A Rare Case of Vulvar Dermatofibrosarcoma Protuberans: A Case Report

Tewodros Deneke*, Abel Tefera, Woubshet Girma, Fanta Assefa, Demsew Alemu and Bahiru Gossaye

A Rare Case of Vulvar Dermatofibrosarcoma Protuberans: A Case Report

Dermatofibrosarcoma protuberans is a low-to-intermediate grade sarcoma
of dermal origin that rarely presents in the vulva.

It is an uncommon soft tissue tumor that has a high
local recurrence rate with an incidence of 0.1% of all cancers and
1% of all soft tissue sarcomas.

It affects adults between the second and fifth decade of life and the involved areas are most frequently the trunk, proximal extremities, head and neck.

DFSP is flesh-colored or slightly yellow–brown skin tumour without epidermal invasion but with intracutaneously and subcutaneous spread. Sometimes the tumour presents as a reddish, flat
elevated, firm lesion with irregular borders or multinodular appearance.

Histopathology with Immunohistochemical findings is used to diagnose DFSP.
As metastasis is rare, morbidity due to local recurrence is a more common issue.

The standard treatment techniques for resectable DFSP are complete surgical excision with
wide local excisions of 2 or 3 cm tumor-free margins whereas; unresectable DFSPs are treated with radiation therapy, which improve local control and reduce the risk of recurrence post-operatively.

early recognition of DFSP is extremely important because of the
excellent prognosis following adequate excision.

A 45-years-old Para 4 women from rural area presented in Jimma
University Medical Center (JUMC) for complaint of swelling on
right vulvar area for the last 17-month duration which increase
in size for the last 6-month.

She visited local traditional healer
3-month back, ointment given to be applied daily base and started
to have discharge with blood from the mass, itching sensation and pain.

Gynecol Obstet Res Open J. 2022; 8(1): 15-18. doi: 10.17140/GOROJ-8-157