Department of Neurosurgery, The Royal Melbourne Hospital, Grattan Street, Parkville 3050, Victoria, Australia.
J Clin Neurosci. 2011 May;18(5):702-4. doi: 10.1016/j.jocn.2010.09.006. Epub 2011 Feb 26.
Gliosarcoma is a rare glioblastoma variant, classically arising in the cerebral hemispheres. We report a patient with primary diffuse leptomeningeal gliomatosis (PDLG) with a sphenoid sinus and sellar mass. An 84-year-old woman presented with progressive headache and right-sided visual failure, associated with ipsilateral oculomotor nerve palsy and left temporal field loss. Neuraxial MRI showed a large lesion within the sphenoid sinus and sella resulting in chiasmal compression, and diffuse cranial and spinal leptomeningeal enhancement. Endoscopic transphenoidal biopsy and debulking of the sphenosellar lesion was performed, and gliosarcoma was diagnosed on histopathological examination. The patient was palliated due to poor performance status. To our knowledge, this is the only report of gliosarcoma within the paranasal sinuses and the second report of PDLG where the histological analysis has confirmed gliosarcoma. We believe this adds significant weight to the theory that heterotopic nests of glial tissue, in this instance within the sphenoid or sella, are the putative origin of PDLG.
胶质肉瘤是一种罕见的胶质母细胞瘤变体，经典地发生在大脑半球。我们报告了一例蝶窦和鞍区肿块的原发性弥漫性软脑膜胶质瘤病（PDLG）患者。一名 84 岁女性因进行性头痛和右侧视力丧失就诊，伴有同侧动眼神经麻痹和左侧颞叶视野丧失。脊柱 MRI 显示蝶窦和鞍内有大病变导致视交叉受压，并弥漫性颅颈软脑膜增强。进行了经蝶窦内镜活检和蝶鞍区病变切除术，并在组织病理学检查中诊断为胶质肉瘤。由于患者的一般状况较差，故进行姑息治疗。据我们所知，这是首例报告发生在副鼻窦的胶质肉瘤，也是第二例报告经组织学分析证实为胶质肉瘤的 PDLG。我们认为，这为异位胶质组织巢的理论提供了有力证据，在这种情况下，这些胶质组织巢位于蝶窦或鞍内，是 PDLG 的潜在起源。
