title: Familial variant of dermatofibroma with malignancy in the proband.
abstract: Multiple fibrous histiocytomas of the dermis (dermatofibromas) were found in six males of one family. A number of the tumors fell within the histologic patterns commonly encountered in dermatofibromas, but some were unusually large and others were cytologically atypical. In addition, the proband died of a malignant fibrous histiocytoma of the lung. We believe that this is a syndrome and a familial variant of a common dermal entity.
mesh_terms: D005350:Fibroma / Q000235:genetics* / Q000473:pathology; D018219:Histiocytoma, Benign Fibrous / Q000473:pathology; D006801:Humans; D008168:Lung / Q000473:pathology; D008175:Lung Neoplasms / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary; D010375:Pedigree; D012867:Skin / Q000473:pathology; D012878:Skin Neoplasms / Q000235:genetics* / Q000473:pathology; D013577:Syndrome