Abstract Purpose Extramammary Paget’s Disease (EMPD) is a rare malignancy commonly affecting anogenital and apocrine gland-rich areas. Perianal EMPD usually presents with skin involvement and, rarely, affects the anal mucosa. Patients typically experience non-specific symptoms such as itching, pain, and erythematous plaques. EMPD has a high recurrence rate and a risk of progression to malignancy. This case report presents a rare perianal EMPD case complicated by adenocarcinoma development. Methods A 58-year-old male patient with chronic perianal lesions was initially misdiagnosed and treated inadequately. Advanced imaging (MRI and PET-CT) confirmed malignancy suspicion, and biopsy revealed invasive adenocarcinoma arising from EMPD. Surgical management included wide local excision, inguinal and iliac lymph node dissection, and perianal reconstruction. Conclusion Early diagnosis and aggressive surgical treatment are crucial in reducing recurrence risk. Lymph node metastases and invasive components significantly impact prognosis. Long-term follow-up and research on molecular markers may contribute to better treatment approaches.
Yılmaz et al. (2025) studied this question.