Východiska: Angiomyofibroblastóm (AMFB) je zriedkavým patologickým nálezom na ženskom genitálnom trakte. Tento nádor patrí do skupiny mezenchymálnych nádorov, ktoré tvoria skupinu nádorov z mäkkých tkanív vonkajších rodidiel a pošvy. Do tejto skupiny sú zaradené fibroepiteliálne stromálne polypy, angiomyofibroblastóm, celulárny angiomyofibróm, agresívny angiomyxóm, myofibroblastóm, vulvárna leiomyomatóza a iné tumory vychádzajúce z hladkej svaloviny. Angiomyofibroblastóm je nezhubný nádor veľmi podobný agresívnemu angiomyxómu (AMM), ktorý sa považuje za malígny mezenchymálny nádor pre jeho lokálny infiltratívny rast. Najčastejší výskyt je na vonkajšom ženskom genitáli a na perineu. Napriek jeho malígnemu rastu nemetastazuje.
Prípad: 44 ročná žena s nálezom angiomyofibroblastómu krčka maternice.
Záver: Rozpoznanie tohto ochorenia je dôležité, aby sme sa vyvarovali nesprávnej diagnóze iných angiomyxoidných ochorení. Je dôležité vedieť, že vykazuje benígne chovanie, na rozdiel od iných agresívnych mezenchymálnych tumorov genitálneho traktu.
1; C. Bíró
2; M. Klačko
1; P. Mikloš
1; D. Ondruš
Authors place of work:
Department of Gynecologic Oncology, St. Elizabeth Cancer Institute, Bratislava, Slovak Republic
1; Histopatologia, Bratislava, Slovak Republic
2; 1st Department of Oncology, Faculty of Medicine, Comenius University, Bratislava, Slovak Republic
Published in the journal:
Klin Onkol 2011; 24(2): 133-136
Backgrounds: Angiomyofibroblastoma (AMFB) is a rare histopathologic finding of the female lower genital tract. This tumor belongs to the group of mesenchymal tumors. Mesenchymal neoplasms of the modified genital skin and mucosa are uncommon. The majority of these lesions are seen in females and, collectively, they form a family of vulvovaginal soft tissue tumors. This family includes fibroepithelial stromal polyps, angiomyofibroblastoma, cellular angiofibroma, aggressive angiomyxoma, vaginocervical myofibroblastoma, vulvar leiomyomatosis, and other smooth muscle tumors. Angiomyofibroblastoma is a benign tumor, histologically very similar to pelvic aggressive angiomyxoma (AMM), a distinctive, locally infiltrative but non-metastasizing mesenchymal neoplasm with a tendency to occur in the female pelvic and perineal regions.
Case: 44 years old woman with angiomyofibroblastoma of cervix uteri.
Conclusion: A recognition of this entity is important to avoid misdiagnosis of other angiomyxoid neoplasms. Furthermore, unlike other, more aggressive, mesenchymal tumors of the lower genital tract, AMFB shows benign behaviour.
Clinically, AMFB typically involves
the vulvar soft tissue of young to middle aged females, that ranges from 25 to
54 years (mean 36.3 years) [1 ]. The tumor typically presents as a vulvar
mass that usually has its epicenter in the labia majora. In the case of vaginal
or cervical finding of AMF, we can find a tumor mass in vagina or polypoid
tumor of cervix uteri. Uncommon sites of these tumors include the female
urethra  and fallopian tube .
tumors develop as slowly growing, marginated masses. Because of their
preferential location on the vulva they may be confused with a Bartholin’s
The process probably aries as a neoplastic
proliferation of hormonally responsive mesenchymal cells native to the unique
subepithelial connective stromal layer normally found through the endocervix,
vagina and vulva of adult women . These tumors develop as slowly growing,
marginated masses. Macroscopically, AMFBs range from 0.5 cm to 14 cm in greatest
dimension with the majority of them between 2–8 cm. The lesions are well-circumscribed,
round, ovoid, or lobulated masses with a soft to rubbery consistency. The
cut surface varies from gray-pink to yellowish brown to tan and is of
homogeneous texture with focal myxoid areas. Microscopically, the margin is
well delineated and non--infiltrative. A complete or partial fibrous
pseudocapsule of varying thickness may be present. Some tumors are bordered in
part by mature adipose tissue or smooth muscle. The tumor is characterized by
rich vascularization in a background of collagenous to edematous stroma
with alternating hyper- and hypocellular regions [5,6]. The stromal background
is edematous rather than myxoid. The nature of the background is supported by
negative staining for Alcian blue stain. The stromal cells possess
a bland, oval or elongated nuclei and either scanty, amphophilic cytoplasm
with ill-defined margins or eosinophilic, tapered cytoplasm with better
delineated cell borders. Intranuclear inclusions and longitudinal nuclear
grooves are common in the spindle cells. Epithelioid mesenchymal cells with
globoid eosinophilic cytoplasm and a single nucleus or occasional
multiple, round nuclei may be present. Mitotic figures are characteristically
rare or absent. The cellularity is quite variable and is somewhat related to
the vascularity. In most cases, the spindled and epitheloid cells proliferate
in a haphazard arrangement. In the more cellular cases, spindled cells form
loosely organizing fascicles. Tumor cells may aggregate or form masses around
blood vessels and those that are close to blood vessels may have
a myoepithelial appearance. The vascular component of the tumor consists
of small to medium-sized, rounded, curvilinear, non-branching, and thin-walled
vessels. Perivascular fibrosis or sclerosis is a feature detected to some
degree in all cases . Strong and diffuse immunoreactivity for both desmin
and vimentin is demonstrated in practically all cases. Only a minority of
cells in some cases show positive immunoreactivity for either smooth muscle
actin or pan-muscle actin [1,6,7,8,9,10]. Tumor cells are negative for S-100
protein, cytokeratin, collagen type IV, CD 68 and myoglobin [10,11,12]. The few
cases examined ultrastructurally have shown fibroblastic features in most
cells, with a minority showing myofibroblastic differentiation [1,6,8].
AMFB is a rare mesenchymal tumor arise in the
superficial lamina propria of the cervix and vagina and is histologically
distinguishable from mesodermal (fibroepithelial) stromal polyp, including the
cellular (pseudosarcomatous) variant, superficial cervicovaginal
myofibroblastoma (SCVM), aggressive angiomyxoma, and other well-recognised
lesions that occur in this location [1,4,5,11]. The most important differential
diagnosis is aggressive angiomyxoma, first described by Steeper and Rosai 
in 1983. Although rare examples have been subsequently reported in males
[1,7,14], the vast majority of these tumors occur in women of reproductive age.
Interestingly, rare tumors with a composite morphology of both AMFB and
aggressive angiomyxoma have been described [14,11]. In addition to aggressive
angiomyxoma, there are a few other entities should also be distinguished
from AMFB. An excellent review of the subject is available . Some of the
major differential diagnoses are discussed here. Cellular angiofibroma shares
similarities with AMFB in terms of age, sex, and location. This lesion
typically presents as a small, well circumscribed mass. In contrast to
AMFB, focal extension into surrounding tissue can be seen. The cellular
component is composed of spindle cells arranged in short intersecting fascicles
that are admixed with thick walled hyalinized blood vessels and collagen
bundles. Although there is brisk mitosis, pleomorphism and necrosis are absent.
These tumors are reported to be benign, with no local recurrences or metastasis
being described. Superficial angiomyxoma occurs most commonly in the fourth decade
of life. Over half of the cases occur in the trunk and lower extremities. The
rest occurs in the upper extremities, head and neck region and most of the
lesions are under 5 cm . In the genital region, about three quarters of
the cases occur in females . Grossly, superficial angiomyxoma can be
polypoid. Histologically, it is a myxoid neoplasm with moderately to
sparsely cellular myxoid nodules with delicate, thin walled capillary sized
blood vessels. The stromal cells are spindle to stellate in shape and bland.
Mitoses are uncommon. Scattered inflammatory cells, particularly neutrophils,
are always present. About a third of cases may have an epithelial
component such as a keratin filled cyst and epithelial strands. Although
benign, about a third of the tumors may be locally destructive. There was
encountered angiomyofibroblastomas with sarcomatous areas. These tumors may
either resemble an angiomyofibroblastoma with „malignant features“ or they may
display sarcomatous areas resembling lyiomyosarcoma or undifferentiated
sarcoma. None of these rare malignant tumors has metastasized .
We report a case of 44-year woman with polypoid
tumor arising from the cervix uteri with histological finding of AMFB.
Gynecological history of patient: menarche in age of 12 years with regular
periods 28–30 days, no history of bleeding between periods. 2 spontaneous
deliveries, 1 miscarriage, no history of oral contraceptives. In the age of 41
years removal of the right ovary for endometriotic cyst, without any other
hormonal medication. There were no history of abnormal PAP smear. Grand mother
died for diagnosis of endometrial carcinoma. Clinical finding were polypoid
formation arising from the cervix with smooth surface, pink color, rubbery
consistency. No other abnormal finding on genital tract.
Polypous formation 2 cm in greatest dimension, well-circumscribed, rubbery
consistency, subepithelial in location, with edematous and myxoid stroma,
numerous thin- walled vessels, focal bleedings, small oval to fusiform only
„stellate“ cells with minimal cytoplasm, basophilic nuclei without markedly
atypias, without proliferation.
Recognation of this entity is important to avoid
misdiagnosis with other angiomyxoid neoplasms. It is important to recognize
this entity as it shows benign behaviour with respect to other mesenchymal
tumors of the lower genital tract, which have a more aggressive behaviour.
The authors declare they have no potential
conflicts of interest concerning drugs, pruducts, or services used in the study.
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met the ICMJE “uniform requirements” for biomedical papers.
Redakční rada potvrzuje, že rukopis práce splnil ICMJE kritéria pro
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Babala Peter, MD.
Department of Gynecologic Oncology
St. Elizabeth Cancer Institute
812 50 Bratislava
Submitted/Obdrženo: 23. 9. 2010
Accepted/Přijato: 5. 1. 2011
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