Aggressive Angiomyxoma: An Unusual Female Pelvic Tumour. Report of Three Cases and Review of the Literature

Juan Antonio Martín-Cartes,María del Carmen Palacios-González,Virginia Gómez-Cabeza de Vaca,Manuel Bustos-Jiménez,Antonio Muñoz Ortega,María Jesús Tamayo-López

doi:10.4236/ss.2010.12008

Abstract

Aggressive angiomyxoma was first described in 1983 by Steeper and Rosai, and fewer than 150 cases have been reported in the world medical literature. It is a soft-tissue tumour of the pelvis and perineum. The recurrence rate is high, and often extensive resections are performed with considerable morbidity. These tumours are benign, locally infiltrative mesenchymal neoplasms with a predilection for the female pelvis and perineum and they usually tend to recur. Furthermore, these tumours often reach too large dimensions before becoming clinically symptomatic; their incidence is higher in women of the reproductive age group; however a few cases of its occurrence outside the pelvis have also been reported. In this study, we reported three cases with aggressive pelvic angiomyxoma treated with surgical methods and used an approach that described by Kraske in order to get access to lower rectal cancers. Accurate preoperative diagnosis should alert the surgeon to the need for wide excision, which is essential for prevention of local recurrence.

Highlights

We reported three cases with aggressive pelvic angiomyxoma treated with surgical methods and used an approach that described by Kraske in order to get access to lower rectal cancers
Accurate preoperative diagnosis should alert the surgeon to the need for wide excision, which is essential for prevention of local recurrence
Aggressive angiomyxoma is a rare tumour of mesenchymal origin first described in 1983 by Steeper and Rosai [1,2] like a distinct mesenchymal tumour of the female pelvis and perineum

Summary

Introduction

Aggressive angiomyxoma is a rare tumour of mesenchymal origin first described in 1983 by Steeper and Rosai [1,2] like a distinct mesenchymal tumour of the female pelvis and perineum. It has later been reported in males. The tumour presents as a large multilobular or polypoid mass or swelling. On gross examination it is rubbery and white or soft and gelatinous. Hormonal suppression seems to be a plausible treatment option because these tumours occur predominantly in premenopausal women of reproductive age, may grow rapidly during pregnancy, and have been shown to express immunohistochemical positivity for estrogenic and progesteronic receptors [12]

Material and Methods

Results

Discussion