Gorlin syndrome and bilateral ovarian fibroma

• DOI: 10.1016/j.ijscr.2012.05.015
• Publication Date: 2012-06-02T23:02:16Z
• Authors: Fernanda Pirschner; Pollyana Marçal B...; George Luiz Conta...; Anna Carolina Bon...; Antônio Chambô Filho
• Keywords: Kyphoscoliosis

Gorlin syndrome (GS), also known as nevoid basal cell carcinoma (NBCCS), is a rare hereditary, autosomal dominant disease that affects various systems. Its prevalence estimated at 1/57,000 to 1/256,000 of the population. It characterized by carcinomas, multiple odontogenic keratocysts, skeletal abnormalities and ovarian fibroma, among other disorders.To report case young patient with bilateral fibroma.A 20-year old presented facial asymmetry, broad nasal root, dental abnormalities, micrognathism, convergent strabismus, pigmented lesions on trunk face, pectus excavatum, kyphoscoliosis palpable mass in abdomen occupying entire pelvic region.Gorlin-Goltz hereditary pathology includes numerous clinical manifestations. Diagnosis genetic confirmation unnecessary.