Surgical Science, 2010, 1, 40-45
doi:10.4236/ss.2010.12008 Published Online October 2010 (http://www.SciRP.org/journal/ss)
Aggressive Angiomyxoma: An Unusual Female Pelvic
Tumour. Report of Three Cases and Review of the
Literature
Juan Antonio Martín-Cartes, Manuel Bustos-Jiménez, María Jesús Tamayo-López, María del
Carmen Palacios-González, Virginia Gómez-Cabeza de Vaca, Antonio Muñoz Ortega
Division of Surgery, Abdominal Wall Surgery Unit, Seville, Spain
Received July 7, 2010; revised August 1, 2010; accepted August 10, 2010
Abstract
Aggressive angiomyxoma was first described in 1983 by Steeper and Rosai, and fewer than 150 cases have
been reported in the world medical literature. It is a soft-tissue tumour of the pelvis and perineum. The re-
currence rate is high, and often extensive resections are performed with considerable morbidity. These tu-
mours are benign, locally infiltrative mesenchymal neoplasms with a predilection for the female pelvis and
perineum and they usually tend to recur. Furthermore, these tumours often reach too large dimensions before
becoming clinically symptomatic; their incidence is higher in women of the reproductive age group; however
a few cases of its occurrence outside the pelvis have also been reported. In this study, we reported three cases
with aggressive pelvic angiomyxoma treated with surgical methods and used an approach that described by
Kraske in order to get access to lower rectal cancers. Accurate preoperative diagnosis should alert the sur-
geon to the need for wide excision, which is essential for prevention of local recurrence.
Keywords: Aggressive Angiomyxoma, Soft Tissue Tumour, Perineum and Pelvis Tumour
1. Introduction
Aggressive angiomyxoma is a rare tumour of mesen-
chymal origin first described in 1983 by Steeper and
Rosai [1-2] like a distinct mesenchymal tumour of the
female pelvis and perineum. However, it has later been
reported in males. In men, the tumour involves analo-
gous sites including the scrotum and inguinal area and
usually appears at an older age [3]. It’s suspected there
might be a relation with hormonal status that might ex-
plain a female to male ratio of slightly more than 6:1.
Have been reported about 150 cases in the world medical
literature [4-8].
These lesions are characterized as soft, non-encapsulat-
ed tumours with finger-like projections infiltrating the
surrounding soft tissues. The tumour presents as a large
multilobular or polypoid mass or swelling. On gross exa-
mination it is rubbery and white or soft and gelatinous.
The tumour grows slowly, and its benign nature is sug-
gested by the histology and by the fact that it shows no
tendency to metastasize [9]. Unlike malignant neoplasms,
do not metastasize, and, histologically, they demonstrate
bland nuclei without atypia or mitotic activity. However
it usually tends locally to recur [10]. Correlation between
tumour size and subsequent risk of recurrence has not
been demonstrated [11].
Surgery is usually the first line of treatment, radical
surgery with wide margins and long-term follow-up is
advised. There has been some interest in the potential use
of hormonal manipulation to manage those tumours after
surgery. Intuitively, hormonal suppression seems to be a
plausible treatment option because these tumours occur
predominantly in premenopausal women of reproductive
age, may grow rapidly during pregnancy, and have been
shown to express immunohistochemical positivity for
estrogenic and progesteronic receptors [12].
2. Material and Methods
From January’09 to April’10, three women with aggres-
sive angiomyxoma were managed in our Unit. Two of
these cases were directly managed in our centre while the
other had previously been operated in Rumania.
We also performed a Medline search from 2006 to

J. A. MARTÍN-CARTES ET AL. 41
2010 with the key words “aggressive angiomyxoma” and
reviewed literature we had found.
This Medline search reported up to 150 cases, 112
cases of this rare neoplasm were reported in women if
limited to the perineum and pelvis.
The most relevant details for the three patients man-
aged in our Unit are detailed below.
Patient 1. A 50-year-old Rumanian female. In 2006,
in Rumania, she was operated on her pelvic area using a
trans-abdominal approach and a great pelvic mass was
resected. The mass was diagnosed as an aggressive an-
gyomixoma postoperatively.
In 2009, after she arrived in Spain, she presented again
symptoms such as presented with a long history of a
vague, poorly defined, painless swelling over the right
ischial tuberosity. On examination, in cases 1 and 3, the
swelling was ill defined, non-tender, and difficult to pal-
pate. MRI scans showed large and abnormal soft tissue
masses (9.6 × 4.5 × 4.5 cm) within the pelvis. Those
masses were iso-intense, but sometimes became very
hyperintense. At present, after 12 months no recurrence.
Patient 2. A 36-year-old female. She presented a
3-year history of swelling left sided in vulva and per-
ineum, big and evident mass on examination and palpa-
tion. She had changed her bowel habit. MRI scans
showed a big mass (24 × 11 × 12.3 cm) in minor pelvis
extending into right ischiorectal fossa, left paravaginal
area and perineum (Figures 1 and 2). After 9 months no
recurrence observed.
Patient 3. A 28-year-old female. She presented a
2-year history of swelling left sided in vulva and per-
ineum, big mass on examination and palpation. She had
changed her bowel habit, suffered from urinary urgency
and had been treated with hormonal stimulators in order
to donate her oocytes. MRI scans showed a big mass (20
× 10 × 9 cm) affecting her minor pelvis and extending
into right ischiorectal fossa and left paravaginal area.
After 9 months no recurrence observed.
In all cases the diagnosis of aggressive angiomyxoma
was made only after initial surgery.
Preoperative misdiagnosis of aggressive angiomyxo-
ma is not infrequent because this rare neoplasm has no
universally typical symptoms.
All cases presented with a long history of a vague,
poorly defined, painless swelling over the right ischial
tuberosity. On examination, in cases 1 and 3, the swell-
ing was ill defined, non-tender, and difficult to palpate,
in case 2 the mass was evident enough. Clinically the
initial differentials were of lipoma, liposarcoma or pelvic
floor hernia.
MRI scans showed large and abnormal soft tissue
masses within the pelvis. Those masses were iso-intense,
but sometimes became very hyperintense, with much
whorled solid tissue within. There was no fluid or fat
within them. The masses were extended from the pubic
symphysis until to the rectum and extended as far as S1
level superiorly, retroperitoneally and inferiorly as far as
the level of the anal sphincter. The MRI appearances
were suggestive of an unusual mesenchymal tumour.
Abdominal and pelvic CT scan, and transvaginal ul-
trasound and intravenous urography (IVU) both con-
firmed the well-circumscribed appearance of the mass
and deviation of rectum, uterus, ureters and bladder.
Surgery revealed a large mass located in the lower
abdomen, the left pelvis extending to the left ischio-rec-
tal region. The tumour had closed contact with muscles.
Complete excision was performed by a Kraske approach.
Coxigeal and partial sacral excision was only made in
case 3.
Patients 1 and 2 stayed at hospital for a week.
Cut section in all cases revealed a gelatinous soft and
greyish coloured tumour with a fine capsule and no lobes,
necrosis or cystic degeneration. Excised tumours had a
gelatinous composition and weighed 500, 1030 and 495 gr
(Figures 1-3).
Microscopic examination showed a relatively hypo-
cellular tumour with myxoid stroma containing numer-
ous vessels, some with thick walls. The cells showed
regular nuclei and stellate fine cytoplasm. There was no
cytologic atypia, no atypical mitotic features or discerni-
ble mitotic activity and no evidence of coagulative tumor
cell necrosis either.
Immunohistochemical studies showed the cells to be
positive for vimentine and dismin. There was a strong
positivity for estrogenic and progesteronic receptors. In
our cases show the cells to be positive for vimentine and
focally positive for dismin and smooth muscle actin.
That is why all cases the masses were diagnosed as
aggressive angiomyxoma. The recurrent tumours showed
similar histological characteristics to the primary ones.
MRI scans showed large and abnormal soft tissue
masses within the pelvis. Those masses were iso-intense,
but sometimes became very hyperintense, with much
whorled solid tissue within. There was no fluid or fat
within them. The masses were extended from the pubic
symphysis until to the rectum and extended as far as S1
level superiorly, retroperitoneally and inferiorly as far as
the level of the anal sphincter. The MRI appearances
were suggestive of an unusual mesenchymal tumour.
Abdominal and pelvic CT scan, and transvaginal ul-
trasound and intravenous urography (IVU) both con-
firmed the well-circumscribed appearance of the mass
and deviation of rectum, uterus, ureters and bladder.
3. Results
The patients 1 and 2 made an uneventful recovery, and
went home 7 days later. The other suffered from a partial

J. A. MARTÍN-CARTES ET AL. 42
skin necrosis and she stayed at hospital for a second
postoperative week. At present, 5-12 months’ follow-up,
there was no evidence of recurrence either clinically or on
imaging studies.
4. Discussion
Aggressive angiomyxoma is a rare benign tumour that
arises from the connective tissue of the perineum or
Figure 1. Case 1. Pelvic mass on physical examination and its images.

J. A. MARTÍN-CARTES ET AL. 43
Figure 2. Case 2. The excised mass and postoperative tomography.
Figure 3. Case 3. Preoperative CT, the excised specimen and the final appearance.

J. A. MARTÍN-CARTES ET AL. 44
lower pelvis. The rarity of this condition makes the pre-
operative diagnosis fairly difficult. It has been related to
hormonal activity; our cases 1 and 2 were pre- meno-
pausal females, and the other had undergone medical
hormonal stimulation treatment in order to donate her
ovules for five years.
Complete surgical excision with tumour-free margins
is the main objective. This surgery is challenging be-
cause of the infiltration and the difficult dissection. In the
past, most authors advocated wide excision even if geni-
to-urinary and digestive tract resections were necessary.
On the one hand, local excision of villous adenomas
and other benign rectal lesions has been accepted for
decades, and the Kraske approach for management of
these lesions has been recommended by several authors.
Surgical resection of rectal disease via a posterior ap-
proach was first described by Kraske at the Fourteenth
Congress of the German Association of Surgeons in
1885. In his initial description, Kraske advocated re-
movingtumors of the middle rectum by excising the
coccyx and a portion of the sacrum. The original proce-
dure described division of the left gluteal muscle inser-
tions, the left sacrotuberous and sacrospinous ligaments,
and the left fourth and fifth sacral nerves.
Furthermore, that approach may be useful to excise
pelvic masses like the above described in order to reach
both upper and lower ends.
On the other hand, the posterior approach to the rectum
utilizing the Kraske procedure provides a better exposure
for distal rectum (5-10 cm from the anal verge) so that we
can locate it on surgical procedure [13-16].
Although Mera-Velasco [17] has reported the utility of
laparoscopic approach, we do not agree with him. We do
not consider doing that approach so as not to have to
fragment the excised specimen. Moreover, it is necessary
to have in mind that releasing the lower end of those
masses is usually the most difficult step in the surgical
procedure.
Because of abdominal and pelvic organ manipulation,
hospital stays are generally longer, and patients are at
higher risk of developing problems such as deep venous
thrombosis and pulmonary embolism while they await the
return of bowel function after procedure.
To sum up, aggressive angiomyxoma should be dis-
tinguished from other, more common neoplasms of the
pelvic tissues. When the mass is not well defined or has
atypical behaviour, improving preparation before surgery,
and direct the surgeon toward complete excision might
be the best option.
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J. A. MARTÍN-CARTES ET AL. 45
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