins |
