Pedunculated Neoplasm of the Vulva

Area of Science:

• Dermatology
• Oncology
• Pathology

Background:

• Extramammary Paget disease (EMPD) is a rare malignancy of the skin, often mimicking eczematous conditions.
• It typically affects apocrine gland-rich areas like the perineum.

Observation:

• This report details an unusual case of EMPD in a 65-year-old woman presenting as a prominent pedunculated neoplasm.
• Initial misdiagnosis and treatment were followed by a confirmed biopsy revealing EMPD infiltration.

Findings:

• The patient underwent surgical excision but subsequently developed brain metastases, signifying a poor prognosis.
• The pathogenesis of EMPD is not fully understood, and differentiating primary from secondary forms is critical.

Implications:

• This case highlights the necessity of considering atypical presentations of EMPD.
• Timely diagnosis and intervention based on recognizing unusual presentations can potentially improve patient outcomes.