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.

1. Angiomyxoma - A Rare Case Report

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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

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Fig. 3 e Lesion after closure.
Fig. 1 e Labial incision.
Fig. 2 e Excision of tumor.
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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.
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cases with long-term follow-up. Virchows Arch.
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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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