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: paban.Sharma@gmail.com

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 vulvo-
vaginal 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.

References:

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Multifocal aggressive angiomyxoma : a case report, J Clin Pathol
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angiomyxoma: case report and review of the literature. Eur J Gynaecol Oncol.
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Vulva, Gynecol Oncol 2001 May, 81(2): 326-9

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7. Rawlinson NJ, West WW, Nelson M, Bridge JA; Aggressive angiomyxoma

 with t(12;21) and HMGA2 rearrangement: report of a case and review of the
literature. Cancer Genet Cytogenet. 2008 Mar;181(2):119-24.

8. Chen L, Schink JC, Panares BN, Barbuto D, Lagasse LD. Resection of a giant

aggressive angiomyxoma in the Philippines. Gynecol Oncol. 1998 Sep;70(3):435-9.