Cardiac Fibroma & Gorlin-Goltz Syndrome
Image Description
Cardiac fibromas show an association with Gorlin-Goltz syndrome (nevoid basal cell carcinoma syndrome). It is an autosomal dominant multisystem disorder with complete penetrance and variable expressivity.
Patients can develop numerous abnormalities, including body overgrowth, odontogenic keratocysts, multiple basal cell carcinomas presenting at an early age, numerous other neoplasms, skeletal anomalies, nevi, ectopic calcification, and pits of hands and feet. Cardiac fibromas are seen in <20% of Gorlin-Goltz syndrome patients. Most patients with cardiac fibromas do not have full-blown manifestations of the syndrome.
About this image: Gorlin-Goltz syndrome in a 42 y/o man who presented with multiple basal cell carcinomas involving face, trunk and limbs, starting at adolescence and increasing in number and size with time. He also had characteristic palmar pits. Family history of similar condition was negative. Image courtesy of: Dr. Nameer Al-Sudany; permission granted by Dr. Ian McColl, GlobalSkinAtlas.com
Patients can develop numerous abnormalities, including body overgrowth, odontogenic keratocysts, multiple basal cell carcinomas presenting at an early age, numerous other neoplasms, skeletal anomalies, nevi, ectopic calcification, and pits of hands and feet. Cardiac fibromas are seen in <20% of Gorlin-Goltz syndrome patients. Most patients with cardiac fibromas do not have full-blown manifestations of the syndrome.
About this image: Gorlin-Goltz syndrome in a 42 y/o man who presented with multiple basal cell carcinomas involving face, trunk and limbs, starting at adolescence and increasing in number and size with time. He also had characteristic palmar pits. Family history of similar condition was negative. Image courtesy of: Dr. Nameer Al-Sudany; permission granted by Dr. Ian McColl, GlobalSkinAtlas.com