Pierson Matt, Marwaha Nitin, Guzman Miguel, Mikulec Anthony A, Coppens Jeroen R
Department of Neurosurgery, Saint Louis University, Saint Louis, Missouri, USA.
Department of Pathology, Saint Louis University, Saint Louis, Missouri, USA.
Surg Neurol Int. 2016 Sep 1;7(Suppl 23):S591-5. doi: 10.4103/2152-7806.189728. eCollection 2016.
Primary leptomeningeal melanocytic neoplasms of the central nervous system are rare. Multifocal lesions typically occur in the setting of cutaneous melanosis. We present the first report of a posterior fossa melanocytoma and subcutaneous melanocytoma of intermediate grade in the absence of cutaneous melanosis.
We present the case of a 22-year-old male with decreased hearing on the right side, ataxia, nausea, vomiting and a scalp mass. Magnetic resonance imaging (MRI) demonstrated occipital and cerebellopontine (CP) angle masses. The patient underwent gross total resection of the scalp mass and subtotal resection of the CP angle mass. Pathologic examination revealed melanocytoma with intermediate grade. The patient underwent stereotactic radiosurgery to the residual CP angle tumor. This case represents, to the author's knowledge, the first report associating a posterior fossa melanocytoma with a subcutaneous melanocytoma of intermediate grade in the absence of cutaneous melanosis.
This case introduces the first report of a new variant of multifocal melanocytoma which is not confined to the central nervous system.
中枢神经系统原发性软脑膜黑素细胞肿瘤较为罕见。多灶性病变通常发生于皮肤黑素沉着的情况下。我们首次报告了一例在无皮肤黑素沉着的情况下发生的后颅窝黑素细胞瘤和中度皮下黑素细胞瘤。
我们报告了一名22岁男性病例，其右侧听力下降、共济失调、恶心、呕吐并伴有头皮肿物。磁共振成像（MRI）显示枕部和小脑脑桥角（CP角）肿物。患者接受了头皮肿物的全切术和CP角肿物的次全切除术。病理检查显示为中度黑素细胞瘤。患者对残留的CP角肿瘤进行了立体定向放射外科治疗。据作者所知，该病例首次报道了在无皮肤黑素沉着的情况下，后颅窝黑素细胞瘤与中度皮下黑素细胞瘤相关联。
本病例首次报告了一种新的多灶性黑素细胞瘤变体，该变体不限于中枢神经系统。
