Vulvar dermatofibrosarcoma protuberans, an unusual anatomical location

Area of Science:

• Oncology
• Dermatology
• Surgical Pathology

Background:

• Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade malignant skin tumor.
• Vulvar DFSP is exceptionally rare, with limited documented cases.
• Accurate diagnosis and complete surgical resection are crucial for managing DFSP.

Observation:

• A 38-year-old female presented with a painful left vulvar mass.
• Previous excision confirmed a benign spindle cell epithelioma; the current mass recurred and enlarged.
• Contrast-enhanced MRI revealed a well-defined, lobulated lesion near the urethra and clitoris.

Findings:

• The patient underwent wide local excision, radical vulvectomy, and lymphadenectomy.
• Postoperative complications included fever, swelling, and dysuria.
• Histopathology confirmed DFSP with all surgical margins negative.
• Follow-up MRI showed complete resolution and no recurrence.

Implications:

• This case underscores the importance of considering rare malignancies like DFSP in vulvar masses, even after prior benign diagnoses.
• Successful surgical management, including radical vulvectomy and lymphadenectomy, can lead to favorable outcomes.
• Multimodality imaging and thorough histopathological examination are vital for accurate diagnosis and treatment planning in complex vulvar lesions.