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鞍区无色素性黑素细胞瘤酷似垂体腺瘤。

Amelanotic melanocytoma of the sella mimicking pituitary adenoma.

作者信息

Lach Boleslaw, Reddy Kesava, Sommer Doron D, Whitton Anthony C, Baweja Reena

出版信息

Clin Neuropathol. 2017 Nov/Dec;36(6):283-287. doi: 10.5414/NP301033.

Abstract

We describe a 50-year-old man with a well-demarcated intra- and extrasellar lesion and clinical signs of a non-functioning pituitary adenoma. Neuropathological examination revealed tumor composed of non-pigmented spindle cells arranged in tightly packed nests separated by delicate vascular septae. There was no significant cellular atypia or mitotic activity, and Ki67-positive nuclei were present in less than 1% of cells. The neoplastic cells were positive for S-100 and vimentin, and negative for numerous cellular markers including HMB45, anti-melanoma cocktail antibodies, and Masson melanin stain. Electron microscopy revealed scattered cells with small numbers of premelanosomes, aiding in the correct diagnosis of an amelanotic melanocytoma. This is the first case report of entirely amelanotic melanocytoma of meninges, and in a very unique location. BRAF was negative supporting the diagnosis of intracranial origin of the tumor. Approximately 2 years after subtotal resection and stereotactic radiosurgery the patient is alive and well with a non-progressive residual tumor.
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摘要

我们描述了一名50岁男性,其鞍内和鞍外有界限清楚的病变,并有无功能垂体腺瘤的临床体征。神经病理学检查显示肿瘤由无色素的梭形细胞组成,这些细胞排列成紧密的巢状,由纤细的血管间隔分隔。未见明显的细胞异型性或有丝分裂活性,Ki67阳性核细胞不到1%。肿瘤细胞S-100和波形蛋白呈阳性,而包括HMB45、抗黑色素瘤混合抗体和Masson黑色素染色在内的多种细胞标志物呈阴性。电子显微镜显示散在的细胞中有少量前黑素小体,有助于正确诊断无色素性黑素细胞瘤。这是首例关于脑膜完全无色素性黑素细胞瘤的病例报告,且位于非常独特的位置。BRAF阴性支持肿瘤起源于颅内的诊断。次全切除和立体定向放射外科治疗约2年后,患者存活且情况良好,残留肿瘤无进展。

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