Angiomyxoma is a rare, slow-growing myxoid neoplasm that occurs almost exclusively in the genital, perineal, and pelvic regions of adult women. It mostly occurs during the reproductive years. It has propensity for local recurrence [1–4].
Macroscopically, they are soft, partly circumscribed, polypoids; across cross section, they have a glistening, homogeneous, gelatinous appearance; and sizes range from a few cm to 20 cm. The typical characteristics are slow growth, gelatinous appearance and locally infiltrative without evidence of nuclear atypia or mitosis [4, 5]. Treatment consists of local excision with tumour-free margins; however, local recurrence is high inspite of apparently complete surgical resection [5, 6].
A 30-year-old woman, P3?0, presented on 20 July 2009, with a chief complaint of painless vulvar mass. The mass had been first noticed 1 year back, which gradually enlarged to the size of 15–20 cm without any remarkable symptoms.
She had discomfort in her routine activities due to the weight and size of mass. Her past history included surgical excision of vulvar mass measuring located at the left major labium 8 years ago, told by her postoperatively, which she did not consider it as significant. She had conceived two times after appearance of growth on vulva. Her menstrual history was normal in terms of duration, flow and frequency.
On local examination, a nontender, polypoidal growth of 15 9 18 cm size was found to arise from left labia majora. Superficial decubitus ulcerations were seen on lower side of growth.
On perspeculum examination, erosion was seen on lower lip of cervix with slightly thick mucoid discharge.
On pervaginum examination, uterus was anteverted, nor- mal in size, mobile, nontender, bilateral adnexa were clear.
Clinically, a diagnosis of leiomyomatous polyp was made, and polypectomy was done.
Gross specimen showed skin covered bosselated tissue mass 18 9 15 9 11 cm with coarse papillary projections (Fig. 1). Cut sections of mass showed gray white area and peripheral area showed myxoid change. A small cystic area filled with mucoid fluid material was seen.
Microscopic examination of tumour revealed as angiomyxofibromatous polyp, as the tumour was composed of spindle and stellate-shaped cells in a myxoid matrix. These cells had eosinophilic cytoplasm and lacked significant nuclear pleomorphism and mitosis. Variable-sized thin- walled capillaries and thick-walled vascular channels were also seen. Some of these vessels showed perivascular hyalinization of their vascular walls (Fig. 2).
These tumours have to be differentiated from angio- myofibroblastoma. These are small, well-circumscribed tumours composed of plump epitheloid cells arranged in perivascular distribution and are not aggressive locally. The distinction between these two entities is of clinical importance, as aggressive angiomyxoma has a much higher risk of recurrence than angiomyofibroblastoma—has a lesion that is usually cured following simple excision.
Aggressive angiomyxoma was first described by Steeper and Rosai in 1983 [7]. The peak incidence is during the third decade of life [1]. It generally involves the genital, perineal, and pelvic region, with vulvar region being the most common site of involvement [4]. Although slowly growing, these lesions aggressively infiltrate the periva- ginal and perirectal soft tissues [2, 3. Imagings such as by computed tomography, ultrasonograph, and bone scan can be performed before surgery since the real extent of tumour is often underestimated by examination, and invasion into the bladder, gastrointestinal tract, and bone has been described [6]. Our patient was not subjected to radiological investigation as its clinical appearance was that of a benign polyp, and she concealed a previous history of surgical excision of mass which she told postoperatively.
The treatment of choice consists of surgical excision with wide tumour-free margins [3]. However, local recur- rences is up to 70 % of cases may be seen after adequate surgical resection [6].
