Author information
Asmita Patil*, Parulekar SV**, Samant PY***, Gwendolyn
Fernandes****
(*Senior Resident, **Professor and head of the department,
*** Additional Professor Department of
Obstetrics and Gynecology, **** Associate Professor, Department of Pathology,
Seth GS Medical College and KEM Hospital, Mumbai, India.)
Abstract
Angiomyofibroblastoma is an
uncommon, benign, nonrecurring, mesenchymal,
myofibroblastic tumor that occurs most commonly in the vulva and vagina. It
usually occurs in premenopausal women. We report a case of
angiomyofibroblastoma of the vulva
occurring in a 38 year old woman in the paraurethral region.
Introduction
Angiomyofibroblastoma is a rare benign mesenchymal tumor that is
commonly observed in the vulvovaginal
region of women.[1-3] It is a
slowly growing tumor which is often misdiagnosed as a Bartholin’s cyst, a
lipoma, cellular angiofibroma, fibroepithelial stromal polyp, superficial
angiomyxoma or a perineal hernia.[4] Histologically, the tumor is a
well-circumscribed lesion composed of alternating hypo- and hypercellular areas
with numerous delicate capillary-sized vessels, the presence of plump stromal
cells, and perivascular condensation of the stromal cells.[5-9] Majority of the reported cases shown that the
mass arises from labia majora but our in our case the mass was arising from paraurethral region.
Case Report
A 38 year old multiparous woman
came to our outpatient department with a history of a mass at the vulvar region.The
mass had gradually grown in size over eight years. There was no dyspareunia.
There had been no history of pain in the swelling at any time, fever, periodic
changes in the size of the swelling or its rupture at any time. She had dysuria
for 2 months. On local examination 3X 3 cm soft swelling was present just
lateral and a little superior to the external urethral meatus on left side. It
appeared cystic. It was freely mobile under the epithelium, and moved
independent of the urethra. General, systemic, per speculum and bimanual pelvic
examination showed no abnormality. Urinalysis showed 10-15 pus cells per high
power field. Microbiologic study of urine showed E. coli sensitive to
nitrofurantoin. She was treated for that and got well. Her hemogram, blood sugars
(fasting and postprandial), liver function tests, renal function tests, chest
radiograph and elelctrocardiogram results were within normal limits. A
urological reference was made. The urologist confirmed that the mass was not
connected with the urinary bladder or urethra.
A No. 14 Foley's catheter was
passed into the urinary bladder through the urethra. The mass was excised under
general anesthesia, through a longitudinal incision placed over the most
prominent part of the mass, medial to the left labium minus, lateral and a
little superior to the external urethral meatus. It appeared encapsulated and
could be dissected easily without any significant blood loss. Redundant part of
the overlying epithelium was excised and the edges were sutured with interrupted
sutures of No. 2-0 polyglactin. The catheter was removed after 24 hours. The
patient made an uneventful recovery. She had no difficulty in micturition
postoperatively.
Microscopically the section of the
mass showed a circumscribed mass with a vascular structure partly enveloping
it. The lesion was composed of prominent vascular channels surrounded by plump
spindle shaped cells and thick bundles of collagen. Individual plump spindle
shaped cells showed prominent oval to spindle shaped nuclei and abundant
eosinophilic cytoplasm. A diagnosis of vulvar angiomyofibroblastoma was made.
Figure 1. Swelling of the vulva,
on the left side of the external urinary meatus.
Figure 2. The tumor in its bed,
after incision in the epithelium above it and dissection of the epithelial
flaps.
Figure 3. Thinly encapsulated tumor with hypercellular and hypocellular
areas. Slit-like spaces seen represent capillary size vessels. (H & E x
100).
Figure 4. Tumor composed of plump spindled to
oval cells and capillaries amidst thin wavy strands or thick bundles of
collagen. (H & E x 400).
Figure 5. Spindle cells show strong positivity for desmin.
Figure 6. Strong positivity for smooth muscle actin.
Discussion
Angiomyofibroblastoma is a rare
benign mesenchymal tumor that is commonly observed in the vulvovaginal region of women.[1-3]
It was first described by Fletcher et al in 1992. [10] It is a
slowly growing mass which is often misdiagnosed as a Bartholin’s cyst, a
lipoma, cellular angiofibroma, fibroepithelial stromal polyp, superficial
angiomyxoma or a perineal hernia.[4] Grossly it is a well
circumscribed but usually nonencapsulated, soft lesion measuring 1-10 cm (mean
4 cm) in diameter. Its cut section of the lesion is gray–pink with a glistening
appearance. Microscopy shows a pseudocapsule, small-medium sized, well-formed vessels, round to spindled
myofibroblasts placed around the vessels, alternating hypercellular
stromal area and
hypocellular edematous area, spindle- or epitheloid-shaped stromal cells
gathered around abundant capillary-type blood vessels and loose edematous stroma in varying proportions. The
tumor cells have bland nuclei and eosinophilic cytoplasm epithelioid to
plasmacytoid neoplastic cells, binucleate or multinucleate cells, interstitial
mast cells, and mature adipocytes. Mitotic figures are rare, tumor necrosis
absent. Longstanding lesions show fibrosis and perivascular hyalinization.[4-9]
It may be mistaken for an aggressive angiomyxoma, which shows abundant myxoid matrix, infiltration of surrounding tissue,
unclear border, low cellularity, thickened
medium-to-large sized vessels, and extravasation of red blood cells.[6,7,8,9]
A cellular
angiofibroma (CA) may be mistaken for an angiomyofibroblastoma too. Both of
them have prominent
vascular components, bland stromal cells, and adipocytes. Angiomyofibroblastoma is distinguished from CA by the presence of
round tumor cells grouped around vessels, and a lack of wispy collagen bundles
and numerous thick-walled, hyalinized vessels.
It is a benign nonrecurring lesions and simple excision is
curative. A recurrence is rare. Still a long term follow up is recommended.
References
1.
Hsu IH, Chang TC, Wu CT, Chen RJ,
Chow SN. Angiomyofibroblastoma of the vulva. Journal of the Formosan Medical
Association, 2004;103:467–471.
2.
Laiyemo R, Disu S, Vijaya G, Wise
B. Post-menopausal vaginal angiomyofibroblastoma: a case report. Archives of
Gynecology and Obstetrics 2005;273:. 129–130.
3.
Varras M, Akrivis C, Demou A,
Kitsiou E, Antoniou N. Angiomyofibroblastoma of the vagina in a postmenopausal
breast cancer patient treated with tamoxifen: clinicopathologic analysis of a
case and review of the literature. International Journal of Gynecological
Cancer. 2006;16:581–585.
4.
Magro G., Caltabiano R.,
Kacerovská D., Vecchio GM, Kazakov D, Michal, M. Vulvovaginal myofibroblastoma:
Expanding the morphological and immunohistochemical spectrum. A clinicopathologic
study of 10 cases. Human Pathology 2012;43, 243-253.
5.
Nielsen GP, Young RH. Mesenchymal
tumors and tumor-like lesions of the female genital tract: a selective review
with emphasis on recently described entities. International Journal of Gynecological
Pathology. 2001;20:105-127.
6.
Micheletti AM, Silva
AC, Nascimento AG, Da Silva CS, Murta EF, Adad SJ. Cellular angiofibroma of the
vulva: case report with clinicopathological and immunohistochemistry study. Sao
Paulo Med J. 2005;123:250-252.
7.
Hlaing T, Tse G. Angiomyofibroblastoma of the male
perineum: an unusual location for a rare lesion. Int J Surg Path 2000;8:79-82.
8.
Wang J, Sheng W, TU X, Shi D, Zhu X, Zhang R.
Clinicopathologic analysis of angiomyofibroblastoma of the female genital
tract. Chin Med J (Engl) 2000;113: 1036-1039.
9.
Laiyemo R, Disu S, Vijaya G, Wise B. Postmenopausal
vaginal angiomyofibroblastoma: a case report. Arch Gynecol Obstet. 2005;273:129-130.
10.
Fletcher CD, Tsang WY, Fisher C,
Lee KC, Chan JK. Angiomyofibroblastoma of the vulva. A benign neoplasm distinct
from aggressive angiomyxoma. Am J Surg Pathol 1992;16:373-382.
Citation
Patil A, Parulekar SV, Samant PY, Fernandes G. Angiomyofibroblastoma of the Vulva. JPGO 2014
Volume 1 Number 7 Available from: http://www.jpgo.org/2014/07/angiomyofibroblastoma-of-vulva.html