Korean J Dermatol.  2006 Aug;44(8):1030-1033.

A Case of Nevoid Basal Cell Carcinoma Syndrome with Meningioma and Sclerosing Stromal Tumor of the Ovary

• Roh BH
• Park SJ
• Lee SY
• Lee JS
• Whang KU

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.

Keyword

Meningioma; Nevoid basal cell carcinoma syndrome; Sclerosing stromal tumor of the ovary

MeSH Terms

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