Aggressive Angiomyxoma : Introduction

Introduction: Aggressive angiomyxoma (deep angiomyxoma) is a rare, locally aggressive mesenchymal neoplasm that most often occurs in the vulvovaginal region, perineum, and pelvis of females. The patients are usually in their 2nd or 3rd decade of life. It presents as a polypoid or pedunculated labial mass which may clinically resemble Bartholin gland cyst, lipoma, or hernia. Similar tumor has been reported in the scrotal region in males. There is a superficial counterpart known as superficial angiomyxoma which is more circumscribed and less likely to recur.

Gross Pathology: It is poorly circumscribed and infiltrative with an edematous, gelatinous cut surface. Recurrent tumors may be more fibrotic with a firm consistency.

Case History: The patient was a 20-year-old female who presented with a progressively enlarging pedunculated mass arising from the right labium majus. It was surgically excised. The specimen measured 2.2 x 1.8 x 1.2 cm and was covered with wrinkled skin. The cut surface showed an ill-defined mass with a homogenous whitish, gelatinous appearance. Microscopic examination confirmed the diagnosis of aggressive angiomyxoma of vulva.

Case courtesy of: Dr. Sanjay D. Deshmukh (Prof. of Pathology) and Dr. Sadhana Khaprde (Head of Pathology Dept.), Dr. Vithalrao Vikhe Patil Medical Foundation's Medical College & Hospitals, Ahmednagar, India.