DERMATOFIBROSARCOMA PROTUBERANS

by Gary White, MD
Dermatofibrosarcoma protuberans (DFSP) Courtesy Michael O. Murphy, MD


Dermatofibrosarcoma protuberans (DFSP) is a slow-growing malignant fibrous tumor that rarely metastasizes (1-3%), but may recur locally. See also Bednar Tumor. In the majority of cases, the tumorigenic mechanism arises from translocation of chromosomes 17 and 22, inducing the formation of the COL1A1-PDGFB fusion gene [JAMA Derm 2015;151;1330].

Clinical

A slowly-growing, firm plaque which later becomes multinodular on the trunk or proximal extremities in an adult is characteristic. Congenital lesions have been reported, and lesions have occurred at sites of prior trauma e.e. vaccination site, bayonet wound. Metastases are rare but local recurrence after surgery is common. DFSP may rarely occur in childhood is often in an acral location [BJD 1998;139;719]. In extremely rare cases, it may be congenital [Clin and Exp Derm 2013;38;441].

An atrophic variant has been described.    Dermal atrophy is present, and dermal thickness may be reduced to less than 50% in the middle of the lesion. 

DFSP in children

DFSP do occur in children [Pediatric Dermatology 2013;30;462]. A review of SEERs data showed 7/17 in one series were congenital. 8/17 were on the trunk.

Workup

A deep incisional biopsy is usually necessary to show the growth pattern and allow for definitive diagnosis. Usually no imaging or other workup is done unless history and physical suggests a problem.

Treatment  

Mohs Micrographic Surgery     

Mohs micrographic surgery is the treatment of choice as it has a low rate of recurrence. Wide surgical excision without the microscopic evaluation of all margins can leave tumor, even if a wide margin of clinically normal skin is removed [JAAD 1997;37;600]. See also BJD 2015;172;1303.

Slow Mohs (Mohs on paraffin-embedded sections) should be considered especially for recurrent tumors as it allows special stains which better distinguish tumor from scar tissue.

Adjuvant radiation therapy is recommended when standard wide excision results in narrow or positive margins and further surgery is impractical [JEADV 2016;30;1107].

Imatinib

On October 19, 2006, the US Food and Drug Administration granted approval for imatinib mesylate (Gleevec) as a single agent for the treatment of DFSP. Imatinib mesylate is indicated for the treatment of adult patients with unresectable, recurrent, and/or metastatic disease. Some people have used it preMohs to shrink the tumor however this runs the risk of causing skip areas that may decrease the success of surgery.

Additional Pictures

Courtesy Michael O. Murphy, MD
Dermatofibrosarcoma protuberans (DFSP)
Dermatofibrosarcoma protuberans (DFSP) Dermatofibrosarcoma protuberans (DFSP)

Dermatofibrosarcoma protuberans (DFSP) Dermatofibrosarcoma protuberans (DFSP)

References

A 6 year old girl presented with a nodule on the upper back. It had been slowly growing since birth. It turned out to be a congenital DFSP [Ann Dermatol. 2015 Oct; 27(5): 597–600].


It is not the intention of RegionalDerm.com to provide specific medical advice, diagnosis or treatment. RegionalDerm.com only intends to provide users with information regarding various medical conditions for educational purposes and will not provide specific medical advice. Information on RegionalDerm.com is not intended as a substitute for seeking medical treatment and you should always seek the advice of a qualified healthcare provider for diagnosis and for answers to your individual questions. Information contained on RegionalDerm.com should never cause you to disregard professional medical advice or delay seeking treatment. If you live in the United States and believe you are having a medical emergency call 911 immediately.