Year : 2008 | Volume
: 51 | Issue : 2 | Page : 259--260
Aggressive angiomyxoma of the vulva presenting as a pedunculated swelling
Shramana Mandal, Kajal Dhingra, Somak Roy, Nita Khurana
Department of Pathology, Maulana Azad Medical College, New Delhi, India
Department of Pathology, Maulana Azad Medical College, New Delhi - 110 002
Aggressive angiomyxoma is a rare, locally aggressive soft tissue tumor that has high propensity for local recurrence. It involves mainly the pelvis, vulva, perineum, vagina and urinary bladder in adult women in the reproductive age. Considering its locally aggressive nature, appropriate management and long-term follow-up is necessary. We describe a case of a 22-year-old young pregnant patient presenting with a large pedunculated swelling on the left labia majora.
|How to cite this article:|
Mandal S, Dhingra K, Roy S, Khurana N. Aggressive angiomyxoma of the vulva presenting as a pedunculated swelling.Indian J Pathol Microbiol 2008;51:259-260
|How to cite this URL:|
Mandal S, Dhingra K, Roy S, Khurana N. Aggressive angiomyxoma of the vulva presenting as a pedunculated swelling. Indian J Pathol Microbiol [serial online] 2008 [cited 2020 Jun 3 ];51:259-260
Available from: http://www.ijpmonline.org/text.asp?2008/51/2/259/41677
Aggressive 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. Although this tumor has bland histological features, it has propensity for local recurrence. ,,,
A 22-year-old patient presented with 4 months' amenorrhea and a slowly growing mass in the left labia majora. The patient was apparently well 4 months back, when she noticed a small pea-size swelling on the left labia majora, which was progressively increasing in size. The mass measured 7 cm in diameter and was attached to the labia by a stalk.
Gross examination showed a well-circumscribed pedunculated skin-covered mass measuring 7 cm in diameter with an attached stalk measuring 2.5 × 1 cm and having a length of 1 cm. The overlying skin showed an ulcer measuring 4 × 1.5 cm over the dependent part. The cut surface was gray-white, homogeneous and had a gelatinous appearance [Figure 1a, b].
Microscopically the tumor was composed of spindle and stellate-shaped cells in a myxoid matrix. These cells had eosinophilic cytoplasm and lacked significant nuclear pleomorphism and mitosis. Also seen were variable-sized thin-walled capillaries and thick-walled vascular channels. Some of these vessels showed perivascular hyalinization of their vascular walls [Figure 1c, d]. The tumor showed weak positivity for acidic mucin (Alcian blue positive) [Figure 1e]. The tumor cells were positive for estrogen receptor (ER), progesterone receptor (PR), vimentin and smooth muscle actin (SMA); and were negative for desmin. Based on these histological features, a diagnosis of aggressive angiomyxoma was made.
Aggressive angiomyxoma was first described by Steeper and Rosai in 1983.  It mostly occurs in women in the reproductive age. The peak incidence is during the third decade of life, suggesting that estrogen may stimulate its growth.  It generally involves the genital, perineal and pelvic region, with vulvar region being the most common site of involvement.  Tumors occurring during pregnancy have a rapid growth as there is a state of increased estrogen and progesterone production during this period.  Our patient was also a young pregnant female who presented with a pedunculated mass in the left labia majora.
On CT scan, these tumors have a well-defined margin with attenuation less than that of muscle. On MRI, these tumors show high signal intensity on T2-weighted images. The attenuation on CT and high signal intensity on MRI are likely to be related to the loose myxoid matrix and high water content of angiomyxoma.  Our patient was not subjected to radiological investigation as its clinical appearance was that of a benign polyp.
Grossly these tumors are soft, partly circumscribed, polypoidal lesions with gelatinous appearance on cut section. Microscopically these lesions are composed of many thick-walled vessels of varying sizes in a loose myxoid and collagenous stroma with spindle and stellate-shaped neoplastic cells. Immunohistochemically these tumors express ER and PR, thus suggesting that they may be hormone dependent.  The tumor cells also express vimentin, desmin and SMA and are negative for S-100.  In the present case also, the tumor cells were positive for ER, PR, vimentin and SMA.
These tumors have to be differentiated from angiomyo-fibroblastoma. These are small, well-circumscribed tumors composed of plump epitheloid cells arranged in perivascular distribution and are not aggressive locally.
Aggressive angiomyxomas are slowly growing and are locally invasive. The excision of these tumors is difficult as they have the same consistency as that of normal connective tissue and therefore have a propensity for local recurrence (36-72%). In this case, the lesion being pedunculated, the excision was complete and the postoperative period was uneventful. There has been no evidence of any local recurrence. Hence long-term follow-up is necessary and magnetic resonance imaging is the preferred method for detecting recurrences. ,
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