Neurocutaneous syndromes and retroperitoneal tumors.
A patient with multiple basal cell carcinoma syndrome, a symptom complex characteristized by nevoid basal cell carcinomas of the skin, jaw cysts, skeletal abnormalities, and hyporesponsiveness to parathormone is presented. In addition, the patient had a retroperitoneal lymphagiomyoma, a hamartomatous lesion, causing ureteral obstruction. The association of neuroectodermic syndromes and retroperitoneal and intra-abdominal tumors is reviewed.
D000328:Adult; D002280:Basal Cell Carcinoma / Q000150:complications*; D005260:Female; D006391:Hemangioma / Q000150:complications / Q000175:diagnosis; D006801:Humans; D008202:Lymphangioma / Q000150:complications* / Q000175:diagnosis; D009378:Neoplasms, Multiple Primary / Q000150:complications* / Q000175:diagnosis; D012186:Retroperitoneal Neoplasms / Q000150:complications* / Q000175:diagnosis; D013577:Syndrome