Angiomyxoma - A Rare Case Report

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Aggressive angiomyxoma is a rare, benign slow-growing
myxoid neoplasm that occurs almost exclusively in the genital, perineal and pelvic regions of adult women. It mostly occurs during the reproductive years with a peak incidence in the third decade of life. It has propensity for local recurrence,1,2 which varies 9e72%. Female:male ratio is 6:1.

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Angiomyxoma - A Rare Case Report

  1. 1. Angiomyxoma - A Rare Case Report
  2. 2. a p o l l o m e d i c i n e 1 0 ( 2 0 1 3 ) 1 6 2 e1 6 4 Available online at www.sciencedirect.com journal homepage: www.elsevier.com/locate/apme Case Report Angiomyxoma e A rare case report Megha Gupta*, Shashi Prateek, Renu Arora Vardhman Mahavir Medical College & Safdarjung Hospital, New Delhi 19, India article info abstract Article history: Aggressive angiomyxoma is a rare, locally aggressive soft tissue tumor that has high Received 15 August 2012 propensity for local recurrence. It involves mainly the pelvis, vulva, perineum, vagina and Accepted 18 February 2013 urinary bladder in adult women in the reproductive age. Considering its locally aggressive nature, appropriate management and long-term follow-up is necessary. We describe a case Keywords: Angimyxoma of a 19-year-old young unmarried girl presenting with a swelling on the left labia majora. Copyright ª 2013, Indraprastha Medical Corporation Ltd. All rights reserved. Benign Aggressive 1. Introduction Aggressive angiomyxoma is a rare, benign slow-growing myxoid neoplasm that occurs almost exclusively in the genital, perineal and pelvic regions of adult women. It mostly occurs during the reproductive years with a peak incidence in the third decade of life. It has propensity for local recurrence,1,2 which varies 9e72%. Female:male ratio is 6:1. 2. Case history A 19-year-old unmarried girl presented with a swelling in the left labia majora. The patient was taken up for marsupialization 2 years back in view of Bartholin’s cyst which was found to be an angiomyxoma on histopathology. She reported to our hospital in view of recurrence of swelling after 1 year which gradually increased to 4 Â 5 cm in posterior one-third of labia majora resembling a Bartholin’s cyst. On P/V/R: soft cystic mass was extending along the left paravaginal area reaching upto fornix, upper margin of which could be reached. CT and MRI showed a mass in left labia majora in posteroinferior region infiltrating into pelvic floor and pelvic cavity, displacing and abutting vagina, anus medially & levator ani laterally, left pelvic cavity seen abutting the left lateral wall of uterus. Wide excision of tumor was done by small labial incision (Figs. 1e2) and after closure no disfigurement seen (Fig. 3) (in most of the reported cases there was disfigurement). Gross and cut section showed a non capsulated graye white homogeneous gelatinous mass of 20 Â 6 Â 3 cm (Fig. 4). On histopathology: Spindle and stellate-shaped cells in a myxoid matrix with eosinophilic cytoplasm. These cells lack a significant nuclear pleomorphism and mitosis with variablesized vascular channels. Some of these vessel walls had perivascular hyalinization (Figs. 5, 6 and 7). The tumor cells were positive for estrogen receptor (ER), progesterone receptor (PR), and vimentin suggestive of aggressive angiomyxoma. 3. Discussion Aggressive angiomyxoma was first described by Steeper and Rosai in 1983.3 The peak incidence is during the third * Corresponding author. E-mail address: payal27@hotmail.com (M. Gupta). 0976-0016/$ e see front matter Copyright ª 2013, Indraprastha Medical Corporation Ltd. All rights reserved. http://dx.doi.org/10.1016/j.apme.2013.02.011
  3. 3. a p o l l o m e d i c i n e 1 0 ( 2 0 1 3 ) 1 6 2 e1 6 4 Fig. 3 e Lesion after closure. Fig. 1 e Labial incision. Fig. 2 e Excision of tumor. 163
  4. 4. 164 a p o l l o m e d i c i n e 1 0 ( 2 0 1 3 ) 1 6 2 e1 6 4 Fig. 4 e Gross examination: non capsulated 20 3 6 3 3 cm C/S: grayewhite, homogeneous and had a gelatinous appearance. Fig. 7 e Stromal cells with oval bland nuclei and delicate bipolar and multipolar cytoplasmic processes. Fig. 5 e Uniform and low cellularity and prominent vasculature. decade of life, suggesting that estrogen may stimulate its growth.1 It generally involves the genital, perineal and pelvic region, with vulval region being the most common site of involvement. Grossly these tumors are soft, partly circumscribed, polypoidal lesions with gelatinous appearance on cut section. Microscopically these lesions are composed of many thickwalled vessels of varying sizes in a loose myxoid and collagenous stroma with spindle and stellate-shaped neoplastic cells. Immunohistochemically these tumors express ER and PR, suggesting they may be hormone dependent.4 The tumor cells also express vimentin, desmin and SMA and are negative for S-100.2 These tumors have to be differentiated from angiomyofibroblastoma. In this case, the excision was complete and the postoperative period was uneventful. A two-year follow-up with MRI showed no recurrence. Conflicts of interest All authors have none to declare. references Fig. 6 e Small tumor cells without nuclear atypia, scattered in fibromyxoid background. 1. van Roggen JF, van Unnik JA, Briare-de-Bruijn IH, Hongendoorn PC. Aggressive angiomyxoma: a clinicopathological and immunohistochemical study of 11 cases with long-term follow-up. Virchows Arch. 2005;446:157e163. 2. Amezcua CA, Begley SJ, Mata N, Felix JC, Ballard CA. Aggressive angiomyxoma: a clinicopathological and immunohistochemical study of 12 cases. Int J Gynecol Cancer. 2005;15:140e145. 3. Magtibay PM, Salmon Z, Keeney GL, Podratz KC. Aggressive angiomyxoma of the female pelvis and perineum: a case series. Int J Gynecol Cancer. 2006;16:396e401. 4. Htwe M, Deppish LM, Saint-Julien JS. Hormone-dependant, aggressive angiomyxoma of the vulva. Obstet Gynecol. 1995;86:697e699.
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