Neurocutaneous Melanosis with Malignant Leptomeningeal Melanoma

神經皮膚性黑色症合併惡性軟腦膜黑色素瘤

張宏嘉(Hung-Chia Chang) ； 王偉銘(Wei-Ming Wang)

《中華皮膚科醫學雜誌》 23卷3期 (2005/09) Pp. 162-167

https://doi.org/10.29784/DS.200509.0009

摘要

神經皮膚性黑色症為有巨大或多發的先天性黑色素細胞母斑合併軟腦膜有良性或惡性的色素細胞腫瘤。我們報告一例神經皮膚性黑色症，病人過去之健康狀況良好且無神經學之異常，於二十一歲時以視力模糊為初始神經學症狀表現。我們被會診而發現病人有一過度巨大，具毛髮的先天性黑色素細胞母斑及數個大小不等之毛髮母斑散佈於驅幹處。病人接受前額開顱手術拿取腫瘤及減壓處置後，發現為軟腦膜黑色症及黑色素瘤。皮膚上之病灶無惡性病變之證據，且腦膜以外之器官無惡性黑色素瘤之發現。病人確定診斷為神經皮膚性黑色症合併惡性軟腦膜黑色素瘤後，接受放射線治療，於三個月後仍不幸死亡。

並列摘要

Neurocutaneous melanosis (NCM) is a rare congenital syndrome characterized by the presence of large or multiple congenital melanocytic nevi with either benign or malignant pigmented cell tumors of the leptomeninges. We report a case of NCM in a patient with previously robust health. No neurological deficit was noted in the patient. At the age of 21 years old, blurred vision initially appeared. We were consulted to evaluate cutaneous lesions in the patient. We found an unduly large, hairy, congenital melanocytic nevus predominantly over the patient's torso with multiple variously sized scattered hairy nevi over his trunk. The patient was diagnosed as NCM with malignant leptomeningeal melanoma following pathologic evaluation of the specimen taken druing frontal craniotomy for removal of tumor and decompression. No other malignant change of the cutaneous lesions and internal organs was noted except in the meninges. The patient received treatment with radiotherapy. However, he died within 3 months.

並列關鍵字

Large congenital melanocytic newts ； Melanoma ； Neurocutaneous melanosis