A Case of Nevoid Basal Cell Carcinoma Syndrome with Meningioma and Sclerosing Stromal Tumor of the Ovary.
- Author:
Byoung Hwa ROH
1
;
Soo Ji PARK
;
Sung Yul LEE
;
Jong Suk LEE
;
Kyu Uang WHANG
Author Information
1. Department of Dermatology, College of Medicine, Soonchunhyang University, Cheonan, Korea. doctornobel@naver.com
- Publication Type:Case Report
- Keywords:
Meningioma;
Nevoid basal cell carcinoma syndrome;
Sclerosing stromal tumor of the ovary
- MeSH:
Adolescent;
Basal Cell Nevus Syndrome*;
Carcinoma, Basal Cell;
Central Nervous System;
Cervical Vertebrae;
Fathers;
Female;
Humans;
Hypertelorism;
Jaw;
Medulloblastoma;
Meningioma*;
Odontogenic Cysts;
Ovary*;
Ribs;
Spinal Dysraphism
- From:Korean Journal of Dermatology
2006;44(8):1030-1033
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Nevoid basal cell carcinoma syndrome is inherited as an autosomal dominant trait with variable expressivity. This is characterized by multiple basal cell carcinomas, skeletal abnormalities, ectopic calcification, palmoplantar pits, central nervous system abnormalities, and multiple odontogenic keratocysts of the jaw. We report a case of nevoid basal cell carcinoma syndrome occurring in 14 year old girl, she had multiple basal cell carcinomas, spina bifida in the cervical vertebrae, forked ribs, hypertelorism, ectopic calcification of falx cerebri, palmoplantar pits, medulloblastoma, meningioma and sclerosing stromal tumor of the ovary. Her father had the same clinical features.
