Deep Aggressive Angiomyxoma of Pelvic Soft Tissue: A Rare Case Report

Excerpt

Deep aggressive angiomyxoma (DAA) is a rare benign tumor with about 250 cases being reported worldwide. It was first reported as a distinct variant of myxoid neoplasm in female pelvis by Steeper and Rosai in 1983. It is a locally aggressive tumor of unknown histogenesis having high potential for local infiltration into adjacent skeletal muscles and adipose tissue. It is commonly seen in females during reproductive age group with peak incidence in the fourth decade. DAA is most often found in pelvic, perineum, genital, or inguinal region. Its exact etiology is not known, but in some cases, chromosomal aberrations have been reported. Most of these tumors are slow growing, presenting as pelvic masses with or without pressure symptoms on surrounding structures. These lesions are soft unencapsulated tumors with fingerlike projections into surrounding structures. Wide surgical excision is the mainstay of managing these tumors since tumor projections increase chances of recurrence. Immunohistochemical evaluations of these tumors show positivity to estrogen and progesterone receptors. Hence, GnRH therapy may be helpful in difficult cases [1, 2]. …