Dermatofibrosarcoma Protuberans of the Vulva: A Review of the MITO Rare Cancer Group

Simple Summary Rare diseases represent a major health problem, since patients face difficulties in obtaining a rapid diagnosis and appropriate treatments. Vulvar dermatofibrosarcoma protuberans is one of these rare entities, in which reaching a correct pathological diagnosis is intricate and surgical techniques are not standardised. The aim of our paper is to review the available literature on vulvar dermatofibrosarcoma protuberans to summarise previous experiences and main issues, in an attempt to improve the management of this rare disease. Dermatofibrosarcoma protuberans of the vulva needs to be diagnosed early and managed by a referral centre, where the patient can receive appropriate management: surgical treatment should aim to obtain free margins, lowering the probability of recurrence. Long-term follow up is needed, since recurrences are documented even after several years.Abstract Background: Vulvar dermatofibrosarcoma protuberans is an extremely rare disease. Its rarity can hamper the quality of treatment; deeper knowledge is necessary to plan appropriate management. The purpose of this review is to analyse the data reported in the literature to obtain evidence regarding appropriate disease management. Methods: We made a systematic search of the literature, including the terms "dermatofibrosarcoma protuberans", "vulva", and "vulvar", alone or in combination. We selected articles published in English from two electronic databases, PubMed and MEDLINE, and we analysed their reference lists to include other potentially relevant studies. Results: We selected 39 articles, with a total of 68 cases reported; they were retrospective case reports and case series. Dermatofibrosarcoma protuberans of the vulva tends towards local recurrence; an early and timely pathological diagnosis, together with an appropriate surgical approach, are of utmost importance to ensure free margins and maximise the curative potential. Conclusions: Even if this is an indolent disease and it generally shows a good prognosis, appropriate management may help in reducing the rate of local recurrences that may hamper patients' quality of life. Management by a multidisciplinary team is highly recommended.