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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 30  |  Issue : 1  |  Page : 58-60

Giant aggressive angiomyxoma of the vulva


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 Publication5-Feb-2016

Correspondence Address:
Banyameen Iqbal
Department of Pathology, Dr. D. Y. Patil Medical College, Hospital and Research Centre, Pimpri, Pune, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-4958.175855

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  Abstract 

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

How to cite this URL:
Kumar H, Iqbal B, Dogra BB, Chandanwale S. Giant aggressive angiomyxoma of the vulva. J Med Soc [serial online] 2016 [cited 2019 Dec 13];30:58-60. Available from: http://www.jmedsoc.org/text.asp?2016/30/1/58/175855


  Introduction Top


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. [1] 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. [2] 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. [1]


  Case Report Top


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 1: Skin covered bilateral soft tissue tumor of vulva

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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 Top


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, [1] 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. [3] "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." [4] 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. [5] 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." [6] 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. [7] 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. [8] 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. [9]


  Conclusion Top


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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Steeper TA, Rosai J. Aggressive angiomyxoma of the female pelvis and perineum. Report of nine cases of a distinctive type of gynecologic soft-tissue neoplasm. Am J Surg Pathol 1983;7:463-75.  Back to cited text no. 1
[PUBMED]    
2.
Haldar K, Martinek IE, Kehoe S. Aggressive angiomyxoma: A case series and literature review. Eur J Surg Oncol 2009;10:10-6.  Back to cited text no. 2
    
3.
Tavassoli FA, Devilee P. Pathology and Genetics: Tumours of the Breast and Female Genital Organs. World Health Organization Classification of Tumours. Lyon: IARC Press; 2003. p. 329.  Back to cited text no. 3
    
4.
Nucci MR, Weremowicz S, Neskey DM, Sornberger K, Tallini G, Morton CC, et al. Chromosomal translocation t(8;12) induces aberrant HMGIC expression in aggressive angiomyxoma of the vulva. Genes Chromosomes Cancer 2001;32:172-6.  Back to cited text no. 4
    
5.
Alameda F, Munné A, Baró T, Iglesias M, Condom E, Lloreta-Trull J, et al. Vulvar angiomyxoma, aggressive angiomyxoma, and angiomyofibroblastoma: An immunohistochemical and ultrastructural study. Ultrastruct Pathol 2006;30:193-205.  Back to cited text no. 5
    
6.
Behranwala KA, Thomas JM. 'Aggressive' angiomyxoma: A distinct clinical entity. Eur J Surg Oncol 2003;29:559-63.  Back to cited text no. 6
    
7.
Güngör T, Zengeroglu S, Kaleli A, Kuzey GM. Aggressive angiomyxoma of the vulva and vagina. A common problem: Misdiagnosis. Eur J Obstet Gynecol Reprod Biol 2004;112:114-6.  Back to cited text no. 7
    
8.
Amin A, El Badawy S, Bull A. Aggressive angiomyxoma of the vulva. J Obstet Gynaecol 2013;33:325-6.  Back to cited text no. 8
    
9.
McCluggage WG, Jamieson T, Dobbs SP, Grey A. Aggressive angiomyxoma of the vulva: Dramatic response to gonadotropin-releasing hormone agonist therapy. Gynecol Oncol 2006;100:623-5.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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