Dermatofibrosarcoma Protuberans

Common Name(s)

Dermatofibrosarcoma Protuberans

Dermatofibrosarcoma protuberans is an uncommon tumor that arises in the skin. The tumor is firm and often flesh-colored although it can be reddish, bluish, or purplish. The tumor is often found on the chest or shoulders, but it can be found on other parts of the body.  Dermatofibrosarcoma protuberans may cause no symptoms, and the initial size of the tumor tends to be around 1 to 5 centimeters. This tumor has a low potential to spread to other tissues (metastasize). Treatment often involves surgery to remove the tumor, such as by MohsÕ micrographic surgery. 
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Dermatofibrosarcoma Protuberans" for support, advocacy or research.

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Sarcoma Alliance

The Sarcoma Alliance strives to improve the lives of people affected by sarcoma through accurate diagnosis, improved access to care, guidance, education and support.

Last Updated: 20 Feb 2013

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Dermatofibrosarcoma Protuberans" for support, advocacy or research.

Logo
Sarcoma Alliance

The Sarcoma Alliance strives to improve the lives of people affected by sarcoma through accurate diagnosis, improved access to care, guidance, education and support.

http://sarcomaalliance.org

Last Updated: 20 Feb 2013

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Dermatofibrosarcoma Protuberans" returned 54 free, full-text research articles on human participants. First 3 results:

Case for diagnosis. Dermatofibrosarcoma protuberans.
 

Author(s): Joanna Pimenta de Araujo Franco, Caroline Cruz Barbosa, Bárbara Ferreira Saraiva da Fonseca, Ricardo Barbosa Lima, Antônio Macedo D'Acri, Carlos José Martins

Journal: An Bras Dermatol. ;89(2):357-8.

 

Dermatofibrosarcoma protuberans is a fibrohistiocytic tumor of intermediate malignancy with aggressive localized growth, high recurrence rate, but low metastatic potential. It appears as a hardened plaque, with slow growth, upon which the development of nodules occurs. It predominates ...

Last Updated: 28 Apr 2014

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Genetic aberrations in imatinib-resistant dermatofibrosarcoma protuberans revealed by whole genome sequencing.
 

Author(s): Jung Yong Hong, Xiao Liu, Mao Mao, Miao Li, Dong Il Choi, Shin Woo Kang, Jeeyun Lee, Yoon La Choi

Journal:

 

Dermatofibrosarcoma protuberans (DFSP) is a very rare soft tissue sarcoma. DFSP often reveals a specific chromosome translocation, t(17;22)(q22;q13), which results in the fusion of collagen 1 alpha 1 (COL1A1) gene and platelet-derived growth factor-B (PDGFB) gene. The COL1A1-PDGFB ...

Last Updated: 7 Aug 2013

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[Dermatofibrosarcoma protuberans: a study of 18 cases].
 

Author(s): Sana Chouk Friaa, Talel Badri, Houda Hammami, Rym Benmously, Hayet Marrak, Aschraf Debbiche, Incaf Mokhtar, Samy Fenniche

Journal: Tunis Med. 2013 May;91(5):342-5.

 

Dermatofibrosarcoma protuberans is the most common mesenchymatous skin tumor. It is often diagnosed late because of its slow development and the lack of symptoms.

Last Updated: 29 May 2013

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Dermatofibrosarcoma Protuberans" returned 5 free, full-text review articles on human participants. First 3 results:

Dermatofibrosarcoma protuberans with fibrosarcomatous transformation of the head and neck.
 

Author(s): Nikolaos Angouridakis, Panagiotis Kafas, Waseem Jerjes, Stefanos Triaridis, Tahwinder Upile, Georgios Karkavelas, Angelos Nikolaou

Journal:

 

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 ...

Last Updated: 15 Feb 2011

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Dermatofibrosarcoma protuberans: clinicopathological aspects of an unusual cutaneous tumor.
 

Author(s): D P Korkolis, I E Liapakis, P P Vassilopoulos

Journal: Anticancer Res.. ;27(3B):1631-4.

 

Dermatofibrosarcoma protuberans is a rare cutaneous tumor with particular characteristics and a high frequency of recurrence after inadequate primary treatment. Its histopathological diagnosis might be difficult. Dermatofibrosarcoma protuberans can be safely distinguished from other ...

Last Updated: 28 Jun 2007

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Dermatofibrosarcoma protuberans treated at a single institution: a surgical disease with a high cure rate.
 

Author(s): Marco Fiore, Rosalba Miceli, Chiara Mussi, Salvatore Lo Vullo, Luigi Mariani, Laura Lozza, Paola Collini, Patrizia Olmi, Paolo G Casali, Alessandro Gronchi

Journal: J. Clin. Oncol.. 2005 Oct;23(30):7669-75.

 

Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade, cutaneous sarcoma with autocrine overproduction of the platelet-derived growth factor (PDGF) beta-chain from gene rearrangement as a key pathogenetic factor, now susceptible of molecular-targeted therapy. The aim of this ...

Last Updated: 19 Oct 2005

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Phase II Pazopanib Study in Advanced Dermatofibrosarcomas
 

Status: Recruiting

Condition Summary: Dermatofibrosarcomas of DARIER FERRAND(DFSP)

 

Last Updated: 28 Jun 2012

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Clinical Outcome of Pediatric Dermatofibrosarcoma Protuberans
 

Status: Recruiting

Condition Summary: Soft Tissue Neoplasms

 

Last Updated: 23 Nov 2005

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Pazopanib Hydrochloride Followed By Chemotherapy and Surgery in Treating Patients With Soft Tissue Sarcoma
 

Status: Recruiting

Condition Summary: Adult Angiosarcoma; Adult Desmoplastic Small Round Cell Tumor; Adult Epithelioid Hemangioendothelioma; Adult Epithelioid Sarcoma; Adult Extraskeletal Chondrosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Fibrous Histiocytoma; Adult Malignant Hemangiopericytoma; Adult Malignant Mesenchymoma; Adult Neurofibrosarcoma; Adult Synovial Sarcoma; Dermatofibrosarcoma Protuberans; Stage IIA Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

 

Last Updated: 16 Jun 2014

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