Apocrine Adenocarcinoma of the Vulva: A Case Report and Review of the Literature.

Kohei Aoyama,Jo Kitawaki,Morio Sawada,Satoru Yasukawa,Taisuke Mori,Hiroshi Matsushima

doi:10.1155/2016/1712404

Abstract

Primary vulvar adenocarcinomas are very rare. We describe the rare case of primary vulvar apocrine adenocarcinoma, a histologically rare subtype of vulvar adenocarcinoma. A 57-year-old Japanese woman presented with an enlarging vulvar mass. A dark-red, hemorrhagic, ulcerated tumor was on the right side of the anterior labial commissure measuring approximately 3.5 × 3.5 cm. Preoperative biopsy showed poorly differentiated carcinoma with partial differentiation to adenocarcinoma. Systemic examination revealed lymph node metastases in both inguinal regions and no other primary source. We performed radical vulvectomy and bilateral inguinal and pelvic lymphadenectomy. Histopathologic diagnosis was apocrine adenocarcinoma of the vulva with inguinal lymph node metastases, pT1bN2bM0. Surgical margins were negative. The patient received no adjuvant chemotherapy or radiation. Inguinal lymph node recurrence occurred after six months. Reresection and adjuvant tomotherapy were performed. After a further 12 months of observation, no rerecurrence was observed. The patient is now on follow-up.

Highlights

We describe the rare case of primary vulvar apocrine adenocarcinoma, a histologic subtype of vulvar sweat gland carcinoma, and investigate its histogenesis and molecular epidemiology with a literature review
We presented a rare case of primary vulvar apocrine adenocarcinoma
Primary vulvar adenocarcinomas are classified into sweat gland carcinomas, primary breast-like adenocarcinomas of the vulva, and extramammary Paget’s disease (EMPD)

Summary

Introduction

Primary vulvar cancer constitutes less than 5% of female genital tract malignancies, and most cases are squamous cell carcinoma. They are classified into sweat gland carcinomas, primary mammary-like adenocarcinomas, and extramammary Paget’s disease (EMPD). Because of their rarity, there is no consensus on their histogenesis, prognosis, or treatment strategies. We describe the rare case of primary vulvar apocrine adenocarcinoma, a histologic subtype of vulvar sweat gland carcinoma, and investigate its histogenesis and molecular epidemiology with a literature review

Case Report

Findings

Discussion