Vulvovaginal Soft Tissue Tumors: An Update
Brigham & Women's Hospital
Initial recognition of the various soft tissue lesions that characteristically occur in
the distal female genital tract began with the description of distinctive, benign stromal polyps in the
early 1960's.  Since the initial description of fibroepithelial stromal polyp, it is now known
that a variety of relatively site-specific stromal tumors can occur in this area, most notably aggressive
angiomyxoma, angiomyofibroblastoma and cellular angiofibroma. Because of their apparently shared origin
from vulvovaginal mesenchyme, these lesions can superficially resemble one another, as well as other soft
tissue neoplasms (such as superficial angiomyxoma or smooth muscle neoplasms), and can be diagnostically
challenging. Differences in clinical behavior between these various soft tissue tumors necessitate
accurate diagnosis to ensure proper treatment; however, because these lesions may exhibit similar
immunohistochemical and ultrastructural profiles, recognition must often depend upon the morphologic
interpretation of routine H&E slides.
While a wide variety of benign (e.g. neurofibroma, nodular fasciitis) and malignant (e.g.
epithelioid sarcoma, liposarcoma) lesions not specific to this anatomic site may also occur in the vulva,
these are not further discussed in this brief overview.
Fibroepithelial stromal polyps
Fibroepithelial stromal polyps (FSP) of the vulvovaginal region exhibit a wide range of
morphologic appearances. This morphologic spectrum has, in part, led to their underrecognition in some
cases and, in those examples which exhibit worrisome histologic features, their misinterpretation as
malignant. FSP usually occur in young to middle aged women in their reproductive years, presenting more
commonly in the vagina, but also occurring in the vulva and rarely the cervix.
occur as a single lesion, or can be multiple, an occurrence particularly associated with pregnancy.
FSP that occur during pregnancy tend to exhibit a greater degree of cellularity and
nuclear pleomorphism, which contributed to use of the term "pseudosarcoma botryoides" by some
These lesions are benign but have the potential for local non-destructive
recurrence, particularly if incompletely excised.
Histologically, FSP are characteristically polypoid, occasionally fronded and usually
contain a conspicuous fibrovascular core; however, the most distinctive aspect is the stroma, which can
exhibit a wide range of morphologic features. On one end of the spectrum, FSP can be bland and
hypocellular, being composed of spindle shaped cells with indistinct cytoplasm set within a loose
collagenous matrix. At the other end of the spectrum, FSP can be hypercellular, exhibit marked nuclear
pleomorphism and frequent mitoses (>10 mitoses/ 10 hpf) including atypical forms ). These
floridly pseudosarcomatous examples of FSP produce an especially worrisome histologic appearance and can
easily be mistaken for a malignant neoplasm. Clues to the identity of both bland and more florid
examples of FSP are the presence of characteristic stellate and multinucleate stromal cells, which tend
to occur near the epithelial-stromal interface and the lack of any clear margin. These lesions extend
right up to the mucosal-submucosal interface and essentially represent a form of localized stromal
hyperplasia. Cellular FSP also tend to exhibit a greater degree of cellularity in the center of the
lesion, becoming less cellular as the lesion extends up to the stromal-epithelial interface. The
vascular component of FSP is variable, but usually composed of medium sized thick-walled vessels. The
stromal cells of FSP exhibit a variable immunophenotype but, in general, they are desmin and vimentin
positive, with actin positivity being less common.
The stromal cells can also be
positive for estrogen and progesterone receptor.
The pathogenesis of FSP is not well understood; however, there is copious indirect evidence suggesting
that they represent reactive hyperplastic lesions that arise from the distinctive myxoid subepithelial
zone of the distal female genital tract. The stellate and multinucleate cells characteristic of FSP can
be seen in normal cervix, vulva and vagina and reactive states involving the female genital tract (such
as uterine prolapse). Similar cells can also be seen in reactive states outside the female genital tract
(such as nasal polyps and ulcers of the gastrointestinal tract), providing further evidence for the
reactive rather than neoplastic nature of these cells. Evidence suggesting that hormonal influences play
a role in their formation includes: (1) the propensity of these lesions to occur during pregnancy and
spontaneously regress following delivery, (2) the association with hormone replacement therapy in the
postmenopausal patient and (3) the stromal cells of FSP can be estrogen and progesterone receptor
positive. Furthermore, FSP are extremely uncommon before the menarche.
Angiomyofibroblastoma (AMF), first recognized in the early 1990s, occurs almost
exclusively in the vulvovaginal region of women but can also occur occasionally in the inguinoscrotal
region of men.
It is a well-circumscribed tumor that clinically is often thought to
represent a Bartholin's gland cyst. AMF usually measures less than 5 cm but can be larger, with tumors
up to 12 cm reported in the literature.  Overall, AMF is a benign non-recurring lesion and
local excision with clear margins is adequate treatment. There is one report of an angiomyofibroblastoma
with sarcomatous transformation that recurred.  In this case, areas apparently typical of
angiomyofibroblastoma merged imperceptibly with a high-grade sarcoma.
Histologically, AMF is characteristically a well-circumscribed lesion composed of
alternating hyper- and hypocellular areas. Plump round-to-spindle shaped cells characteristically
cluster, or are present in linear array, around numerous delicate capillary-sized vessels set within a
variably edematous to collagenous matrix. The stromal cells can be somewhat epithelioid or plasmacytoid,
and are often multinucleate, having moderate amounts of eosinophilic cytoplasm and nuclei with fine
chromatin and inconspicuous nucleoli. Occasionally intralesional adipose tissue may be
present.  Mitoses are not a prominent feature and are generally difficult to find. Stromal
mast cells can be abundant. The stromal cells of AMF are typically desmin positive and actin negative,
although they can occasionally exhibit actin positivity.
Cellular angiofibroma (CA) is a more recently described benign stromal tumor that occurs
mainly in the vulva or perineum of middle-aged women,
however, it also occurs in the
inguinoscrotal region of men
(where it was briefly known as angiomyofibroblastoma-like
tumor ). Examples in retroperitoneum have also been recognized.  When CA occurs
in the vulva, it is characterized by its generally small size (< 4 cm) and usually well-circumscribed
margin; examples in men tend often to be larger and less clearly marginated. CA behaves in a benign
fashion. To date, no case has either recurred locally or metastasized, although I have now seen three
cases with clear histologic evidence of sarcomatous transformation, most often as a microscopic focus.
Local excision with negative margins appears to be adequate treatment for these lesions.
Histologically, CA is usually well circumscribed, although focal extension into
surrounding soft tissue can be seen. It is a cellular neoplasm, being composed of bland spindle shaped
cells arranged in short intersecting fascicles, wispy collagen bundles and numerous thick-walled, often
hyalinized, vessels. A usually minor component of adipose tissue is present in about 25% of cases. The
spindle cells have bland oval to fusiform nuclei and scant, pale eosinophilic cytoplasm with indistinct
borders, being rather similar in appearance to the spindle cells of spindle cell lipoma. Mitotic
activity can be brisk but no tumor cell necrosis or pleomorphism is seen. Mild degenerative nuclear
atypia may be a feature, more often in males. Mast cells are often abundant. The tumor cells are CD34
positive in 60% of cases and SMA positive in 20%. Occasional cases are desmin positive, but CAF is
negative for S-100 protein, keratin and epithelial membrane antigen. ER and PR may be positive in 40-50%
Aggressive angiomyxoma (AA) is a non-metastasizing, locally infiltrative tumor of the
pelvi-perineal region that has the potential for local, occasionally destructive
Although originally thought to occur only in women, it is now well
recognized to arise also in analogous sites in men.
In women, AA tends to occur most
commonly in the reproductive years with a median incidence in the fourth decade. In men, the median age
at presentation is in the sixth to seventh decades. Clinically, AA is most often thought to be a labial
cyst, particularly a Bartholin's gland cyst. It characteristically has a myxoid or gelatinous appearance
but can appear more fibrous, especially when it recurs. Local, sometimes destructive recurrence occurs
in approximately 30-40% of cases, sometimes many years (even decades) after the initial
However, it is nowadays recognized that more than one recurrence is unusual
and that most recurrences are cured by just one additional surgical intervention, suggesting that these
lesions are not as "aggressive" as first thought. Except for positive surgical margins, there are no
clinical or histological means for predicting which tumors are likely to recur. Intuitively one might
think that size is associated with the potential for recurrence, with smaller lesions being less likely
to recur; however, tumors as small as 3 cm have recurred multiple times over many
years.  Treatment should be complete excision whenever possible.
Histologically, AA is characteristically poorly circumscribed with extension into
surrounding soft tissue. It is generally a paucicellular neoplasm, being composed of bland spindle
shaped cells set within a copious myxoid matrix containing variably sized, but most often medium to
large-sized, thick walled, often hyalinized, vessels. The spindle shaped cells have bland,
round-to-ovoid nuclei with dispersed chromatin and moderate amounts of pale eosinophilic cytoplasm
discernible as delicate bi- or multi-polar cell processes. Delicate fibrillary collagen is dispersed
throughout the myxoid stroma but characteristically condenses around the vascular component, forming
loose cuffs. Another characteristic feature is the presence of bundles of eosinophilic smooth muscle
cells (so-called myoid bundles) which are usually arranged in loose clusters or tight whorls adjacent to
the blood vessels and which appear to 'spin off' into the surrounding stroma. Although originally
thought to be desmin negative, it is now well known that the stromal cells of AA often exhibit desmin and
actin positivity. Positivity for ER and PR is also common.
Cytogenetic analysis, performed in only a few cases
has revealed clonal aberrations
involving chromosome 12. One case of AA was found to have a clonal chromosomal translocation involving
chromosome 12, specifically t(8;12)(p12;q15),  resulting in aberrant expression of HMGIC protein, a DNA architectural factor important in transcriptional regulation,
which is rearranged in a variety of other benign mesenchymal neoplasms. 
Prepubertal vulval fibroma
Added to the list of seemingly site-specific distinctive vulvovaginal stromal lesions is
the very recently described prepubertal vulval fibroma (PVF). 
As the name suggests, PVF  appears to occur exclusively in the vulva
(principally labia majora) of prepubertal females, most often before the age of 10. It presents as a
painless unilateral swelling, most often 3-5 cm in size, with margins which are hard to define
surgically. Even with relatively limited follow-up data thus far, at least 30% of these lesions appear
to recur locally, sometimes more than once. Such recurrences seem always to be the consequence of
incomplete local excision.
PVF is invariably poorly marginated and is characterized by bland spindle-shaped
fibroblastic cells with pale cytoplasm set in a variably collagenous to edematous stroma. These cells
ramify between pre-existing structures, entrapping fat cells, variably sized vessels and nerve bundles.
In come cases, the lesion extends right up to the mucosal-submucosal interface. Mitoses are scarce.
Immunostains show positivity only for CD34.
The unilaterality of these lesions, lack of any evident associated hormonal abnormality
and tendency for local recurrence would suggest that these unusual lesions are neoplastic rather than
reactive or hyperplastic in nature.
Superficial angiomyxoma, although not site-specific, is included here because of the
tendency for it to be confused with both aggressive angiomyxoma and with stromal polyps.
Superficial angiomyxoma (SA), sometimes also known as cutaneous myxoma, can occur in a
wide variety of cutaneous locations, particularly the head and neck region, the trunk and the genital
It occurs most commonly in the fourth decade, presenting as a slowly growing
painless mass that usually measures less than 5 cm. SA is benign; however, it has an approximate 30-40%
rate of local non-destructive recurrence and should be completely excised with a clear margin whenever
possible. The presence of multiple cutaneous myxomas and angiomyxomas arising at certain sites, such as
the breast or external ear, are highly associated with Carney's complex. This association is less clear
with SA arising in the genital area.  However, if multiple SA are present, then the
possibility of Carney's complex should be raised, particularly since patient's with Carney's complex may
have cardiac myxoma(s) and are at risk for sudden death.
SA is characteristically centered in the dermis and subcutaneous tissue and, because of
this feature, can often be clinically and histologically polypoid in appearance. SA is a myxoid neoplasm
with a distinctive multilobulated growth pattern. It is composed of bland, slender spindle and stellate
shaped cells set within a copious myxoid stroma that also contains delicate thin walled capillary sized
vessels. Scattered inflammatory cells, particularly stromal neutrophils, are very often present – the
latter being a helpful histologic clue, particularly in the absence of necrosis or ulceration. Nuclear
pleomorphism and mitoses are uncommon. In approximately 25% of cases, SA contains an epithelial
component, usually in the form of squamous epithelial-lined cysts, buds of basaloid cells or strands of
squamous epithelium. These foci are presumed to result from entrapment of adnexal structures or
secondary to stimulation of the overlying epithelium by the tumor. In our experience, the spindle cells
of SA are negative for actin, S-100 and desmin.
Smooth muscle tumors
Smooth muscle tumors occur infrequently in the distal female genital tract, being much more common in
the uterus. In soft tissue, smooth muscle tumors were originally classified based upon their location –
superficial or deep-seated. Genital smooth muscle tumors were originally placed in the category of
superficial smooth muscle tumors, which included pilar leiomyoma and angioleiomyoma, and were believed to
have similar criteria for malignancy. However, smooth muscle tumors of the distal female genital tract
differ both clinically and histologically from pilar (cutaneous) smooth muscle tumors and these should
not be co-classified.  Therefore, in soft tissue, three general categories of smooth muscle
tumors are currently recognized, each with their own criteria for malignancy – superficial (including
nipple), genital (vulva, scrotum) and deep-seated.
Smooth muscle tumors of the vulva occur over a wide age range, but they occur most commonly in the
fourth and fifth decades.
As with other mesenchymal lesions occurring at this site,
genital smooth muscle tumors are clinically often felt to represent a cyst. They typically present as a
subcutaneous lump noticed either by the patient or by their physician during routine gynecologic
examination. Of interest, these lesions can be present for many years. The tumors vary in size but when
they are benign or of uncertain malignant potential, they are usually well circumscribed and between 3
and 5 cm, while those that are malignant tend to be infiltrative and of larger size, often greater than 5
Smooth muscle tumors of the vulva exhibit three principal histologic patterns – spindled, epithelioid
and myxohyaline. These patterns can be mixed or pure. The spindle cell pattern is comparable to that of
leiomyomas in the uterine corpus, being composed of spindle shaped cells with moderate amounts of
tapering eosinophilic cytoplasm and ovoid to elongate nuclei with rounded or blunt ends forming
intersecting fascicles. In the myxohyaline pattern, which is curiously common in smooth muscle tumors of
the vulvovaginal region, the neoplastic spindle cells are separated by myxohyaline material with
entrapment of small fascicles imparting a lacy or plexiform appearance. Prominent myxoid change alone
with mucin pooling can also be seen.  In the epithelioid pattern, a combination of spindle
and epithelioid cells are often encountered, with the former having rounded cells with moderate amounts
of eosinophilic or clear cytoplasm present in clusters or sheets.
Histologic criteria which predict recurrent potential
Because of the relative infrequency of smooth muscle tumors that occur in the vulva and
the relatively limited series of cases with long-term follow-up, there is difficulty in reliably
predicting recurrent potential. While a combination of size, circumscription and mitotic thresholds have
been proposed to separate benign smooth muscle tumors from those with recurrent
in my experience, any mitotic activity, nuclear pleomorphism or evidence of an
infiltrative margin, regardless of the size of the tumor, is associated with locally recurrent
potential. I have seen tumors of this type recur decades after the initial excision, often showing more
worrisome histologic features in the recurrence, such as nuclear pleomorphism, increased mitoses or
infiltrative margins. These observations raise the intriguing but troublesome possibility that smooth
muscle tumors of the vulva (and perhaps those arising elsewhere) represent a biologic continuum without
clearly definable cut-offs between benign and malignant. Lack of very long-term follow-up, which is
understandably difficult to obtain, limits studies that propose criteria for recurrent potential. We
therefore advocate the use of the concept of "atypical smooth muscle tumor" for lesions which do not
fulfill the criteria for the diagnosis of sarcoma (see below) but which have one of the following
features: infiltrative margin, nuclear atypia or any mitotic activity. The use of this term should also
include a cautionary note that these lesions can be associated with late recurrence and that the patient
should be followed-up. In all cases in which the diagnosis of an atypical smooth muscle tumor is
rendered, at least a 1-cm margin of excision is recommended whenever possible with close, long-term
Histologic criteria for malignancy (metastatic potential)
Distinction between atypical smooth muscle tumors and leiomyosarcoma can also be difficult
and remains a major diagnostic problem with important clinical consequences relative to potential
differences in surgical management. In our opinion, the currently established criteria probably
underdiagnose malignancy; nevertheless, they are useful and appear valid. We utilize the criteria
proposed by Tavassoli and Norris,  which were more recently validated
tumors with three or more of the following features are best classified as sarcomas: 1) > 5 cm in
size 2) infiltrative margins 3) > 5 mitoses / 10 hpf 4) moderate to severe cytologic atypia. It is
our view that the presence of coagulative tumor necrosis is also a histologic feature, which in
combination with any of the four aforementioned ones, should seriously raise the possibility of sarcoma.
Vulvar leiomyomatosis is an unusual but interesting phenomenon that is characterized by multinodular
mucosal-based masses that are the result of a diffuse, often multinodular proliferation of smooth
This process can involve the clitoris leading to clitoral hypertrophy in some
patients. Curiously, patients with vulvar leiomyomatosis can also develop numerous smooth muscle
neoplasms in the esophagus (esophageal leiomyomatosis), which can occur either synchronously or
The pathogenesis of this unusual phenomenon is unclear; however, there
is an association with Alport's syndrome. The molecular genetic basis for Alport's syndrome involves
defects in type IV collagen, which is a major component in all basement membranes (including those of
kidney and smooth muscle cells). Different subtypes of collagen type IV are present in basement
membranes of different tissue types (such as COL4A6 in smooth muscle), and mutations/deletions of this
area can sometimes, but not always, lead to Alport's syndrome in association with
Mesenchymal neoplasms of the vulva can be separated broadly into those that also occur
outside the lower female genital tract and those that are relatively site-specific. The relatively
site-specific lesions should be classified, whenever possible, as distinct entities based upon their
clinical, histologic and biologic differences; however, they can be diagnostically challenging because
not only can they exhibit superficial histologic similarities but also ancillary techniques, such as
immunohistochemistry, are often not helpful. In addition, it is my experience that vulvovaginal stromal
lesions are morphologically heterogeneous and a proportion of cases (particularly in consultation
material) defy precise classification, necessitating the designation 'vulvovaginal stromal tumor NOS'.
As more cases are gathered together, it seems possible that additional reproducible 'entities' may be
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