de Tella O I, Agner C, Aguiar P H, Herculano M A, Prandini M N, Stavile J N
Department of Neurosurgery, Federal University of Sao Paulo, Sao Paulo, Brazil.
Acta Neurochir (Wien). 2003 Dec;145(12):1121-6. doi: 10.1007/s00701-003-0121-3. Epub 2003 Oct 13.
Meningeal melanocytoma generally occurs in the posterior fossa. Orbital manifestation is rarely encountered.
A thirty-five year-old man presented with progressive proptosis of his right eye. Computed tomography (CT) and Magnetic Resonance Imaging (MRI) of the brain showed an expansive intraconal mass lesion occupying the superior orbital compartment, the entire orbital apex, and the optic foramen. Histological analysis and Immunohistochemical staining for S-100 and HMB-45 monoclonal antibodies confirmed melanocytoma.
Microsurgical removal was accomplished through a fronto-orbital craniotomy. Chemotherapy and irradiation followed the initial intervention. The patient returned for follow up two years after surgery, complaining of headache and right visual loss. A subfrontal tumor with massive edema was found on follow up CT scan.
Meningeal melanocytomas are rare benign pigmented tumors of the central nervous system. They are predominant in the posterior fossa and spinal cord and frequently mistaken for melanomas, especially on frozen sections. Orbital presentation is rare. The natural history is poorly defined.
脑膜黑素细胞瘤通常发生于后颅窝。眼眶表现罕见。
一名35岁男性出现右眼进行性眼球突出。脑部计算机断层扫描(CT)和磁共振成像(MRI)显示眶内有一占位性肿块,占据眶上间隙、整个眶尖和视神经管。组织学分析以及针对S-100和HMB-45单克隆抗体的免疫组织化学染色证实为黑素细胞瘤。
通过额眶开颅术完成了显微手术切除。初次干预后进行了化疗和放疗。患者术后两年复诊,主诉头痛和右眼视力丧失。随访CT扫描发现额下有一伴有大量水肿的肿瘤。
脑膜黑素细胞瘤是中枢神经系统罕见的良性色素性肿瘤。它们主要发生于后颅窝和脊髓,常被误诊为黑色素瘤,尤其是在冰冻切片时。眼眶表现罕见。其自然病程尚不明确。