Saint-Blancard P, Goasguen O, Kossowski M, Dulou R
Service d'anatomie pathologique, hôpital d'instruction des armées Percy, 101, avenue Henri-Barbusse, 92140 Clamart, France.
Rev Med Interne. 2008 Jul;29(7):587-90. doi: 10.1016/j.revmed.2007.12.010. Epub 2008 Feb 13.
Schwannomas are common ubiquitous benign tumours of the nervous sheaths. Among the various histopathological types, the pigmented schwannoma has to be distinguished from melanoma. We report a case of melanotic schwannoma of the cerebellopontine angle in a 52-year-old man with a slowly progressive cerebellar syndrome since 1978, who presented with a posterior cerebral fossa bleeding. Melanotic schwannoma is a rare form of schwannoma. The diagnosis remains difficult and melanotic schwannoma has been described as a part of the Carney complex. Distinguishing melanotic schwannoma from malignant melanoma is of paramount importance in planning the management, but these two tumours are probably sharing the same origin. It must be distinguished from classic schwannoma because its behaviour is unpredictable, requiring long-term follow-up.
神经鞘瘤是常见的遍布全身的神经鞘良性肿瘤。在各种组织病理学类型中，色素性神经鞘瘤必须与黑色素瘤相鉴别。我们报告一例52岁男性的小脑桥脑角黑色素性神经鞘瘤病例，自1978年以来该患者患有缓慢进展的小脑综合征，并出现后颅窝出血。黑色素性神经鞘瘤是神经鞘瘤的一种罕见形式。诊断仍然困难，黑色素性神经鞘瘤已被描述为卡尼综合征的一部分。在制定治疗方案时，将黑色素性神经鞘瘤与恶性黑色素瘤区分开来至关重要，但这两种肿瘤可能起源相同。它必须与经典神经鞘瘤相鉴别，因为其行为不可预测，需要长期随访。
