Clinical Features and Treatment of Dermatofibrosarcoma Protuberans Affecting the Vulva: A Literature Review.
BACKGROUND
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.


OBJECTIVE
To review the available literature and discuss the clinical course of DFSP affecting the vulva.


METHODS
We reviewed the existing English-language literature on DFSP of the vulva with respect to clinical presentation, diagnosis, treatment, and outcome.


RESULTS
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.


CONCLUSIONS
This literature review comprehensively reviews and describes the clinical presentation of vulvar DFSP and the treatment options for this rare vulvar neoplasm.
*Department of Medicine, Division of Dermatology, Creighton University School of Medicine, Omaha, Nebraska; †Department of Biology, Loyola Marymount University, Los Angeles, California.
Austin H
AH
Nguyen
Shannon Q
SQ
Detty
Maria I
MI
Gonzaga
Christopher
C
Huerter
D018223:Dermatofibrosarcoma / Q000175:diagnosis* / Q000628:therapy*; D005260:Female; D006801:Humans; D012878:Skin Neoplasms / Q000175:diagnosis* / Q000628:therapy*; D014846:Vulvar Neoplasms / Q000175:diagnosis* / Q000628:therapy*