Rotin D L, Shishkina L V, Shevelev I N, Zelenkov P V
Zh Vopr Neirokhir Im N N Burdenko. 2010 Apr-Jun(2):33-6; discussion 36.
We present a case report of the very rare tumor--melanotic schwannoma. Since its first description on 1932 only approximately 100 cases were reported in literature. Due to intensive pigmentation and many features that common for metastatic melanoma and melanotic schwannoma differentiation between these tumors is a challenging and important problem. 61-year-old male patient underwent surgical removal of pigmented extramedullary mass lesion at C2-C3 level. Melanotic schwannoma was diagnosed on the basis of morphological and immunohistochemical signs. Despite positive expression of so-called 'melanoma markers', (HMB-45, S-100, Melana A) cells of melanotic schwannoma also express type IV collagen and laminin which are not characteristic for melanoma. In addition, Ki-67 is very low in melanotic schwannoma, thus it confirms benign character of this tumor. Mentioned immunohistochemical profile allowed to diagnose melanotic schwannoma. It is distinctly clear that prognosis in melanotic schwannoma is incomparably better than in melanoma. Surgical removal is the best treatment option, and postoperative radiation therapy is not necessary as in melanoma.
我们报告一例极为罕见的肿瘤——黑色素性神经鞘瘤。自1932年首次被描述以来，文献中仅报道了约100例。由于色素沉着强烈以及许多特征与转移性黑色素瘤相同，区分这些肿瘤是一个具有挑战性且重要的问题。一名61岁男性患者接受了C2 - C3水平色素性髓外肿块病变的手术切除。根据形态学和免疫组化特征诊断为黑色素性神经鞘瘤。尽管黑色素性神经鞘瘤细胞表达所谓的“黑色素瘤标志物”（HMB - 45、S - 100、Melana A），但它们也表达IV型胶原蛋白和层粘连蛋白，而这些并非黑色素瘤的特征。此外，黑色素性神经鞘瘤中Ki - 67极低，这证实了该肿瘤的良性特征。上述免疫组化特征有助于诊断黑色素性神经鞘瘤。显然，黑色素性神经鞘瘤的预后比黑色素瘤好得多。手术切除是最佳治疗选择，且不像黑色素瘤那样需要术后放疗。
