Intermediate-grade meningeal melanocytoma associated with nevus of Ota: a case report and review of the literature.
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.
University of Pittsburgh, Pittsburgh, Pennsylvania, USA.
Donghoon
D
Shin
Milind
M
Sinha
Douglas S
DS
Kondziolka
John M
JM
Kirkwood
Uma N M
UN
Rao
Ahmad A
AA
Tarhini
D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D044385:GTP-Binding Protein alpha Subunits / Q000235:genetics*; D043802:GTP-Binding Protein alpha Subunits, Gq-G11; D006801:Humans; D008297:Male; D008543:Melanins / Q000096:biosynthesis; D008544:Melanocytes / Q000473:pathology*; D008545:Melanoma / Q000150:complications / Q000235:genetics* / Q000628:therapy; D008577:Meningeal Neoplasms / Q000150:complications / Q000235:genetics* / Q000628:therapy; D008875:Middle Aged; D009507:Nevus of Ota / Q000235:genetics* / Q000473:pathology; D055815:Young Adult
C554061:GNAQ protein, human; D044385:GTP-Binding Protein alpha Subunits; D008543:Melanins; D043802:GTP-Binding Protein alpha Subunits, Gq-G11