Dermatofibrosarcoma

Dermatofibrosarcoma

Infobox_Disease
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ICDO = 8832/3
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MeshID = D018223

Dermatofibrosarcoma protuberans (DFSP) [cite journal |author=Mendenhall WM, Zlotecki RA, Scarborough MT |title=Dermatofibrosarcoma protuberans |journal=Cancer |volume=101 |issue=11 |pages=2503–8 |year=2004 |month=Dec |pmid=15503305 |doi=10.1002/cncr.20678 |url=http://www3.interscience.wiley.com/journal/109720419/abstract Review. ] is a rare tumor. There is only about 1 case per million per year. DFSP is a fibrosarcoma, [DorlandsDict|three/000028732|dermatofibrosarcoma] more precisely a cutaneous soft tissue sarcoma.

Presentation

About 90% of DFSPs are low grade sarcomas. About 10% are mixed; they contain a high-grade sarcomatous component (DFSP-FS); therefore, they are considered to be intermediate-grade sarcomas. All DFSPs rarely lead to a metastasis (fewer than 5% do metastasise), but DFSPs can recur locally. DFSPs most often arise in patients who are in their thirties, but sometimes have been described in children or the elderly.

Treatment

The standard of care for patients with DFSP is surgery. Usually, complete surgical resection with wide margins is performed. The addition of adjuvant radiotherapy (irradiation) improves local control in patients with close or positive margins during the surgery. A special surgical technique, the "Mohs micrographic surgery" (MMS), can be employed in patients with DFSP. MMS is technically possible if the DFSP is in an anatomically confined area. A high probability of cure of DFSP can be attained with MMS as long as the final margins are negative. [cite journal |author=Gloster HM, Harris KR, Roenigk RK |title=A comparison between Mohs micrographic surgery and wide surgical excision for the treatment of dermatofibrosarcoma protuberans |journal=J Am Acad Dermatol. |volume=35 |issue=1 |pages=82–7 |year=1996 |month=Jul |pmid=8682970 |doi= |url=] Patients who have a recurrent DFSP can have further surgery, but the probability of adverse effects of surgery and/or metastasis is increased in these patients.

Imatinib is a so-called "biologic", i.e. a newer medicinal drug. As all medicinal drugs which have a name that ends on -ib, imatinib is a small molecular pathway inhibitor; imatinib inhibits tyrosine kinase. It may be able to induce tumor regression in patients with recurrent DFSP, unresectable DFSP or metastatic DFSP.

References


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Look at other dictionaries:

  • dermatofibrosarcoma — Fibrosarcoma o tumor fibroso de la piel. Diccionario Mosby Medicina, Enfermería y Ciencias de la Salud, Ediciones Hancourt, S.A. 1999 …   Diccionario médico

  • Dermatofibrosarcoma protuberans — Classification and external resources Histopathological image of dermatofibrosarcoma protuberans. Local recurrence long after the first excision. H E stain …   Wikipedia

  • dermatofibrosarcoma protuberante — una neoplasia voluminosa, protuberante, nodular, fibrótica que tiene lugar en la dermis, usualmente del tronco, extendiéndose en la grasa subcutánea. A veces es clasificado como un histiocitoma maligno ICD 10: (M8832/3)] imagen clínica [véase… …   Diccionario médico

  • Dermatofibrosarcoma protuberans — Klassifikation nach ICD 10 C44 Sonstige bösartige Neubildungen der Haut Dermatofibrosarcoma protuberans …   Deutsch Wikipedia

  • dermatofibrosarcoma protuberans — A relatively slowly growing dermal neoplasm consisting of one or several firm nodules that are usually covered by dark red blue skin, which tends to be fixed to the palpable masses; histologically, the neoplasm resembles a cellular dermatofibroma …   Medical dictionary

  • dermatofibrosarcoma — der·ma·to·fi·bro·sar·co·ma .fī brō sär kō mə n, pl mas also ma·ta mət ə a fibrosarcoma affecting the skin * * * der·ma·to·fi·bro·sar·co·ma (dur″mə to fi″bro sahr koґmə) [dermato + fibrosarcoma] a fibrosarcoma of the skin …   Medical dictionary

  • Dermatofibrosarcoma protuberans — Dermato|fi|bro|sarco̱ma pro|tu̱berans [↑Derma u. ↑Fibrosarkom; lat. protuberareDermatofibrosarcoma protuberans= hervorschwellen] s; , ta ...rạntia: knollenbildender Tumor an der Haut …   Das Wörterbuch medizinischer Fachausdrücke

  • dermatofibrosarcoma protuberans — a tumour probably derived from histiocytes that may occur in any part of the body. It is locally invasive but tends not to metastasize. It often recurs locally despite excision …   The new mediacal dictionary

  • dermatofibrosarcoma protuberans — A type of tumor that begins as a hard nodule and grows slowly. These tumors are usually found in the dermis (the inner layer of the two main layers of tissue that make up the skin) of the limbs or trunk of the body. They can grow into surrounding …   English dictionary of cancer terms

  • Дерматофибросаркома Выбухающая (Dermatofibrosarcoma Protuberans) — опухоль, возможно, происходящая из гистиоцитов; может развиться в любой части тела. Является локально инвазивной, но не дает метастазов. Часто требуется ее полное иссечение. Источник: Медицинский словарь …   Медицинские термины

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