|Year : 2016 | Volume
| Issue : 1 | Page : 58-60
Giant aggressive angiomyxoma of the vulva
Harsh Kumar1, Banyameen Iqbal1, Bharat Bhushan Dogra2, Shrish Chandanwale1
1 Department of Pathology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Pimpri, Pune, Maharashtra, India
2 Department of Plastic Surgery, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Pimpri, Pune, Maharashtra, India
|Date of Web Publication||5-Feb-2016|
Department of Pathology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Pimpri, Pune, Maharashtra
Source of Support: None, Conflict of Interest: None
Aggressive angiomyxoma (AA) is rare, soft, myxoid, mesenchymal neoplasm arising in the Pelvis and Perineal regions, which is locally aggressive. We are reporting a case of a 17-year-old female who presented with a well-circumscribed pedunculated polypoidal mass in the right labium majora diagnosed to be AA. Surgical excision was done, and histopathological examination confirmed it to be AA.
Keywords: Aggressive angiomyxoma, Soft-tissue tumor, Vulval tumors
|How to cite this article:|
Kumar H, Iqbal B, Dogra BB, Chandanwale S. Giant aggressive angiomyxoma of the vulva. J Med Soc 2016;30:58-60
| Introduction|| |
Aggressive angiomyxoma (AA) is a soft, myxoid, mesenchymal neoplasm arising in the pelvis and perineal regions, which is locally aggressive and rare. Steeper and Rosai, in 1983, first described its histologic characteristics and its tendency to recur and infiltrate locally.  It always involves the vulvovaginal, perineal, and pelvic regions of reproductive age group women. Until date, there have been only 250 cases reported in literature.  The term "aggressive" denotes its ability for local aggression and recurrence after excision. AA has a low tendency to metastasize. Until now, there has been no final conclusion on its pathogenesis, but a fibroblastic/myofibroblastic origin has been proposed. 
| Case Report|| |
A 17-year-old female presented in the Department of Gynecology with a mass on the right labium majora which is slowly growing for the last 4 months. Local examination was done which showed a well-circumscribed pedunculated mass measuring about 7.5 × 4.5 cm. On examination, it was nontender, soft and spongy in consistency. It was skin covered, outer surface was rough, cut surface had a smooth, gelatinous, and pale appearance [Figure 1]. Vagina, cervix, and uterus were normal and healthy. The tumor was surgically removed and sent for histopathology examination. Histopathology examination shows spindle and stellate mesenchymal cells with no atypia, embedded in a loose myxoid stroma. Small to medium sized blood vessels were noted in the stroma with extravasated red blood cells in the stroma. No evidence of any nerve hypertrophy was seen. Focal collections of the perivascular mononuclear cell infiltrate are seen in [Figure 2]. Immunohistochemistry studies revealed S-100 negative [Figure 3], CD34 positive [Figure 4], and smooth muscle actin (SMA)-focal positivity in the vessel wall. It was diagnosed to be angiomyxoma of the vulva.
|Figure 2: Photomicrograph showing high power view of the stroma with spindle and stellate mesenchymal cells without atypia (H and E, ×40)|
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|Figure 3: Photomicrograph showing immunohistochemical stain S-100 negativity|
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|Figure 4: Photomicrograph showing immunohistochemical stain CD34 positivity|
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| Discussion|| |
AA is a distinct and uncommon, mesenchymal tumor with a predilection for pelvis and perineal regions, especially in females and less frequently in males. Steeper and Rosai,  in 1983, were the first to report nine cases of this pelvic neoplasm and described them as AA. In 2003, this term was re-classified by the World Health Organization as deep angiomyxoma.  "The pathogenesis of AA is not clear. Some studies have demonstrated a definite translocation at the level of chromosome 12 with a consequent aberrant expression of the high mobility group protein isoform I-C involved in DNA transcription."  AA is believed to be a hormonally responsive tumor and is positive for estrogen receptor and/or progesterone receptor. It is thought to arise from mesenchymal cells of the pelvic region and/or perineal region, especially in females. A few studies also suggest AA to arise from multipotent perivascular progenitor cells as it often shows variable myofibroblastic and fibroblastic features.  AA also shows positivity for CD34 and SMA and negativity for S-100 as in this case. AA is a locally invasive neoplasm, and it needs to be differentiated from benign lesions which are known to have a low risk of recurrence and also from fully malignant myxoid tumors. The differential diagnosis "ranges from benign tumors such as myxolipoma, myxoid neurofibroma, and myxoid leiomyoma to myxofibrosarcoma, myxoid variant of liposarcoma, leiomyosarcoma, malignant fibrous histiocytoma, and botryoid rhabdomyosarcoma."  This neoplasm may also be clinically misdiagnosed as vaginal polyps, myxoma, lipoma, vulvar mass, vulvar abscess, Bartholin's cyst, Gartner's duct cyst, vaginal cyst, vaginal prolapse, pelvic floor hernia, fibromatosis, and other benign and malignant soft tissue tumors of the pelvis and perineum.  Surgical excision is the preferred treatment. However, this tumor carries a high rate of recurrence after complete excision due to local infiltration. There are reports of around 50-70% of patients having recurrence after surgical resection.  Radiotherapy and chemotherapy are not useful because of low mitotic activity in these tumors. Various methods have been tried to lower the chances of recurrences and are even successful. Certain hormonal therapies with tamoxifen, raloxifene, or gonadotropin-releasing hormone analogs are useful as they have been seen to reduce tumor size. Complete excision of large tumors is now possible because of hormonal therapy, which even controls the recurrence in these aggressive tumors. 
| Conclusion|| |
AA is a very uncommon, aggressive, mesenchymal tumor with a preference for perineal areas in females and less commonly in males. Treatment of choice is excision, but the tumor carries a high risk of recurrence after complete excision. Various hormonal therapies have been tried to reduce the chances of recurrences after excision and some have even been useful.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]