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JSSN 2008; 11 (2) : 23-25

Aggressive angiomyxoma of vulva extending into pelvisA case report

Paban Sharma MD, consultant, Gyne/Obst department, Patan HospitalBhaktabatsal Raut MD resident, Gyne/Obst department, Patan HospitalCorrespondence to: paban.Sharma@gmail.com

Abstract:

Aggressive angiomyxoma is a rare, locally aggressive soft tissue tumor thatchiefly involves the vulvo-perineal and pelvic region of young female patients.A 23 years old lady, Para 2 from western Nepal presented with complaint of painless swelling of left vulva since 4 years. Abdomino- vulval resection performed for complete enucleation of mass weighing 1 Kg.Histopathological findings confirmed the diagnosis to be an aggressiveangiomyxoma.

Key words

Aggressive Angiomyxoma, Vulvo-perineal Resection.

Introduction:

Aggressive angiomyxoma is a rare, locally aggressive benign soft tissuetumor chiefly involving vulvo-perineal region of young female patients. Itwas first reported as a distinct variant of myxoid neoplasm in female vaginaand pelvis by Steeper and Rosai in 1983(1). These lesions have predilectionfor female pelvic soft parts, slowly growing with frequent recurrences. Manyauthors have subsequently reported this lesion in female and male patients.Men are affected in about 5% of the cases and a wide variety of sites aroundthe genital tract such as perineum, perianal region, scrotum, inguinal region

and pelvic soft tissues are affected (2). Signs and symptoms at presentationmay include discomfort from mass, visible mass, and pressure effects onadjacent organs. Clinically this tumor most frequently diagnosed asdifferentials of vulval mass and the reports are also available on itsmultifocal presentation.

Case report:

A 23 years lady, Para 2, from Baglung district of Western Nepal, presentedto Patan hospital OPD on March 2007(2064/1/3

) with complaint of swelling in the left vulval region for the duration of four years. The swellingwas painless, slowly progressing in size, producing discomfort in the perineum for last 2 months.Clinical examination of the patient revealed a round and uniform mass in theleft vulva about 6×6cm external dimension. The mass was soft to firm inconsistency, slight restriction of mobility, non tender, without any impulseon coughing and non reducible. All the other systemic examination findingsare within normal limits.The case was provisionally diagnosed to be fibrolipoma of left vulva and planned for excision on the next day.During operation, an elliptical incision given over the mass and the massgradually dissected out from adjacent tissues, but as the procedure proceededits deeper extension detected extending up into the pelvis. Because of itsdeeper extension into the pelvis, laparotomy was done with the help of surgeon to approach the retropubic space. Finally, the whole mass wasenucleated by doing simultaneous dissection from above and below. Themass was lying on left side of the urinary bladder pushing the bladder towards the right side and upward. Weight of the tumor was 1000gm.She had a brief hematuria just after the procedure. She received 2 units of blood in the post-operative period, otherwise post-operative period wasuneventful. She was discharged on 7

post-operative day. She came for follow op only once after 1 week, after that she did not turn up.Histopathology report showed a hypercellular myxoid stroma with the presence of stellate cells and large dilated blood vessels suggestingAggressive angiomyxoma of the vulva. There was no atypia or mitoticactivity.