A Clinical, Histologic, and Molecular Study of 9 Cases of Congenital Dermatofibrosarcoma Protuberans
نویسندگان
چکیده
منابع مشابه
A clinical, histologic, and molecular study of 9 cases of congenital dermatofibrosarcoma protuberans.
BACKGROUND The diagnosis of dermatofibrosarcoma protuberans (DFSP) in childhood is often difficult because of the deceptive appearance of the lesions. Little is known about congenital DFSP, the frequency of which is probably underestimated because the initial lesion may pass unnoticed. OBSERVATIONS We studied 9 DFSP congenital cases (8 plaques and 1 nodule) initially suspected to be benign le...
متن کاملTransformed dermatofibrosarcoma protuberans: a clinicopathological study of eight cases.
BACKGROUND Fibrosarcomatous (FS) or malignant fibrous histiocytomatous (MFH) transformation of dermatofibrosarcoma protuberans (DFSP) is a rare, but well known, entity. DFSPs with sarcomatous areas have questionable biological behaviour. Several studies suggest that they have a higher risk for local recurrence and distant metastases than ordinary DFSPs. One recent study described no difference ...
متن کامل[Congenital dermatofibrosarcoma protuberans: a case report].
Dermatofibrosarcoma protuberans is a rare low-grade fibrohistiocytic tumor with onset normally at ages between 20 and 50 years. It presents as a violaceous plaque or macule with an appearance suggestive of vascular lesion, on which nodular lesions appear later. Histological diagnosis is based on the presence of a spindle-cell tumor arranged in small bundles in a characteristic cartwheel pattern...
متن کاملCongenital dermatofibrosarcoma protuberans: variability in presentation.
BACKGROUND Dermatofibrosarcoma protuberans (DFSP) is an uncommon low-grade fibrohistiocytic tumor that usually occurs on the trunk or proximal extremities and typically appears during the second to fifth decade of life. It most commonly begins as a red-blue plaque that grows slowly and ultimately becomes nodular. The tumor is associated with a high recurrence rate but low metastatic potential. ...
متن کاملCongenital Dermatofibrosarcoma Protuberans: A Case Report and Literature Review.
Congenital dermatofibrosarcoma protuberans (DFSP) is an extremely rare skin tumor that is commonly misdiagnosed, or is often diagnosed long after the initial presentation. Although many cases of DFSP are diagnosed in adulthood, there are some differences between adult DFSP and congenital DFSP. We report a case of congenital DFSP that was initially misdiagnosed as a simple vascular lesion. The d...
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ژورنال
عنوان ژورنال: Archives of Dermatology
سال: 2007
ISSN: 0003-987X
DOI: 10.1001/archderm.143.2.203