Mancari Rosanna, Cioffi Raffaella, Magazzino Francescapaola, Attademo Laura, Sant'angelo Miriam, Taccagni Gianluca, Mangili Giorgia, Pignata Sandro, Bergamini Alice
Gynecologic Oncology Unit, IRCCS Regina Elena National Cancer Institute, 00144 Rome, Italy.
Department of Obstetrics and Gynaecology, San Raffaele Scientific Institute, 20132 Milan, Italy.
Cancers (Basel). 2024 Jan 3;16(1):222. doi: 10.3390/cancers16010222.
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.
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.
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.
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.
外阴隆突性皮肤纤维肉瘤是一种极其罕见的疾病。其罕见性可能会影响治疗质量；需要更深入的了解来规划适当的治疗方案。本综述的目的是分析文献中报道的数据，以获取有关适当疾病管理的证据。
我们对文献进行了系统检索，检索词包括“隆突性皮肤纤维肉瘤”“外阴”和“外阴的”，单独或组合使用。我们从两个电子数据库PubMed和MEDLINE中筛选出以英文发表的文章，并分析其参考文献列表以纳入其他可能相关的研究。
我们选择了39篇文章，共报道了68例病例；这些都是回顾性病例报告和病例系列。外阴隆突性皮肤纤维肉瘤易于局部复发；早期及时的病理诊断以及适当的手术方法对于确保切缘阴性并最大限度地提高治愈潜力至关重要。
即使这是一种进展缓慢的疾病，总体预后良好，但适当的管理可能有助于降低可能影响患者生活质量的局部复发率。强烈建议由多学科团队进行管理。
