Dermatofibrosarcoma protuberans with fibrosarcomatous transformation of the head and neck
نویسندگان
چکیده
Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous neoplasm associated with a high cure rate. We present a case of aggressive DFSP with fibrosarcomatous areas in the head and neck. A 28-year-old Mediterranean female presented with a 45-day history of rapidly growing cutaneous lesion of the face. Surgical biopsy confirmed the diagnosis of DFSP. Subsequently, the patient underwent wide local surgical resection, followed by reconstruction. Histopathology report revealed fibrosarcomatous transformation and the patient underwent adjuvant radiotherapy. The patient continues to be disease free at the 35-month follow-up.Although DFSP behave as non-aggressive malignancy, surgery with complete removal of the affected area is the intervention of choice. Moreover, adjuvant treatment and follow-up of the patient is essential in order to prevent recurrence.
منابع مشابه
Fibrosarcomatous dermatofibrosarcoma protuberans: A case report of an aggressive soft tissue sarcoma
Dermatofibrosarcoma protuberans is a rare soft tissue neoplasm arising from mesenchymal cells, making up about 6% of all soft tissue tumours. Often found on the scalp, neck, trunk and extremities, it has an intermediateto low-grade malignancy potential except the fibrosarcomatous variant (high-grade), which is more likely to metastasize than other types. Here we present the case of a 46-year-ol...
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Dermatofibrosarcoma protuberans (DFSP) is an unusual, locally aggressive, cutaneous neoplasm of low to intermediate grade malignant potential. Fibrosarcomatous DFSP (FS-DFSP), a rare variant of DFSP, has got a higher tendency for recurrence and metastasis resulting in significant morbidity and reduced survival. We present a case of this rare malignancy in a 23-year-old female who presented with...
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Dermatofibrosarcoma protuberans (DFSP) is a relatively uncommon soft tissue tumor with intermediate-to-low grade malignancy. There is usually little nuclear pleomorfism and only low to moderate mitotic activity. Conventional DFSP, usually have fewer than 5 mitotic figures/10 high-power fields. We describe a case of a 74year old male patient who presented to the surgical clinic with active bleed...
متن کاملDermatofibrosarcoma protuberans with fibrosarcomatous transformation*
To the Editor, Dermatofibrosarcoma protuberans (DFSP) is an uncommon sarcoma of the skin and soft tissue with an intermediate level of malignancy. DFSP with fibrosarcomatous differentiation (FS-DFSP) is a rare variant of DFSP, involving greater aggression, higher rates of local recurrences and a higher metastatic potential. We describe the case of a 70-year-old woman who presented with a painle...
متن کاملImaging findings of head and neck dermatofibrosarcoma protuberans.
BACKGROUND AND PURPOSE Dermatofibrosarcoma protuberans is a rare, locally aggressive sarcoma of the skin in children and adults, usually involving the trunk and extremities and less commonly the head and neck. Despite clinical reports in the literature on the management of dermatofibrosarcoma protuberans, there are limited articles describing its imaging features. MATERIALS AND METHODS We ret...
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