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. Treatment often involves surgery to remove the tumor, such as by Mohs’ micrographic surgery. If you or a family member has been diagnosed with dermatofibrosarcoma protuberans, talk with your doctor about the most current treatment options.
 

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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How do you compare to others with this condition?

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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 58 free, full-text research articles on human participants. First 3 results:

Dermatofibrosarcoma protuberans: a study of clinical, pathologic, genetic, and therapeutic features in Korean patients.
 

Author(s): Zhenlong Zheng, Junjei Piao, Ji-Hye Lee, Song-Ee Kim, Soo-Chan Kim, Kee Yang Chung, Mi Ryung Roh

Journal: Yonsei Med. J.. 2015 Mar;56(2):440-6.

 

Dermatofibrosarcoma protuberans (DFSP) carries a translocation resulting in the collagen type I alpha 1 (COL1A1)-platelet-derived growth factor beta (PDGFB) fusion gene, which is responsible for PDGFB activation. The purpose of this study is to evaluate the clinicopathological, genetic, ...

Last Updated: 16 Feb 2015

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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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Neoadjuvant imatinib in advanced primary or locally recurrent dermatofibrosarcoma protuberans: a multicenter phase II DeCOG trial with long-term follow-up.
 

Author(s): Selma Ugurel, Thomas Mentzel, Jochen Utikal, Peter Helmbold, Peter Mohr, Claudia Pföhler, Meinhard Schiller, Axel Hauschild, Rüdiger Hein, Eckhardt Kämpgen, Ivonne Kellner, Martin Leverkus, Jürgen C Becker, Philip Ströbel, Dirk Schadendorf

Journal: Clin. Cancer Res.. 2014 Jan;20(2):499-510.

 

Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous tumor. COL1A1-PDGFB gene fusion is frequent in DFSP, rendering tumor cell proliferation and survival dependent on PDGFRβ (platelet-derived growth factor receptor β) signaling. This trial investigated imatinib as neoadjuvant ...

Last Updated: 20 Jan 2014

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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 6 free, full-text review articles on human participants. First 3 results:

[Dermatofibrosarcoma protuberans : report of 27 cases and review of the literature].
 

Author(s): Nawal Hammas, Ikram Badioui, Kaoutar Znati, Amal Benlemlih, Laila Chbani, Hind El Fatemi, Taoufiq Harmouch, Youssef Bouyahyaoui, Faouzi Boutayeb, Abdelmajid Mrini, Omar Mesbahi, Fatima Zahra Mernissi, Afaf Amarti

Journal:

 

Last Updated: 9 Dec 2014

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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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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

Clinical Outcome of Pediatric Dermatofibrosarcoma Protuberans
 

Status: Recruiting

Condition Summary: Soft Tissue Neoplasms

 

Last Updated: 23 Nov 2005

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Spanish Registry of Mohs Surgery
 

Status: Recruiting

Condition Summary: Skin Neoplasms; Carcinoma, Basal Cell; Carcinoma, Squamous Cell; Dermatofibrosarcoma; Melanoma; Histiocytoma, Malignant Fibrous; Neoplasms, Adnexal and Skin Appendage

 

Last Updated: 10 Aug 2015

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To Find a Safe Dose and Show Early Clinical Activity of Weekly Nab-paclitaxel in Pediatric Patients With Recurrent/ Refractory Solid Tumors
 

Status: Recruiting

Condition Summary: Neuroblastoma;; Rhabdomyosarcoma;; Ewing's Sarcoma;; Ewing's Tumor;; Sarcoma, Ewing's;; Sarcomas, Epitheliod;; Sarcoma, Soft Tissue;; Sarcoma, Spindle Cell;; Melanoma;; Malignant Melanoma;; Clinical Oncology;; Oncology, Medical;; Pediatrics, Osteosarcoma;; Osteogenic Sarcoma;; Osteosarcoma Tumor;; Sarcoma, Osteogenic;; Tumors;; Cancer;; Neoplasia;; Neoplasm;; Histiocytoma;; Fibrosarcoma;; Dermatofibrosarcoma

 

Last Updated: 17 Aug 2015

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