Introduction DFSP is a rare sarcoma of soft tissues. Most of them of low grade and good prognosis. More frequent in trunk of males. Here we present a case vulvar DFSP.
Methods Review of clinical chart, imagining, and pathology.
Case A post menopausal 50y-old patient, nulliparous with no medical history who presented vulvar lesion of fast growing and bleeding. Physical examination evidenced a tumor of 20 cm. compromising the mount of venus to the left with ulcerated areas. MRI and CT-scan confirmed a vulvar solid mass of 12.5 cm. without deep infiltration and hemorrhagic areas. Pathology concluded myxoid-DFSP. Neoadjuvant radiotherapy was given to decreased size with good results. Then, radical hemivulvectomy was performed with selective groin dissection of a suspicious lymph node. Ultrafast biopsy technique was used meanwhile the wound was covered with moltopren, and 24h later deep close margin, was informed so amplification of margin and rectus flap was performed successfully.
Discussion DFSP of vulvar location represents <1% of cases. Has slow growing and rarely lymph node compromise and local recurrences. Pathology usually exhibits spindle cells, with CD34 IHC stain positivity, nevertheless 10 to 20% could have aggressive sarcomatous areas. The usual treatment is radical vulvectomy or Mohs technique. Owing the proximity of gentle structures margins status represents a challenge. For this reason, RT can be an alternative to reduce size before surgery as in this case. Reaching a sure oncological result and maintaining esthetics.
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