Open AccessIntermediate-grade meningeal melanocytoma associated with nevus of Ota
Author(s) -
Donghoon Shin,
Milind Kumar Sinha,
Douglas Kondziolka,
John M. Kirkwood,
Uma Rao,
Ahmad A. Tarhini
Publication year - 2015
Publication title -
melanoma research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.072
H-Index - 73
eISSN - 1473-5636
pISSN - 0960-8931
DOI - 10.1097/cmr.0000000000000163
Subject(s) - melanocytoma , gnaq , nevus of ota , pathology , melanoma , medicine , lesion , nevus , dermatology , cancer research , mutation , gene , biology , biochemistry
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.