Shin Donghoon, Sinha Milind, Kondziolka Douglas S, Kirkwood John M, Rao Uma N M, Tarhini Ahmad A
University of Pittsburgh, Pittsburgh, Pennsylvania, USA.
Melanoma Res. 2015 Aug;25(4):273-8. doi: 10.1097/CMR.0000000000000163.
Meningeal melanocytomas are rare melanin-producing tumors that are often found to be benign. However, a small subset of these tumors can present as intermediate-grade melanocytomas (IGMs) that have histopathological features that are between those of benign melanocytomas and malignant melanomas. IGMs have the potential to recur and metastasize or progress to a more histologically high grade melanoma. Melanocytomas appear to differ from primary and metastatic melanoma by their prolonged clinical course and they appear to have different driver mutations (i.e. mutation of GNAQ gene). The association of a meningeal melanocytoma with nevus of Ota is extremely rare. To our knowledge, there have been only 10 reported cases of synchronous occurrence and only one of the cases involved an IGM. We report the second case of intermediate-grade meningeal melanocytoma that is associated with congenital nevus of Ota. Histopathological work-up confirmed the intermediate grade of the lesion and a driver GNAQ mutation was identified consistent with previous reports.
脑膜黑素细胞瘤是罕见的产生黑色素的肿瘤，通常为良性。然而，这些肿瘤中的一小部分可表现为中间级黑素细胞瘤（IGM），其具有介于良性黑素细胞瘤和恶性黑色素瘤之间的组织病理学特征。IGM有复发、转移或进展为组织学上更高分级黑色素瘤的可能。黑素细胞瘤似乎因其临床病程较长而与原发性和转移性黑色素瘤不同，并且它们似乎具有不同的驱动突变（即GNAQ基因突变）。脑膜黑素细胞瘤与太田痣的关联极为罕见。据我们所知，仅有10例同步发生的报道病例，其中只有1例涉及IGM。我们报告第二例与先天性太田痣相关的中间级脑膜黑素细胞瘤。组织病理学检查证实了病变的中间分级，并鉴定出一个驱动GNAQ突变，与先前报道一致。
