Aggressive Angiomyxoma Jssn

JSSN 2008; 11 (2) : 23-25

Aggressive angiomyxoma of vulva extending into pelvis A case report Paban Sharma MD, consultant, Gyne/Obst department, Patan Hospital Bhaktabatsal Raut MD resident, Gyne/Obst department, Patan Hospital Correspondence to: [email protected] Abstract: Aggressive angiomyxoma is a rare, locally aggressive soft tissue tumor that chiefly 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 aggressive angiomyxoma. Key words: Aggressive Angiomyxoma, Vulvo-perineal Resection.

Introduction: Aggressive angiomyxoma is a rare, locally aggressive benign soft tissue tumor chiefly involving vulvo-perineal region of young female patients. It was first reported as a distinct variant of myxoid neoplasm in female vagina and pelvis by Steeper and Rosai in 1983(1). These lesions have predilection for female pelvic soft parts, slowly growing with frequent recurrences. Many authors 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 around the genital tract such as perineum, perianal region, scrotum, inguinal region

and pelvic soft tissues are affected (2). Signs and symptoms at presentation may include discomfort from mass, visible mass, and pressure effects on adjacent organs. Clinically this tumor most frequently diagnosed as differentials of vulval mass and the reports are also available on its multifocal presentation.

Case report: A 23 years lady, Para 2, from Baglung district of Western Nepal, presented to Patan hospital OPD on March 2007(2064/1/3rd) with complaint of swelling in the left vulval region for the duration of four years. The swelling was 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 the left vulva about 6×6cm external dimension. The mass was soft to firm in consistency, slight restriction of mobility, non tender, without any impulse on coughing and non reducible. All the other systemic examination findings are 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 mass gradually dissected out from adjacent tissues, but as the procedure proceeded its deeper extension detected extending up into the pelvis. Because of its deeper extension into the pelvis, laparotomy was done with the help of surgeon to approach the retropubic space. Finally, the whole mass was enucleated by doing simultaneous dissection from above and below. The mass 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 was uneventful. She was discharged on 7th 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 suggesting Aggressive angiomyxoma of the vulva. There was no atypia or mitotic activity.

Figure 1. An elliptical incision given over the mass.

Initial mass seen in vulva

Figure 2. Showing extension to deep pelvis.

Figure 3. Complete mass after excision.

Discussion: Aggressive angiomyxoma (AAM) is a rare mesenchymal neoplasm that usually occurs in the pelvic-perineal region. This is basically a disease of reproductive age but has been reported in the extremes of age. Although this is locally infiltrating tumor, distant metastasis have been reported even in the lungs(6). Diagnosis of aggressive angiomyxoma clinically is difficult as its incidence is low and its presentation mimics a lot of differentials of vulvovaginal mass such as Bartholin’s cyst, abscess, vaginal cyst, lipoma, levator and perineal hernia. The final diagnosis only can be made after histopathology report. (5)

In our case also, the initial diagnosis made clinically was fibrolipoma and thought to be a simple benign local mass. In a case report by A Kaur and colleagues in Karnataka, India (2000), mentioned that aggressive angiomyxoma was detected in a case who was admitted with a diagnosis of left bartholin cyst. The case was also found to have an additional mass on opposite side with multifocal presentation. (1) Etiology of this disease is not known, but the translocation between arms of chromosomes have been reported (7) On gross examination these tumors are soft, bulky masses with homogenous glistening surface. Maximam reported weight of the resected tumar mass was 19.8 kg(8). Differential diagnosis histologically ranges from benign tumors such as myxolipoma, myxoid neurofibroma, and myxoid leiomyomato respective sarcomas. The distinctively striking vascular component in aggressive angiomyxoma helps in ruling out most of the above mentioned neoplasms as differentials. At pathologic analysis, aggressive angiomyxoma are poorly circumscribed lesions composed of stellate and spindle shaped cells distributed in a myxoid matrix. The lesions have abundant supply of blood vessels that are often thickened and hyalinized. (4) Most of the cases of aggressive angiomyxoma of pelvic parts in female patients exhibits positivity for estrogen and progesterone receptor, however it can not be used to distinguish aggressive angiomyxoma from its biological mimics. Hormone reactivity is significant in that aggressive angiomyxoma may arise from specialized hormonally responsive stromal cells of perineum and may potentially play a therapeutic role in unresectable tumors in the future (3). Surgery with margin-free excision is considered the treatment of choice. Other treatment modalities are the use of hormone antagonists, such as tamoxifen, or GnRH analogs, Radiotherapy and also selective angiographic embolism. High recurrence rate of the tumor, up to 70% within a 2-year period, makes long-term imaging follow-up of the patient necessary(4) and MRI seems to be the appropriate investigation. Conclusion: Aggressive angiomyxoma is a rare, benign neoplasm that can be mistaken both clinically and on microscopy for several other conditions The tumor is

locally infiltrative, multifocal sometimes requiring complete clinical examination and investigations for proper diagnosis. Clinicians should consider the diagnosis of aggressive angiomyxoma when a patient presents with an atypical vulvo-perineal mass, as an incorrect diagnosis may lead to repeated surgical procedures.

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