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148 Glandular Tumors and Precursors, Pathology of the Vulva
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Microscopy
Bartholin glands and ducts are composed of a
variety of cells including squamous (at the vestibular orifice of the duct), transitional (in the duct),
mucinous (in the glands), myoepithelial (at the
periphery of the glands), and neuroendocrine
(in the duct and glands) which may explain the
wide spectrum of carcinomas developing from
them. The histologic types of carcinomas arising
from Bartholin glands are squamous cell carcinoma (31%), adenoid cystic carcinoma (30%),
and adenocarcinoma (25%) (Di Donato et al.
2017). The adenocarcinomas can exhibit a variety
of microscopic patterns including mucinous,
nested solid, papillary, glandu lar, signet ring,
enteric, or clear cell. Rare cases of transitional
cell carcinoma, adenosquamous carcinoma, neuroendocrine carcinoma, myoepithelial carcinoma,
and epithelial myoepithelial carcinoma have also
been described (Desouki and Fadare 2021;Di
Donato et al. 2017; Sahin Aker et al. 2020). In
most cases, a component of b enign Bartholin
glands can be found associated with the
carcinoma.
Immunophenotype
Most cases are positive for ER, PR, CAM5.2,
CEA, and EMA. Cases with an enteric phenotype
are positive for CK20, CDX2+, CEA+, and
CA19–9+ and negative for PAX8, ER, PR, and
GATA3.
Molecular Features
The squam ous cell carcinomas arising in
Bartholin glands are associated with HPV
(Nazeran et al. 2019; Ouldamer et al. 2013).
Differential Diagnosis
Clinical differential diagnosis includes a cyst or an
abscess. Histologic differential diagnosis is with a
squamous cell carcinoma or adenocarcinoma of
the vulva not arising from Bartholin glands or
with a metastasis. The following criteria have
been proposed for a carcinoma to be designated
as Bartholin gland carcinoma: The tumor should
involve the region of Bartholin glands, the tumor
should be histologically compa tible with an origin
from Bartholin glands, there should be an area of
transition between the tumor and benign Bartholin
glands, and there should be no evidence of another
primary tumor (Nazeran et al. 2019).
Bartholin Gland Hyperplasia, Adenoma,
and Adenomyoma
Synonyms
Nodular hyperplasia or Bartholin gland.
Definition
Bartholin gland hyperplasia is defined as a benign
growth of normal Bartholin glands and ducts with
preservation of normal ductal and acinar arrangement. Bartholin gland adenomas are defined as a
growth of tightly packed acini and lobules.
Bartholin gland adenomyoma demonstrates in
addition a fibromuscular component.
Clinical Features
• Incidence
These are uncommon lesions; however, they
are probably underreported (Santos
et al. 2006).
• Age
Age range is 19–56 years with an average of
35 years.
• Site
Posterolateral introitus.
• Treatment
No treatment or simple excision.
• Outcome
These are benign lesions with no tendency for
recurrence.
Macroscopy
Bartholin gland hyperplasia presents as a nontender solid or solid and cystic nonencapsulated
mass, usually less than 5 cm in diameter (Koenig
and Tavassoli 1998; Santos et al. 2006). Adenomas and adenomyomas are usually encapsulated.
Microscopy
Bartholin gland hyperplasia shows increased
number and density of mucinous acini, a lobular
architecture and irregular contour. There is preservation of the normal relationship of acini to
Glandular Tumors and Precursors, Pathology of the Vulva 149
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Definition
Hidradenoma papilliferum (HP) is a benign neoplasm with apocrine features involving the vulva
that presumably arises from the anogenital
mammary-like glands (El-Khoury et al. 2016).
HP is similar to mammary intraductal papilloma
(Konstantinova et al. 2016a). The malignant
counterpart of HP is hidradenocarcinoma
papilliferum, an extremely rare tumor characterized histologically by cytologic atypia and an
invasive component.
Glandular Tumors and Precursors, Pathology of the
Vulva, Fig. 4 Bartholin gland hyperplasia glandular
lesion with irregular contour and lobular architecture composed of numerous mucinous acini and ducts with preservation of the normal relationship of acini to ducts
Clinical Features
• Incidence
HP represents 60% of all vulvar adnexal
tumors, and it is the most common benign
ducts (Fig. 4). Bartholin gland adenoma is usually
well circumscribed and sometimes encapsulated
and demonstrates a dense proliferation of acini
without preservation of the relationship with the
ducts. A fibromuscular component is seen in
adenomyoma (Koenig and Tavassoli 1998; Santos
et al. 2006).
vulvar neoplasms (Baker et al. 2013).
• Age
HP affects women from the third to the ninth
decade with a median age of 49 years
(Konstantinova et al. 2016a).
• Site
HP affects more commonly the interlabial sul-
cus or labia majora at sites where anogenital
Immunophenotype
The glands in Bartholin gland hyperplasia are
positive for CAM5.2, AE1/AE3, high molecular
weight cytokeratin, EMA, and polyclonal CEA
and are negative for p53, ER, PR, and monoclonal
CEA (Santos et al. 2006).
mammary glands are located (El-Khoury
et al. 2016).
• Treatment
Treatment usually includes a minimal surgical
excision.
• Outcome
HP is a benign tumor with an indolent
Differential Diagnosis
behavior.
Bartholin gland hyperplasia, adenoma, and
adenomyoma need to be differentiated from other
causes of Bartholin gland enlargement including
Bartholin duct cyst and Bartholin gland carcinoma.
Bartholin duct cysts show a cystic architecture and
are lined by squamous, transitional, mucinous, ciliated, or flattened nonspecific epithelium often
Macroscopy
HP usually presents as a 1–2 cm solitary, asymptomatic nodule or papule (Konstantinova et al.
2016a; Woodworth et al. 1971). Occasionally the
patient may experience pain, discharge, or
bleeding.
accompanied by acute and chronic inflammation.
Bartholin gland carcinoma shows cytologic atypia
and an infiltrative architecture.
Microscopy
HP presents as a well-circumscribed nodule, solid
or cystic, occasionally ulcerated, involving dermis
or lamina propria and occasionally showing con-
Hidradenoma Papilliferum
tinuity with the epidermis (Fig. 5). Histology
shows papillary processes covered by epithelium
Synonyms
Papillary hidradenoma.
and delimiting arborizing, trabecular spaces
(Fig. 6). In addition, glandular structures of
G
150 Glandular Tumors and Precursors, Pathology of the Vulva
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Glandular Tumors and Precursors, Pathology of the
Vulva, Fig. 5 HP well-circumscribed dermal nodule
containing papillary and glandular structures
Glandular Tumors and Precursors, Pathology of the
Vulva, Fig. 6 HP papillary architecture with an arboriz-
ing or trabecular pattern
variable size may be seen (Fig. 7). The lining of
the papillae is typically double layered with
basally located myoepithelial cells characterized
by oval nuclei and occasional clear cytoplasm and
apical columnar epithelial cells with eosinophilic
cytoplasm and decapitation secretion (Fig. 8). The
epithelial cells contain PAS-positive, diastaseresistant granules. Areas resembling apocrine
metaplasia of the breast with prominent eosinophilia and small nuclei may be seen (Fig. 9). HPs
may exhibit a solid growth pattern, spindle cells,
areas resembling sclerosing adenosis of the breast
or atypical and usual ductal hyperplasia. Rarely,
HPs can demonstrate a connection to the epidermis and associated plasma cells thus resembling
Glandular Tumors and Precursors, Pathology of the
Vulva, Fig. 7 HP mixed papillary and glandular architec-
ture with variable sized ducts
Glandular Tumors and Precursors, Pathology of the
Vulva, Fig. 8 HP the papillae and ducts are lined by a
double layered epithelium with basally located
myoepithelial cells characterized by oval, hyperchromatic
nuclei and occasional clear cytoplasm and apical columnar
epithelial cells with eosinophilic cytoplasm and decapitation secretion
syringocystadenoma papilliferum (Parks et al.
2012). Mitotic figures may be seen; however,
their presence does not impact the benign behavior (Fig. 10; Baker et al. 2013 ; Konstantinova
et al. 2016a).
Immunophenotype
Immunohistochemically, the epithelial cells
express low-molecular weight keratin, CK7,
EMA, CEA, GCDFP-15, and estrogen and progesterone receptors. The myoepithelial cells are
positive for S100 and SMA.
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remarkable for an infiltrative architecture and
demonstrate cytologic atypia.
Paget Disease
Synonyms
Extramammary Paget disease (EMPD); Primary
vulvar Paget disease.
Definition
Primary in situ adenocarcinoma of the vulvar skin
Glandular Tumors and Precursors, Pathology of the
Vulva, Fig. 9 HP prominent eosinophilia and small nuclei
resembling apocrine metaplasia of the breast
Glandular Tumors and Precursors, Pathology of the
Vulva, Fig. 10 HP mitotic figures might be encountered
(arrow), but their presence does not indicate a more aggressive behavior
Molecular Features
A subset of HPs demonstrates mutations in
PIK3CA or AKT1 genes, similar to the alterations
seen in mammary intraductal papilloma (Goto
et al. 2017; Konstantinova et al. 2017b; Liau
et al. 2016; Pfarr et al. 2016).
Differential Diagnosis
The complex architecture of HP which can at
times also exhibit an increased mitotic activity
raises a differential diagnosis with carcinoma,
including hidradenocarcinoma papilliferum. As
opposed to HP which demonstrates a well
circumscribed profile, carcinomas are usually
with predominately intraepithelial growth of tumor
cells. Secondary involvement of skin by a visceral
carcinoma, usually from urogenital or gastrointestinal tracts, is designated as secondary Paget disease.
Clinical Features
• Incidence
Uncommon disease; represents approximately
1% of anogenital neoplasms.
• Age
Affects women >60 years of age (van der
Linden et al. 2016.
• Site
EMPD affects more frequently the labium
majus followed by labium minus and clitoris
and can extend to skin or vaginal mucosa.
• Treatment
Treatment of choice for EMPD is surgical
excision with large margins or even
vulvectomy.
• Outcome
Even with treatment, local recurrences are
quite common ranging from 33% to 60%,
mainly due to the extension of the lesion
beyond the clinically abnormal area. The prog-
nosis is relatively good in the primary cases;
however, mortality is as high as 50% in EMPD
associated with an underlying adnexal or vis-
ceral carcinoma. Poor prognosis is also associ-
ated with nodules in the primary lesion,
elevated CEA levels, tumor invasion level,
and presence of lymph node metastases.
Macroscopy
EMPD presents as a flat erythematous patch with
scales and well demarcated borders. The clinical
G
152 Glandular Tumors and Precursors, Pathology of the Vulva
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differential diagnosis often includes eczema.
Rarely it can present with hyperpigmentation
(pigmented EMPD) or with multifocal lesions
involving genital area and one or both axillae
(Hilliard et al. 2009).
Microscopy
Histologic examination of EMPD shows infiltration of epidermis by large cells with abundant
clear or eosinophilic cytoplasm, distributed single
or in clusters. The atypical cells are concentrated
in the lower half of the epidermis; however, in
some areas, the entire thickness of the epidermis
may be involved (Fig. 11). The cells have large
pleomorphic nuclei and prominent nucleoli.
Occasionally glandular differentiation or signet
ring cells may be seen (Fig. 12) (Kazakov et al.
2011). In pigmented variant of EMPD, a large
number of tumor cells contain melanin pigment
and there is also an increase in epidermal dendritic
melanocytes, this variant often mimicking melanoma in situ (Hilliard et al. 2009; Fig. 13). Most
cases demonstrate involvement of adnexal structures by neoplastic cells (Konstantinova et al.
2016c). In the anogenital region, EMPD may be
associated with epidermal hyperplasia in the form
of papillomatosis, syringocystadenocarcinoma
papilliferum in situ-like areas, syringoma-like
structures, or lesions mimicking fibroepithelioma
which may hide the histological features of
EMPD (Kazakov et al. 2011; Konstantinova
et al. 2016b). In 7–12% of cases, EMPD is associated with invasive carcinoma within the dermis
(Fig. 14) (Desouki and Fadare 2021). Cases with
dermal invasion depth > 1 mm seem to b e associated with a higher incidence of nodal metastases
Glandular Tumors and Precursors, Pathology of the
Vulva, Fig. 11 EMPD low power shows atypical cells
concentrated predominantly in the lower half of the epidermis; however, in some areas, the entire thickness of the
epidermis may be involved
Glandular Tumors and Precursors, Pathology of the
Vulva, Fig. 12 EMPD the cells have large pleomorphic
nuclei and prominent nucleoli. Occasionally glandular differentiation or signet ring cells may be observed
Glandular Tumors and Precursors, Pathology of the
Vulva, Fig. 13 Paget disease Pigmented EMPD showing
atypical Paget cells with cytoplasmic melanin (red arrows).
A population of reactive dendritic melanocytes is also
noted (brown arrow)
Glandular Tumors and Precursors, Pathology of the
Vulva, Fig. 14 Invasive EMPD showing invasive adeno-
carcinoma cells in the dermis
Glandular Tumors and Precursors, Pathology of the Vulva 153
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(Shiomi et al. 2013). Secondary EMPD is associated with an underlying invasive carcinoma, usually of urogenital or gastrointestinal origin.
Immunophenotype
The tumor cells contai n usually mucin and stain
positive with diastase-PAS, mucicarmine, and
alcian blue. By immunohistochemistry, EMPD
cells express low molecular weight cytokeratins
(CK7, Cam 5.2), AE1/AE3, and EMA (Battles
et al. 1997; Fig. 15). About 50% of EMPD cases
are positive for GCDFP-15 (usually positive in
primary disease), and there is variable expression
of CEA. EMPD is also reported to express in some
cases androgen receptor and HER-2/neu raising
the possibility for targeted therapy; however, they
are usually negative for estrogen and progesterone
receptors (Liegl et al. 2005). CD23 has been
found to be expressed in EMPD (and also in
mammary Paget’s) and was negative in lesions
in the differential diagnosis including Bowen’s
disease, melanoma in situ, and sebaceous carcinoma thus being proposed as a useful diagnostic
marker (Carvalho et al. 2007).
number alteration burden seems to correlate with
prognosis (Ishida et al. 2021).
Differential Diagnosis
Differentiating primary from secondary EMPD
has signifi cant clinical consequences. Usually
clinico-pathologic correlation is required for a
correct diagnosis; however, histologic and
immunophenotypic characteristics can be helpful.
A colorectal origin may be suggested by the presence of dirty necrosis (Fig. 16 ). Primary EMPD is
positive for MUC1 and MUC5AC, negative for
MUC2, and demonstrates variable expression of
CK20, while secondary EMPD is less likely to
express MUC5AC and may label with CK20,
CDX2, and MUC2, especially cases associated
with rectal adenocarcinoma (Kuan et al. 2001;
Figs. 17 and 18). Other entities in the differential
G
Molecular Features
ERBB2 mutations/amplification, ERBB3 mutations, and FGFR1 amplifications have been
found to occur in a mutually exclusive manner
and are proposed to represent driver alterations
for EMPD. Other abnormalities include mutations
in KMT2C, TP53, PIK3CA, NUP93, AFDN, and
CUX1 genes and recurrent deletions of regions
spanning CDKN2A gene. In addition, copy
Glandular Tumors and Precursors, Pathology of the
Vulva, Fig. 15 Paget disease Paget cells are positive for
CK7
Glandular Tumors and Precursors, Pathology of the
Vulva, Fig. 16 Secondary EMPD from a colorectal car-
cinoma. The presence of dirty necrosis is a clue to
diagnosis
Glandular Tumors and Precursors, Pathology of the
Vulva, Fig. 17 Secondary EMPD from a colorectal car-
cinoma. The tumor cells are positive for CK20
154 Glandular Tumors and Precursors, Pathology of the Vulva
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Di Donato, V., Casorelli, A., Bardhi, E., Vena, F., March-
etti, C., Muzii, L., & Benedetti Panici, P. (2017).
Bartholin gland cancer. Critical Reviews in Oncology/
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critrevonc.2017.06.005.
El-Khoury, J., Renald, M. H., Plantier, F., Avril, M. F., &
Moyal-Barracco, M. (2016). Vulvar hidradenoma
papilliferum (HP) is located on the sites of mammary-
like anogenital glands (MLAGs): Analysis of the pho-
tographs of 52 tumors. Journal of the American Acad-
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10.1016/j.jaad.2016.01.034.
Goto, K., Maeda, D., Kudo-Asabe, Y., Hibiya, T., Hayashi,
Glandular Tumors and Precursors, Pathology of the
Vulva, Fig. 18 Secondary EMPD from a colorectal car-
cinoma. The tumor cells are positive for CDX2
diagnosis include high- grade squamous
intraepithelial lesion, melanoma in situ (in the
case of pigmented EMPD), pagetoid dyskeratosis,
vulvar Toker cells, and clear cell papulosis.
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Lymphoid and Myeloid
Tumors, Pathology of the
Fallopian Tube
Isabel Alvarado-Cabrero
Department of Pathology, Mexican Oncology
Hospital, Mexico City, Mexico
Malignant Lymphomas of the
Fallopian Tube
Synonyms
Lymphoid tumors.
Definition
Malignant neoplasms composed of lymphoid cells.
Clinical Features
• Incidence
Primary tubal lymphomas are exceedingly
rare; on the other hand, the fallopian tube is
involved in many cases of malignant lymphoma of the female genital tract, including
approximately one-quarter of the cases in
which the tumor presents as an ovarian mass
(Scully et al. 1998).
• Age
These tumors may be present in young adults
as wel l as in elderly women (Noack et al. 2002;
Alduaij et al. 2010).
• Site
Fallopian tube.
• Clinical Presentation
Patients may presen t with abdominopelvic
pain and/or mass; however, some cases are
incidental findings (Noack et al. 2002).
• Treatment
Treatment options include surgery or radio-
therapy for patients with localized disease,
and single-agent chemotherapy such as an
anthracycline-based regimen for patients with
disseminated disease (Cho et al. 2011).
• Outcome
The prognosis of primary lymphomas of the
fallopian tube is similar to that of nodal lym-
phomas of comparable stage. Lymphoid neo-
plasms are staged according to the Lugano
classification (Cheson et al. 2014).
Macroscopy
Tumors are not grossly recognizable (Alduaij
et al. 2010) or could be present as bulky masses
in extranodal involvement of the fallopian tube
(Nezhat et al. 2013).
Microscopy
The morphology of malignant lymphomas in the
fallopian tube is comparable to that in nodal and
other extranodal sites (Fig. 1). Primary tubal lymphomas cases reported include the following:
marginal zone B-cell lymphoma of MALT-type
© Springer Nature Switzerland AG 2023
S. Stolnicu, R. Ali-Fehmi (eds.), Gynecologic Pathology, Encyclopedia of Pathology,
https://doi.org/10.1007/978-3-030-97378-0
158 Lymphoid and Myeloid Tumors, Pathology of the Fallopian Tube
https://t.me/med1917
• Age
There is a wide age range, with the median
patient age being in the third or fourth decade
of life (Magdy et al. 2019).
• Site
Fallopian tube.
• Clinical Presentation
The clinical presentation of myeloid
sarcoma depends on its location, and the pre-
senting symptoms usually occur due to mass
effect exerted by the tumor or organ dysfunc-
tion due to infiltration (Tripathi et al. 2005).
Lymphoid and Myeloid Tumors, Pathology of the
Fallopian Tube, Fig. 1 Lymphoma of the fallopian
tube. The tumor is invading the fimbriated end
• Treatment
The treatment strategy largely depends on
timing of the presentation of myeloid sarcoma
(MS): either with acute myeloid leukemia
(AML) at presen tation or isolated at relapse
(Magdy et al. 2019).
(Cho et al. 2011; Nezhat et al. 2013; Noack et al.
2002), follicular lymphoma (Alduaij et al. 2010 ),
and a bilateral T-cell lymphoma (Gaffan et al.
2004).
• Outcome
Patients presenting with myeloid sarcoma have
better outcomes than those with AML
(Hernández et al. 2002).
Immunophenotype
Immunophenotyping of primary fallopian tube
lymphomas is similar to that of nodal lymphomas.
Differential Diagnosis
Malignant lymphomas of the fallopian tube
should be differentiated from undifferentiated carcinomas and other small round cell tumors with
appropriate stains (e.g., Cytokeratins).
Myeloid Tumors
Synonyms
Myeloid neoplasms; Myeloid sarcoma.
Definition
Malignant neoplasms of hematopoietic origin
composed of primitive myeloid cells.
Clinical Features
• Incidence
Involvement of the fallopian tube by myeloid
neoplasms is rare (Oliva et al. 1997).
Macroscopy
These lesions often grow as a solid mass (Garcia
et al. 2006).
Microscopy
Microscopic examination reveals a predominantly
diffuse pattern of growth, but cords and pseudoacinar spaces are also present focally in several
cases. Maturing cells with recognizable myeloid
differentiation may be seen (Oliva et al. 1997).
Immunophenotype
Tumor cells are positive for CD13, CD33, CD34
(blasts), CD43, CD68, CD117, myeloperoxidase,
and lysozyme (Garcia et al. 2006).
Molecular Features
Molecular analysis should be performed as needed.
References and Further Reading
Alduaij, A., Hansen, K., & Zhang, C. (2010). Primary
follicular lymphoma of the fallopian tube found inci-
dentally in a patient treated for endometrial carcinoma:
a case report. Diagnostic Pathology, 28(5), 44–48.
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