Nguyen Austin H, Detty Shannon Q, Gonzaga Maria I, Huerter Christopher
*Department of Medicine, Division of Dermatology, Creighton University School of Medicine, Omaha, Nebraska; †Department of Biology, Loyola Marymount University, Los Angeles, California.
Dermatol Surg. 2017 Jun;43(6):771-774. doi: 10.1097/DSS.0000000000001113.
Dermatofibrosarcoma protuberans (DFSP) is a low-to-intermediate grade cutaneous neoplasm with a low propensity for metastasis and a high rate of local recurrence. It typically presents as a dermal plaque or nodule on the trunk, limbs, or head and neck region. Vulvar DFSP has also been described, although it is less common.
To review the available literature and discuss the clinical course of DFSP affecting the vulva.
We reviewed the existing English-language literature on DFSP of the vulva with respect to clinical presentation, diagnosis, treatment, and outcome.
Thirty three case reports and series were included (n = 54 patients). Vulvar DFSP most commonly presents as a slowly enlarging tender or asymptomatic mass on the labia majora, with histological findings of classic DFSP. Most patients were treated with wide local excision. Three patients were treated with Mohs micrographic surgery, which may decrease local recurrence and seems well suited for use in vulvar DFSP.
This literature review comprehensively reviews and describes the clinical presentation of vulvar DFSP and the treatment options for this rare vulvar neoplasm.
隆突性皮肤纤维肉瘤（DFSP）是一种低至中级别的皮肤肿瘤，转移倾向低，但局部复发率高。它通常表现为躯干、四肢或头颈部的皮肤斑块或结节。外阴DFSP也有报道，不过较为少见。
回顾现有文献并讨论累及外阴的DFSP的临床病程。
我们回顾了关于外阴DFSP的现有英文文献，内容涉及临床表现、诊断、治疗及预后。
纳入了33篇病例报告及系列研究（n = 54例患者）。外阴DFSP最常见的表现是大阴唇上缓慢增大的压痛性或无症状肿块，组织学表现为典型的DFSP。大多数患者接受了广泛局部切除。3例患者接受了莫氏显微外科手术，该手术可能会降低局部复发率，似乎很适合用于外阴DFSP。
这篇文献综述全面回顾并描述了外阴DFSP的临床表现以及这种罕见外阴肿瘤的治疗选择。
