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Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva 209
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Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 17 Cellular angiofibroma – microscopic features.
Higher magnification reveals numerous thick-walled
small to medium-sized blood vessels as a well as a monotonous population of spindle cells arranged in short fascicles or without a particular architectural pattern.
A fascicular arrangement can also be present. Occasional
blood vessels show hyalinization
Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 18 Cellular angiofibroma – microscopic features.
Tumor cells are short and spindled yet slightly plump
with oval nuclei showing bland features. Occasional mast
cells can be seen in the background. Mitotic activity is not
appreciated in this case; however, brisk mitoses canbe seen
in some examples
are occasionally present (Schoolmeester and
Fritchie 2015). Tumors may have intralesional
adipocytes, which can be prominent in some
cases (Iwasa and Fletcher 2004). Areas of myxoid
matrix can be seen occasionally (Nucci et al.
1997). Brisk mitotic activity can rarely be identi-
fied as well as mild nuclear atypia in the form of
enlargement or hyperchromasia (Iwasa and
Fletcher 2004; Nucci et al. 1997).
Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 19 Cellular angiofibroma – immunohistochemical
features. The majority of cellular angiofibromas (~60%)
strongly and diffusely express CD34
M
Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 20 Cellular angiofibroma – immunohistochemical
features. Loss of nuclear Rb expression in tumor cells is
typical. Retained expression is seen in a background blood
vessel as well as in scattered infiltrating inflammatory cells
Immunophenotype
The majority of tumors (60%) express CD34
[Fig. 19; (Iwasa and Fletcher 2004)]. Smooth
muscle markers such as SMA and desmin are
expressed in only a small fraction of tumors:
21% and 8%, respectively (Iwasa and Fletcher
2004). Nuclear expression of Rb is lost, similar
to mammary-type myofibroblastoma (MTMF)
and spindle cell lipoma (SCL) [Fig. 20; see
“Molecular Features” below; (Chen et al. 2012)].
ER and/or PR reactivity is seen in a majority of
cases.
210 Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva
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Molecular Features
Cellular angiofibroma is genetically comparable
to MTMF and SCL, which also show loss of
13q14. This region contains the RB1 gene that
encodes the Rb protein, an important tumor suppressor (Chen et al. 2012).
Differential Diagnosis
Cellular angiofibroma can be mistaken for solitary
fibrous tumor (SFT) due to its microscopic
appearance as well as CD34 reactivity. However,
SFT is positive for STAT6 by immunohistochemistry due to recurrent translocations involving its
encoding gene (Doyle et al. 2014). Cellular
angiofibroma also retains expression of nuclear
Rb. Cellular angiofibroma is distinguished from
aggressive angiomyxoma by its more superfi cial
location and well-circumscribed borders.
Angiomyofibroblastoma typically shows regional
variation in cellularity, whereas cellular
angiofibroma is usually uniformly cellular. Additionally, angiomyofibroblastoma contains thinwalled vessels compared to the thick-walled,
often hyalinized vessels of cellular angiofibroma.
Examples of cases with a prominent fascicular
architecture can resemble schwannoma; however,
cellular angiofibroma lacks expression of S100
protein (Iwasa and Fletcher 2004). MTMF and
SCL are morphologically and genetically similar
but arise at different anatomic locations. SCL is
commonest in the head and neck and shoulder
region, while MTMF most commonly occurs in
the breast, with occasional cases occurring in the
inguinal/perineal region in addition to other anatomic sites (Chen et al. 2012). In comparison to
cellular angiofibroma, although morphologically
similar, MTMF has less prominent vessels and
contains interspersed coarse hyalinized co llagen
bundles (Chen et al. 2012; Crum et al. 2018).
Fibroepithelial Stromal Polyp
Definition
Fibroepithelial stromal polyp (FESP) is a benign
tumor that occurs most commonly in the vagina
and vulva of reproductive-aged women. FESP is
thought to be distinct from fibroepithelial polyp
due to its more prominent stroma that typically
expresses hormone receptors.
Clinical Features
• Incidence
Uncommon.
• Age
Reproductive-aged females are the most com-
monly affected patient population. However,
they can develop in the setting of hormone
replacement therapy as well as in the pediatric
population. Lesions can often develop during
pregnancy, which may involve the develop-
ment of multiple lesions (Nucci et al. 2000;
O’Quinn et al. 1982).
• Sex
This tumor occurs exclusively in females.
• Site
Vagina and vulva are the most common ana-
tomic sites. The cervix is frequently affected.
• Treatment
Simple excision is typically curative.
• Outcome
Local recurrence is rare.
Macroscopy
Lesions are polypoid or pedunculated in appearance and are located superficially.
Microscopy
Microscopic examination reveals a polypoid
tumor that shows epithelial hyperplasia (Fig. 21).
Lesions lack an identifiable interface with normal
background stroma (Nucci et al. 2000). A central
fibrovascular core containing thick-walled vessels
is typically present (Fig. 22). Stromal edema can
be a feature. Dilated thin-walled vessels are often
present (Fig. 23), particularly in the superficial
aspect of the lesion (O’Quinn et al. 1982). Stromal
cells are usually spindled to stellate in appearance
with bland nuclear features (Figs. 24, 25, and 26).
Pseudosarcomatous change such as nuclear
atypia, increased cellularity, and increased mitotic
activity exceeding 10 per 10 high-power fields
may be present (Nucci et al. 2000; Schoolmeester
and Fritchie 2015). Atypical mitoses and bizarre
cytomorphology may also be identified. These
feature has given rise to the term “pseudosarcoma
Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva 211
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Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 21 Fibroepithelial stromal polyp – microscopic fea-
tures. Scanning magnification reveals a polypoid lesion
with fibrous stroma and overlying epithelial hyperplasia.
Numerous thin-walled blood vessels are seen in the
background
Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 22 Fibroepithelial stromal polyp – microscopic fea-
tures. This example shows a prominent central fibrovascular core, which is seen in most examples. Note the
edematous stroma in this example
botryoides.” Multinucleate stromal cells may
often be present and can be an important diagnostic clue (Nucci et al. 2000).
Immunophenotype
FESPs strongly and diffusely express ER and
PR. Desmin expression, which can be diffuse
(Figs. 27 and 28), is seen in a minority of cases.
Differential Diagnosis
The differential diagnosis includes other soft tissue tumors of the female reproductive tract, which
Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 23 Fibroepithelial stromal polyp – microscopic fea-
tures. Foci of storiform architecture are seen in this example. Background prominent thin-walled vessels are present.
Note the uniform density of cellularity throughout, without
an increase near the stromal-epithelial junction. Additionally, lesional cells are present immediately adjacent to the
stromal-epithelial interface
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Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 24 Fibroepithelial stromal polyp – microscopic fea-
tures. High-power image showing spindled to stellate stromal cells with bland nuclear features
all show somewhat similar histologic features and
require careful histopathologic examination to
separate. The presence of pseudosarcomatous
change can raise consideration for leiomyosaroma
or rhabdomyosarcoma and can often result in
misclassification as a malignancy (Nucci et al.
2000). In contrast to the botryoid variant of
embryonal rhabdomyosarcoma, FESP does not
show an increase in cellularity near the stromalepithelial junction, termed the cambium layer
(Fig. 23). Additionally, FESP lacks an identifiable
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Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 25 Pseudosarcomatous fibroepithelial stromal
polyp – microscopic features. Increased cellularity with
nuclear enlargement and hyperchromasia can be seen in
pseudosarcomatous examples of fibroepithelial stromal
polyp
Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 26 Pseudosarcomatous fibroepithelial stromal
polyp – microscopic features. Areas of bizarre
cytomorphology are seen with hyperchromatic, smudgy
nuclei
lesion/normal stroma interface and often contains
multinucleate stromal cells. Aggressive
angiomyxoma does not present as a polypoid
mass, instead arising in deep soft tissue with an
infiltrative border. Angiomyofibroblastoma presents as a subcutaneous mass rather than a
polyp. Cellular angiofibroma shows loss of Rb
by immunohistochemistry and often contains
hyalinized vessels. Superficial angiomyxoma
often shows a multifocal growth pattern and may
contain epithelial structures such as cysts or
abnormal hair follicles.
Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 27 Pseudosarcomatous fibroepithelial stromal
polyp – microscopic features. Numerous multinucleated
stromal cells are identified, which can be a useful diagnostic feature. Note the presence of enlarged nuclei with vesicular chromatin and prominent nucleoli
Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 28 Pseudosarcomatous fibroepithelial stromal
polyp – immunophenotypic features. Strong and diffuse
expression of desmin can be seen in a minority of cases,
which can increase the potential for confusion with
leiomyosarcoma or rhabdomyosarcoma
Granular Cell Tumor
Definition
The vast majority of granular cell tumors (GCTs)
are benign and are distinguished by their characteristic cytologic appearance. These nerve sheath
tumors occur at a wide range on anatomic locations including cutaneous, mucosal (particularly
tongue), and rarely visceral sites.
Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva 213
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Clinical Features
• Incidence
Uncommon.
• Age
Vulvar GCT is most common in adults in the
fourth to sixth decade of life, but a wide age
range is affected (Horowitz et al. 1995;
Machado et al. 2016). Vulvar GCTs can also
occur in the pediatric population (Schmidt
et al. 2003).
• Sex
The tumor is usually reported to have a female
predilection (Horowitz et al. 1995).
• Site
The tumor has a propensity for mucosal and
soft tissue of the head and neck region (Billings
et al. 2019), with the tongue being the most
commonly affected anatomic site (Lack et al.
1980), but has a wide anatomic distribution.
Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 29 Granular cell tumor – microscopic features. At
scanning magnification, this example of a granular cell
tumor occurring in the perianal area shows diffuse involvement of the dermis and an infiltrative border at the base that
extends i nto skeletal muscle of the external anal sphincter
Perianal and vulvar tumors are not uncommonly encountered. The labium majus is the
most commonly involved anatomic location of
the vulva (Horowitz et al. 1995). Other
reported sites include the clitoris and perineum.
• Treatment
Simple excision is typically curative (Billings
et al. 2019).
• Outcome
Local recurrence of benign granular cell
tumors is uncommon (Lack et al. 1980). Malignant GCTs are extremely rare and only comprise approximately 2% of GCTs (PérezGonzález et al. 2015). Malignant granular cell
tumors of the vulva have been described
(Horowitz et al. 1995; Schmidt et al. 2003).
Macroscopy
GCTs typically present as a subcutaneous or
dermal-based mass. Tumors can rarely be multicentric (Fanburg-Smith et al. 1998).
Microscopy
Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 30 Granular cell tumor – microscopic features. This
example of granular cell tumor shows marked pseudoepitheliomatous hyperplasia of the overlying epidermis
which can be mistaken for invasive squamous cell carcinoma, particularly in superficial biopsies. The characteristic epithelioid tumor cells with abundant granular
cytoplasm can be seen in surrounding nests of squamous
epithelium. Note the absence of cytologic atypia within
keratinocytes
Microscopic examination reveals an unencapsulated
dermal-based tumor with a poorly circumscribed
border that can infiltrate into underlying skeletal
muscle (Fig. 29). Marked pseudoepitheliomatous
hyperplasia is commonly seen in the overlying epidermis, which can mimic squamous cell carcinoma
(Fig. 30). Tumor cells usually contain small nuclei
with condensed chromatin and a re arranged in nests
and trabeculae that intercalate among dermal collagen fibers (Fig. 31); however, enlarged nuclei with
vesicular chromatin can be present focally in benign
M
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Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 31 Granular cell tumor – microscopic features.
Tumor cells are epithelioid with abundant granular, eosinophilic cytoplasm. Tumor cells often form small nests or
trabeculae. Cell borders are indistinct, giving rise to a
syncytial appearance. Nuclei are typically small with condensed chromatin
Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 32 Granular cell tumor – microscopic features. This
example of a malignant granular cell tumor metastatic to a
regional lymph node shows geographic necrosis
GCTs and should not be interpreted as evidence of
malignancy (Billings et al. 2019). Criteria for malignancy inc lude the following histopathologic characteristics: necrosis (Fig. 32), spindle cell morphology,
vesicular nuclei with large nucleoli (Fig. 33), elevated mitotic activity (>2 mitoses/10 high-power
fields at 200 magnification; Fig. 33), high nuclear
to cytoplasmic ratio, and cellular pleomorphism
(Fig. 33). Tumors that satisfy three of six of these
Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 33 Granular cell tumor – microscopic features.
High-power view of the malignant granular cell tumor in
Fig. 32 exhibiting enlarged nuclei with pleomorphism,
vesicular chromatin, prominent nucleoli, and numerous
mitotic figures
criteria are classified as histologically malignant,
one to two of six as atypical, and those that show
only focal pleomorphism but do not fulfil any of the
other criteria are classified as benign (FanburgSmith et al. 1998). Metastasis, however, remains
the sole unequivocal sign of malignancy, and tumors
classified as histologically atypical rarely metastasize (Machado et al. 2016). Perineural growth is a
common finding in GCT and should not be
interpreted as evidence for malignant behavior
(Fig. 34).
Immunophenotype
Tumor cells are usually strongly and diffusely
positive for S100 protein (Fig. 35 ) and SOX-10.
GCTs do not typically express specific markers of
melanocytic differentiation such as Melan-A or
HMB-45. CD68 and NKI/C3 are expressed in
the majority of tumors. Neuron-specific enolase
(NSE) is typically also expressed. Chromogranin
is typically negative (Machado et al. 2016).
Malignant granular cell tumors have a similar
immunohistochemical profile (Fanburg-Smith
et al. 1998).
Molecular Features
Children with several genetic syndromes are at
increased risk for developing multiple GCTs.
Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva 215
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granular cell tumors harbor recurrent mutations
in PIK3CA, potentially providing a therapeutic
target (Machado et al. 2016).
Differential Diagnosis
Due to the frequent presence of pseudoepitheliomatous hyperplasia, granular cell tumor
can often be mistaken for invasive squamous cell
carcinoma, which is perhaps the most important
diagnostic pitfall (Wolber et al. 1991). Strong
S100 protein and SOX-10 positivity can cause
confusion with melanocytic lesions. Additionally,
rare cases of GCT express Melan-A, which can
Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 34 Granular cell tumor – microscopic features.
Benign granular cell tumors often show perineural growth,
which should not be interpreted as evidence for
malignancy
cause considerable confusion, as most GCTs do
not express specific melanocytic markers. The
cytologic features, nested appearance, and TFE3
positivity can raise consideration for alveolar soft
parts sarcoma (ASPS), which commonly arises in
deep soft tissue. ASPS does not typically express
S100 protein or SOX-10, and characteristically
harbors the ASPL-TFE3 translocation, which is
not seen in GCT. ASPS shows PAS-positive,
diastase-resistant crystals within the cytoplasm of
tumor cells. However, the cytoplasmic granules of
GCTs, which represent lysozymes, are also PASpositive and diastase resistant (Crum et al. 2018).
Furthermore, although uncommon, smooth muscle
tumors such as leiomyoma can show granular cell
change (Shimokama and Watanabe 1992). A small
immunohistochemical panel that includes S100
protein, smooth muscle actin (SMA), and desmin
Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 35 Granular cell tumor – immunohistochemical fea-
tures. Tumor cells typically strongly and diffusely express
S100 protein (nuclear and cytoplasmic). Expression of
specific melanocytic markers such as Melan-A or HMB45 is usually not observed
can resolve this differential diagnosis.
Nodular Fasciitis
Definition
Nodular fasciitis is a benign yet rapidly growing
The occurrence of multiple GCTs has been
observed in a patient with PTEN hamartoma
tumor syndrome (Marchese et al. 2003) and has
been described in patients with LEOPARD and
neoplasm that is transient, often completely
resolving without treatment. Nodular fasciitis is
known to raise clinical concern for malignancy
due to its rapid growth.
Noonan syndromes with germline PTPN11 mutations (Ramaswamy et al. 2010; Schrader et al.
2009). One report has demonstrated mutations in
KDR, GNAQ, and ATM; however, driver mutations in PTPN11 or PTEN were not detected in
sporadic GCTs (França et al. 2018). Malignant
Clinical Features
• Incidence
Uncommon.
• Age
Young adults.
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• Sex
Males and females are equally affected.
• Site
The upper limbs, particularly the forearm, are
the most common sites of involvement; however, a wide range of anatomic locations have
been described, including the subcutaneous
tissue of the vulva (O’Connell et al. 1997 ;
Pichler Sekulic and Sekulic 2016), most commonly the labia.
• Treatment
Simple excision is typically curative (Oliveira
and Chou 2014).
• Outcome
Local recurrence, even after incomplete excision, is rare (O’Connell et al. 1997). Tumors
left untreated often resolve spontaneously
Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 36 Nodular fasciitis – microscopic features. Nodular
fasciitis shows alternating areas of cellularity with a
myxoid background. Cystic degeneration is common
(André M. Oliveira and Chou 2014).
Macroscopy
Nodular fasciitis usually presents as a rapidly
growing subcutaneous mass; however, tumors
that have been stable for a number of years also
occur. Tumors may rarely arise in the dermis
(Goodlad and Fletcher 1990).
Microscopy
Microscopic examination reveals an often infiltrative tumor wit h varying levels of cellularity
by geographic region (Fig. 36). The stroma is
characteristically myxoid, and cystic degeneration is common. Tumor cells are characteristically arranged in short, intersecting fascicles
resembling tissue culture fibroblasts (Fig.
37). Hypocellular areas have a characteristically
“feathery” appearance (Fig. 38). Cytologic
atypia is absent; however, brisk mitotic activity
is readily detected (Fig. 39). The composite cells
are myofibroblastic in appearance with vesicular
chromatin and pale to slightly eosinophilic cytoplasm. Extravasated red blood cells are often
prominent.
Immunophenotype
Tumor cells are usually strongly and diffusely positive for smooth muscle actin (Fig. 40). However,
other markers of myoid differentiation, such as
Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 37 Nodular fasciitis – microscopic features. Within
more cellular areas, tumor cells are arranged in short,
intersecting fascicles resembling tissue culture fibroblasts
desmin, are typically negative. Markers of nerve
sheath differentiation, such as S100 and SOX-10,
are not expressed. CD34 is typically negative.
Molecular Features
Nodular fasciitis is characterized by recurrent
translocations involving USP6. Translocations
involving USP6 are not specific to nodular fasciitis, however, and can be found in cellular fibroma
of tendon sheath (Carter et al. 2016), aneurysmal
Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva 217
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Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 38 Nodular fasciitis – microscopic features. Hypo-
cellular areas often have a “feathery” appearance
Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 39 Nodular fasciitis – microscopic features. Tumor
cells lack cytologic atypia and have vesicular chromatin;
however, mitoses are usually readily detected. Extravasated red blood cells are a common occurrence
bone cyst (Oliveira et al. 2004), and giant cell
reparative granuloma of the hands and feet
(Agaram et al. 2014).
Differential Diagnosis
Due to its rapid growth, abundant mitotic activity,
and often infiltrative appearance, nodular fasciitis
can be mistaken clinically and histologically for
a sarcoma. However, the absence of cytologic
atypia and distinctive appearance at scanning
magnification lead to the correct diagnosis.
Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 40 Nodular fasciitis – immunohistochemical fea-
tures. Tumor cells typically strongly express smooth muscle actin but are negative for desmin
Myxofibrosaroma is distinguished by the presence
of “curvilinear” vessels and presence of cytologic
atypia. Low-grade fibromyxoid sarcoma, which at
low magnificationshows well-demarcatedalternating myxoid and collagenous areas, usually
expresses MUC4 by immunohistochemistry.
Desmoid fibromatosis characteristically contains
longer fascicles of tumor cells, and most cases
show nuclear expression of beta catenin.
Dermatofibroma/benign fibrous histiocytoma
shows collagen entrapment at the periphery and a
storiform to fascicular growth pattern. Although
the myxoid variant of dermatofibrosarcoma protuberans in particular can mimic nodular fasciitis,
this tumor shows characteristically uniform
cytomorphology with a well-formed storiform
growth pattern and typically lacks brisk mitotic
activity, with lesions growing very slowly over a
period of years before coming to clinical attention.
Smooth muscle tumors such as leiomyoma are
typically well circumscribed and exhibit a fascicular growth pattern, containing spindle cells with
brightly eosinophilic cytoplasm and cigar-shaped,
blunt-ended nuclei. Although nodular fasciitis is
usually positive for smooth muscle actin, desmin,
which is strongly expressed in most leiomyomas, is
usually negative. Leiomyosarcoma usually shows
high-grade nuclear atypia as well as pleomorphism
and may contain tumor necrosis.
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Superficial Angiomyxoma
Definition
Superficial angiomyxoma is a benign, slowgrowing neoplasm that occurs in the dermis and
superficial subcutaneous tissue. Although sporadic cases are common, superficial
angiomyxoma can occur in the context of Carney
complex, an autosomal dominantly inherited disorder with several manifestations including
pigmented skin lesions (lentigines and blue
nevi), myxomatous tumors (including cardiac
myxomas and myxoid fibroadenomas of the
breast), and various endocrine disorders (Carney
et al. 1986). Superficial angiom yxoma in the
external ear appears to be pathognomonic of Carney complex (Ferreiro and Carney 1994).
Clinical Features
• Incidence
Uncommon.
• Age
Adults.
• Sex
Affects males and females equally. Tumors of
the genital region are likely more common in
females (Fetsch et al. 1997).
• Site
Most common on the head and neck but can
arise at a wide variety of anatomic sites, including the vulva, mons pubis, or vagina (Fetsch
et al. 1997).
• Treatment
Simple excision.
• Outcome
Local recurrence can occur (Calonje et al.
1999). Accordingly, complete excision is
recommended.
Microscopy
Histologic examination reveals an ill-defined,
hypocellular tumor composed of bland spindle
cells within a myxoid background (Fig. 41).
Tumors may be multinodular. An increased number of thin-walled blood vessels can usually be
identified (Fig. 42). Mitotic activity is uncommon,
and cytologic atypia is not a feature (Fig. 43).
Epithelial proliferations, particularly cysts, are
often found in association with superficial
Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 41 Superficial angiomyxoma – microscopic features.
Scanning magnification image of a vulvar tumor reveals a
polypoid, superficial dermal-based hypocellular neoplasm
with a myxoid background
Macroscopy
Superficial angiomyxoma presents as a slowgrowing and painless dermal or superficial subcutaneous mass most commonly in the head and
neck area of adults that usually measures less
than 4 cm. Tumors of the vulva and vagina, as
well as scrotum in male patients, however, have
been described and can occur as a polypoid lesion
(Fetsch et al. 1997).
Mesenchymal Tumors and Mixed Epithelial and Mesenchymal Tumors, Pathology of the Vulva,
Fig. 42 Superficial angiomyxoma – microscopic features.
Tumors are typically hypocellular with an increase in thinwalled blood vessels and a myxoid background
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