Vulvar Dermatofibrosarcoma Protuberans in a Postmenopausal Woman: A Rare Case Report and Insights Into Treatment.

Authors

Naw Phu Ngone Ko Ko, Ei Ei Phyo Naing

Published in

Cureus. Volume 18. Issue 11. Pages e118959. Epub Nov 01, 2026.

Abstract

Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous fibroblastic neoplasm characterized by locally infiltrative growth, a tendency for local recurrence, and low metastatic potential. Vulvar involvement is exceptionally uncommon and presents diagnostic and therapeutic challenges because of both its rarity and the anatomical importance of preserving vulvar function while obtaining adequate oncological clearance. We report the case of a 56-year-old postmenopausal woman, para 4, with a BMI of 32 kg/m², who presented with a 2 × 3 cm non-tender, freely mobile lesion of the left vulva. Excisional biopsy demonstrated a proliferation of spindle cells in a characteristic storiform pattern, with diffuse CD34 immunoreactivity, confirming DFSP. The closest histological margin following the initial excision was 0.3 cm. The patient was subsequently referred to a gynaecological oncologist, and following a multidisciplinary discussion, she underwent wide local re-excision with bilateral inguinofemoral lymphadenectomy. Histopathological examination demonstrated clear surgical margins, while all 13 lymph nodes showed reactive changes without evidence of tumour involvement. The case was discussed with a radiation oncologist, and adjuvant radiotherapy was not recommended after complete surgical excision and in the absence of residual or regional disease. No systemic therapy was administered. The patient remained free of documented recurrence for more than three years. This case highlights the importance of considering DFSP in the differential diagnosis of unusual vulvar masses, obtaining histopathological confirmation, and achieving complete excision with negative margins. It also illustrates the value of multidisciplinary management in rare vulvar soft-tissue tumours.

PMID:
42968853
Bibliographic data and abstract were imported from PubMed on 02 Nov 2026.

Read full publication at:
Please sign in to see all details.

Stats

1-terrible, 9-excellent. How would you rate this publication? Sign in in to submit your rating.

Post a comment

You need to be signed in to post comments. You can sign in here.

Comments

There are no comments yet.