Dermatofibrosarcoma protuberans of the vulva: margins assessment and reconstructive options – a report of two cases

Abstract

Background: Dermatofibrosarcoma Protuberans (DFSP) of the vulva is rare and oncologic surgery with free margins may lead to severe functional damage, requiring multidisciplinary approach regarding resection, margin assessment and reconstruction. Case Report: Two cases of DFSP in vulva were treated in a single institution. A 28-year-old patient with an incisional biopsy in the vulvar region revealing DFSP underwent a partial vulvectomy with clitoris preservation. Pathological studies revealed free margins and reconstructive surgery was performed. This patient is disease free in a 40 months follow up. The other, a 57-year-old patient was also referred after an incomplete resection of a DFSP in the vulvar region. A 1-cm margim resection followed by Complete Circumferential and Peripheral Deep Margin Assessment (CCPDMA) was performed. Although the upper lateral margin was positive, it was possible to perform another wide local excision with preservation of the clitoris and primary closure. Conclusion: DFSP of the vulva requires an accurate evaluation of margins, resections following oncological principles and reconstruction. Although being a very challenging lesion that usually implies difficult surgical management, if treated in a multidisciplinary environment, with surgical oncologists, experienced dermatopathologists and reconstructive surgeons can achieve good results. Even in difficult cases that presents with large lesions and compromising challenging areas, a complete oncologic resection can be performed minimizing functional damage for the patient.

Recurrent Dermatofibrosarcoma Protuberans of the Vulva: A Rare Occurrence and Review of Literature

“…Another method is CCPDMA (Complete Circumferential Peripheral and Deep Margin Assessment) which involves excision of the tumor with a margin <3 cms followed by histological evaluation by the pathologist and additional immunohistochemistry in doubtful cases. The reported recurrence is less than 10% ( 13 ). Currently, there are no randomized controlled trials or prospective studies available to determine the superiority of one surgical technique over the other.…”

Dermatofibrosarcoma Protuberans Vulvar. Tres casos clínicos de localización inusual.

“…Aunque al emplear esta técnica se reduce el área de tejido sano a extirpar, se recomienda el tratamiento multidisciplinar con cirugía plástica, sobre todo en zonas como el clítoris o introito vaginal, donde la reconstrucción juega un papel muy importante 11 .…”

Dermal Substitutes Use in Reconstructive Surgery for Skin Tumors: A Single-Center Experience

“…Scalp defects can also be covered using delayed local flaps and tissue expansion. However, in the oncological patient who requires resection, tissue expansion should be used carefully as a reconstructive option since the tumor resection is not delayed because of use of the expansion [ 6 , 17 ].…”