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.
背景：外阴隆突性皮肤纤维肉瘤是一种极为罕见的疾病。其罕见性可能影响治疗质量，因此需要更深入的认识以制定恰当的治疗方案。本综述旨在通过分析文献数据，获取关于该疾病规范管理的循证依据。 方法：我们系统检索了文献数据库，检索词包括“隆突性皮肤纤维肉瘤”“外阴”及其组合形式。从PubMed和MEDLINE两个英文数据库中筛选已发表文献，并通过分析参考文献纳入其他潜在相关研究。 结果：共纳入39篇文献，累计报告68例病例，均为回顾性病例报告及病例系列研究。外阴隆突性皮肤纤维肉瘤具有局部复发倾向，早期准确的病理诊断与恰当的手术方案对确保切缘阴性、实现最佳治愈效果至关重要。 结论：尽管本病呈惰性进展且总体预后良好，但规范治疗有助于降低局部复发率，避免影响患者生活质量。强烈建议组建多学科团队进行综合诊疗。
Dermatofibrosarcoma Protuberans of the Vulva: A Review of the MITO Rare Cancer Group
肿瘤(癌症)患者之家