Dermatofibrosarcoma Protuberans: A Rare Malignancy

OBJECTIVE Dermatofibrosarcoma protuberans (DFSP) is a fibrohistiocytic tumor characterized by protuberant growth pattern of skin and subcutaneous tissue. The annual incidence is 4.2 per million. It is often seen between the ages of 20?50 years. This study aimed to evaluate the characteristics of pat...

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Bibliographic Details
Published in:Türk onkoloji dergisi 2017, Vol.32 (4)
Main Author: Üyetürk, Ümmügül
Format: Article
Language:eng ; tur
Subjects:
Biopsy
Cytoplasm
Family medical history
Females
Inhibitor drugs
Medical diagnosis
Metastasis
Patients
Radiation therapy
Sarcoma
Stains & staining
Surgery
Surgical outcomes
Targeted cancer therapy
Thyroid gland
Tumors
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Summary:OBJECTIVE Dermatofibrosarcoma protuberans (DFSP) is a fibrohistiocytic tumor characterized by protuberant growth pattern of skin and subcutaneous tissue. The annual incidence is 4.2 per million. It is often seen between the ages of 20?50 years. This study aimed to evaluate the characteristics of patients with DFSP. METHODS Patients who visited our clinic were retrospectively evaluated. RESULTS All patients were females and the median age was 42 (29?60) years. The evaluated tumor sites were observed in the body/trunk in one patient, at the hip in two, and at the proximal lower extremity in two. Surgical excision was done in all of them as initial treatment. Re-excision was needed in three patients. One of the patients underwent punch biopsy for diagnosis and surgical removal of the tumor with a negative surgical margin. One of the patients underwent radiotherapy. The median follow-up time was 10 (6?26) months. During the median follow-up, there was no local recurrence or metastasis. CONCLUSION As initial treatment, it is important to surgically remove tumors that had no distant metastasis. Positive or near surgical margins should be resected again. If a negative surgical margin cannot be achieved, radiotherapy should be used as the treatment. Imatinib mesylate is used for treatment in unresectable, recurrent, and/or metastatic tumors.
ISSN:1300-7467
2717-8978
DOI:10.5505/tjo.2017.1635