2026 Volume 44 Issue 2 Pages 167-172
Synopsis: Apocrine adenocarcinomas of the vulva are exceedingly rare. A nulliparous woman in her 60's presented with an enlarged, right-sided labia majora vulvar mass measuring approximately 5 cm. A biopsy confirmed apocrine adenocarcinoma. Systemic examination revealed bilateral inguinal lymph node metastases. The patient had difficulty with hip abduction during flexion due to Parkinson's disease. Radiotherapy (60 Gy/30 Fr) was administered to the primary tumor, bilateral inguinal and pelvic lymph node metastasis as an alternative to surgical resection. Treatment was completed without major complications. The primary tumor decreased to 5 mm in diameter, and the bilateral inguinal lymph nodes resolved. Although the mass continued to shrink for approximately one year following treatment, it later enlarged, suggesting local recurrence. Immunostaining was positive for estrogen receptor; thus, an aromatase inhibitor was used off-label with ethics committee approval. However, this therapy was ineffective, and the patient opted for best supportive care. Due to the rarity of vulvar apocrine adenocarcinoma, no standard treatment exists for this condition. Surgery is generally the first-line treatment; however, in this case, surgery was not feasible, and radiotherapy and hormonal therapies were pursued. Further case studies are needed to establish optimal treatment strategies.
