Aggressive angiomyxoma of female vulva

GA Gore, LM Dandekar 
 Consultant Obstetrician and Gynecologist, Gourai Maternity and Nursing Home, Bina Apartments, Vazira Naka, Borivali (West), Mumbai - 400 091, India

Correspondence Address:
G A Gore
Consultant Obstetrician and Gynecologist, Gourai Maternity and Nursing Home, Bina Apartments, Vazira Naka, Borivali (West), Mumbai - 400 091
India

Full Text

Sir,

A 47-year-old woman presented with a vulval swelling since 4 years. It started as a warty growth on the lateral side of the left labium and increased to the present size. Local examination revealed a pedunculated nontender mass arising from the lateral side of the left labium. It was tri-lobed; 2 of them 14 Χ 7 cm and the third was about 4 Χ 2 cm. They were rubbery in consistency. There were several small warts on the skin round the base of the pedicle. There were no palpable nodes or any other masses either in the inguinal region or on pervaginal examination.

Patient was advised surgical excision of the same.

The mass was excised from its base, including the warty growth on the surrounding skin and with a wide free skin margin. Edges were approximated and closed.

Microscopic appearance: A paucicellular soft tissue tumor composed of small- and medium-sized vessels surrounded by a myxomatous component containing stellate or spindle tumor cells. There were fibrinoid changes in some of the vessels, and there was no mitotic activity in the stellate cells. The margin was free of tumor [Figure 1],[Figure 2],[Figure 3].

Aggressive angiomyxoma is a rare, locally infiltrative mesenchymal tumor, [1] found usually in women of reproductive age. It is a slow-growing, low-grade neoplasm involving the pelvis and perineum with a high risk for local recurrence. Microscopically, it shows hypocellular, myxoid tissue with numerous medium-sized vessels. Tumor cells are spindle cells, fibroblasts, and myofibroblasts, lacking significant nuclear atypia and mitotic activity. Cells are immunoreactive to muscle actin but do not show S-100 protein. Mesenchymal tumors present a diagnostic dilemma because a wide variety of them exhibit a myxoid stroma which can be so prominent that it is difficult to differentiate intramuscular myxoma and myxoid liposarcoma.

All cases of aggressive angiomyxoma of pelvic soft tissue in female patients exhibit positivity for estrogen receptor, ER and/or progesterone receptor PR .The presence of ER in the tumor and its enlargement in pregnancy [2] suggest the possibility of hormone dependence of this neoplasm. [2] This feature of hormone dependency also suggests that gonadotropin releasing hormone agonists, GnRH agonists, may be of value in managing cases of aggressive angiomyxoma, either primary or recurrent, which are not amenable to surgical excision. [3] GnRH agonists may also be used for reduction in size, so that more conservative surgery can be performed. Only a small number of cases have been documented in men.

In conclusion, aggressive angiomyxoma merits wider recognition as a distinctive tumor type and a long-term follow-up is required.

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