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Miscellaneous Tumors, Pathology of the Ovary 289
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EWT may lead physicians to recommend
excessively aggressive adjuvant chemotherapy, given many EWT are likely adequately
treated with surgery alone.
• Outcomes
All the patients were alive at their respective
follow-ups, running from 6 months to 9 years
(Isaac et al. 2000; Oner et al. 2002; Pereira
et al. 2000; Sahin and Benda 1988).
Macroscopy
This tumor tends to be unilateral, ranging from
12 to 19 cm in the reported cases. The y are usually
solid wi th cystic and necrotic areas; however, they
may also be multilocular.
Microscopy
The cases present with a mixture of blastema,
epithelium (tubular and glomeruloid structures),
and mesenchymal tissue. The EWTs appear to
arise from persistent mesonephric duct remnants,
while teratoid EWTs arise from misplaced totipotent nephrogenic blastemal elements. The
teratoid extrarenal Wilms tumor may show
areas of mature teratoma and multiple foci of
the triphasic tumor.
Immunophenotype
Tumor cell stain positive for WT1, CD99, and
NSE, and negative for CD10, GFAP, ER, PR,
and inhibin.
Differential Diagnosis
The differential diagnosis includes retiform
Sertoli-Leydig cell tumors that can show glandular, papillary, and spindle cell stroma with heterologous elements. Immunohistochemistry show
tumor cells to stain positive for inhibin.
Paraganglioma
Synonyms
Pheochromocytoma.
Definition
A neuroendocrine neoplasm usually arising in
specialized neural crest cells associated with autonomic ganglia (paraganglia).
Clinical Features
• Incidence
This is a rare tumor.
• Age
The range of reported cases is between 15 and
68 years (Elliot et al. 2012; Fawcett and
Kimbell 1971; Mahdavi et al. 2003;
McCluggage and Young 2006).
• Clinical presentation
Patients present with symptoms referable to an
ovarian mass or with hypertension secondary
to elaboration of epinephrine or norepineph-
rine (Elliot et al. 2012; Fawcett and Kimbell
M
Miscellaneous Tumors,
Pathology of the Ovary,
Fig. 4 Paraganglioma:
Section showing the
characteristic nested (zelle
ballen) growth pattern of
neoplastic cells
290 Miscellaneous Tumors, Pathology of the Ovary
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1971; Mahdavi et al. 2003; McCluggage and
Young 2006). Two cases were incidental findings within an ovarian teratoma (Elliot et al.
2012; Mahdavi et al. 2003).
• Sex
Female.
• Site
Ovary.
• Outcomes
In a series of three reported cases, there was
extra-ovarian involvement in two of the three
cases (McCluggage and Young 2006). The
data regarding long-term implications of this
entity as a whole are limited.
Miscellaneous Tumors, Pathology of the Ovary,
Fig. 5 Paraganglioma: The neoplastic cells are round
and fairly monotonous. Interspersed larger sized
sustentacular cells are also seen
Macroscopy
The tumors have been solid with a brown, tan, or
yellow color, ranging in size up to 22 cm. Two
cases were found within ovarian teratomatous
neoplasms (Elliot et al. 2012; Mahdav i
et al. 2003).
germline SDHB mutation. Such tumors more
commonly have malignant behavior. Less commonly associated with von Hippel Lindau
disease.
Microscopy
These tumors are composed of groups of polygonal cells with an epithelioid appearance
arranged in nests (“ zellballen”) separated by
stroma and thin-walled vascular channels
(Fig. 4). The nuclei of these polygonal cells
tend to be central, and cytoplasm is eosinophilic
or clear with granularities. There may be multinucleated giant cells. The mitotic activity is usually low (Fig. 5).
Immunophenotype
The tumor cells stain positive for neuroendocrine
markers, such as chromogranin, synaptophysin,
and CD56. They tend to be negative for
cytokeratin and EMA. Select cases have S100
staining that highlights sustentacular cells around
the periphery of the nests of polygonal cells (Elliot
et al. 2012, McCluggage and Young 2006). Cases
may stain positive for inhibin or calre tinin
(McCluggage and Young 2006).
Molecular Features
Cases show frequent association w ith her ed itar y
neoplasia syndromes that include frequently
Differential Diagnosis
Granular cell tumor with lack of fine vascular
network may possess a diagnostic challenge.
These tumor cells show abundant eosinophilic
cytoplasm and diffuse S100 staining. Carcinoid
tumor of the ovary may also show the similar
immunoprofile; however, they usually lack the
classic zellballen pattern and the sustentacular
cells.
Solid Pseudopapillary Neoplasm
Synonyms
Solid and pseudopapillary tumor.
Definition
A tumor morphologically identical to the neoplasm of the same name in the pancreas.
Clinical Features
• Incidence
This is an extremely rare tumor.
• Age
Patients range in age from 17 to 57 years old.
Miscellaneous Tumors, Pathology of the Ovary 291
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• Clinical presentation
Patients present with nonspecific symptoms
related to an ovarian mass (Cheuk et al. 2011;
Deshpande et al. 2010).
• Sex
Female.
• Site
Ovary.
• Outcomes
One of the three patients with follow-up died
of the disease. The fatal tumor exhibited
necrosis, lymphovascular invasion, and a
higher mitotic rate than the other tumors
(Cheuk et al. 2011; Deshpande et al. 2010;
Syriac et al. 2012).
Macroscopy
Tumors are often larger than 10 cm, containing solid
and cystic areas on cut surfaces, with the solid
component being friable and ranging from yellow
to tan (Cheuk et al. 201 1; Deshpande et al. 2010).
Microscopy
The tumor cells tend to grow in sheets and nests,
while less commonly growing in cords and
pseudopapillae. The nests and sheets are
surrounded by septae that may be hyalinized
and contain delicate vascularity. The pseudopapillae have myxoid or myxohyaline cores.
These are lined by one to several cell layers.
Microcysts filled with colloid-like material may
be present. The cells have pale, eosinophilic
cytoplasm and often contain perinuclear vacuoles and intracellular eosinophilic globules. The
nuclei are uniform and round and may contain
grooves (Cheuk et a l. 2011; Deshpande
et al. 2010).
Immunophenotype
The tumor cells have nuclear and cytoplasmic
beta-catenin positivity and lack E-cadherin
staining. They are diffusely positive for CD56
and have focal membranous positivity for
CD117 as well as CD 10 positivity. They are
negative for cytokeratins, chromogranin, inhibin,
and calretinin (Cheuk et al. 2011; Deshpande
et al. 2010).
Molecular Features
As with pancreatic solid pseudopapillary neoplasm, CTNNB1 mutations are usually present.
Differential Diagnosis
Microcystic stromal tumor may also show a
vague lobulated and diffuse growth of cells
with solid and cystic areas, with collagenous
bands. Cytology of the cells is usually bland.
Tumor cells stain positive for cyclin D-1,
vimentin, CD10, and E-cadherin. These tumors
are EMA negative.
Malignant Mesothelioma
Synonyms
Mesothelioma.
Definition
Tumor of the mesothelial lining involving one or
both ovaries.
Clinical Features
• Incidence
Rare, mostly bilateral. Ranges in size from 5 to
15 cm.
• Age
Wide age range (30–85).
• Clinical presentation
Usually presents with abdominal pain, with/
without ascites.
• Sex
Female.
• Site
Ovary.
• Treatment
Bilateral salpingo-oophorectomy, staging
debulking, with/without chemotherapy.
• Outcomes
Relatively good if confined to the ovary. Poor
outcome is peritoneal involvement is seen.
Macroscopy
Gross examination usually reveals a white, mostly
solid and homogeneous mass that may show
M
292 Miscellaneous Tumors, Pathology of the Ovary
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focal friable areas. Small cystic component occasionally seen. Residual ovary may be encased
completely by the tumor. Papillary excrescences
can also be appreciated.
Microscopy
Typically involves the surface and parenchyma.
Two histologic subtypes are seen. (1) The epithelial subtype is the most frequent subtype. It shows
a tubulocystic and papillary growths with simple
papillae lined by a single cell layer. Branching
papillae much less common. Diffuse, cord-like,
trabecular, or adenom atoid growths are usually
uncommon.
(2) Biphasic subtype shows both epithelial and
spindle cell component. It shows a prominent
fascicular growth common with variable cellularity. Prominent hyalinization of stroma with/without necros is may be seen. Psammoma bodies
often seen in papillary areas. Plasma cell and
lymphocytic inflammatory infiltrate as well as
histiocytes may be present. Mitotic activity is
higher in the biphasic histotype.
Immunophenotype
Tumor cells stain positive for CAM5.2, EMA,
calretinin, WT1, and D2–40; PAX8, Ber-ep4,
and CEA are usually negative; and BAP1 is
often lost.
Molecular Features
The most frequent genetic alteration noted was
biallelic inactivation of the BAP1 gene. Recurrent
alterations in epigen etic regulatory genes SETD2
and DDX3X are also noted.
Differential Diagnosis
Serous carcinoma showing papillary architecture
and psammoma bodies may possess a diagnostic
challenge to mesothelioma. Serous carcinoma
may also show variable sized papillae, significant
nuclear atypia, and are positive for estrogen receptor and negative for D2–40.
References and Further Reading
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j.gore.2016.12.004.
Cheuk, W., Beavon, I., Chui, D. T., & Chan, J. K. (2011).
Extrapancreatic solid pseudopapillary neoplasm:
Report of a case of primary ovarian origin and review
of the literature. International Journal of Gynecologi-
cal Pathology, 30, 539–543.
Deen, S., Duncan, T. J., & Hammond, R. H. (2007). Malig-
nant female adnexal tumors of probable Wolffian origin. International Journal of Gynecological Pathology,
26, 383–386.
Deshpande, V., Oliva, E., & Young, R. H. (2010). Solid
pseudopapillary neoplasm of the ovary: A report of
3 primary ovarian tumors resembling those of the pancreas. The American Journal of Surgical Pathology, 34,
1514–1520.
Devouassoux-Shisheboran, M., Silver, S. A., & Tavassoli,
F. A. (1999). Wolffian adnexal tumor, so-called female
adnexal tumor of probable Wolffian origin (FATWO):
Immunohistochemical evidence in support of a
Wolffian origin. Human Pathology, 30, 856–863.
Dickersin, G. R., & Scully, R. E. (1993). An update on the
electron microscopy of small cell carcinoma of the
ovary with hypercalcemia. Ultrastructural Pathology,
17,411–422.
Dickersin, G. R., Kline, I. W., & Scully, R. E. (1982). Small
cell carcinoma of the ovary with hypercalcemia:
A report of eleven cases. Cancer, 49, 188–197.
Eichhorn, J. H., Bell, D. A., Young, R. H., Swymer, C. M.,
Flotte, T. J., Prefer, R. I., & Scully, R. E. (1992a). DNA
content and proliferative activity in ovarian small cell
carcinomas of the hypercalcemic type. Implications for
diagnosis, prognosis, and histogenesis. American Jour-
nal of Clinical Pathology, 98, 579–586.
Eichhorn, J. H., Young, R. H., & Scully, R. E. (1992b).
Primary ovarian small cell carcinoma of pulmonary
type. A clinicopathologic, immunohistologic, and
flow cytometric analysis of 11 cases. The American
Journal of Surgical Pathology, 16, 926–938.
Elliot, V. J., Shaw, E. C., Walker, M., Jaynes, E., &
Theaker, J. M. (2012). Ovarian paraganglioma arising
from mature cystic teratoma. International Journal of
Gynecological Pathology, 31, 545–546.
Fanghong, L., Szallasi, A., & Young, R. H. (2008).
Wolffian tumor of the ovary with a prominent spindle
cell component: A report of a case with brief discussion
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Fawcett, F. J., & Kimbell, N. K. (1971). Phaeochro-
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Gynaecology of the British Commonwealth, 78,
458–459.
Fukunaga, M., Endo, Y., Miyazawa, Y., & Ushigome,
S. (1997). Small cell neuroendocrine carcinoma of the
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Gao, F. F., Krasinskas, A. M., & Chivukula,
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Miscellaneous Tumors,
Pathology of the Uterine
Corpus
Eman Abdulfatah
University of Michigan, Ann Arbor, MI, USA
Adenomatoid Tumor
Synonyms
Benign mesothelioma of the genital tract.
Definition
• Benign tumor of mesothelial origin.
Clinical Features
• Incidence
Uncommon, <5% of all hysterectomy
specimens.
• Age and epidemiology
Reproductive age (mean 45 years).
• Site
Most common in the fundus, close to cornum,
outer myometrium, subserosal or intramural.
• Treatment
Surgical excision (hysterectomy or rarely sim-
ple excision).
• Outcome
Benign behavior, no reports of recurrence or
malignant transformation.
Macroscopy
• Small, round to oval, well circumscribed.
• Gray-white, rubbery cut surface, may have
small cystic spaces.
Microscopy
• Composed of clefts and spaces lined by cuboidal, low columnar, or flattened epithelial-like
cells (Fig. 1).
• Surrounded by loose and edematous or dense
and hyalinized connective tissue.
• Cells may exhibit marked vacuolation.
• Morphological patterns: adenoid,
angiomatoid, cystic, glandular, solid, tubular,
plexiform, and canalicular.
Immunophenotype
• CK5/6, calretinin, WT1, and D2–40 positive.
• Negative for vascular/endothelial markers
(CD31, CD34, and ERG).
• Negative for CEA, MOC31, BerEP4, and
B72.3.
Differential Diagnosis
• Epithelioid hemangioendothelioma
– Positive for CD34 and factor VIII.
• Lymphangioma
– Positive for CD34, CD31, and factor VIII.
• Signet ring cell carcinoma
– Positive for mucicarmine, EMA, BerEP4,
cytological atypia, and brisk mitosis.
Alveolar Soft Part Sarcoma
Definition
• Malignant neoplasm of uncertain differentiation, possibly of neural origin.
Clinical Features
• Incidence
Very rarely described in the female genital tract.
• Age and epidemiology
Mean age 29 years.
• Site
Most common in uterine corpus and cervix/
lower uterine segment.
• Treatment
Surgical excision (hysterectomy) with or without chemotherapy.
Miscellaneous Tumors, Pathology of the Uterine Corpus 295
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Miscellaneous Tumors,
Pathology of the Uterine
Corpus, Fig. 1 (a and b)
Adenomatoid tumor
composed of clefts and
spaces lined by cuboidal or
flattened cells which
express mesothelial
markers (calretinin in inset),
surrounded by loose and
edematous or dense and
hyalinized connective tissue
• Outcome
Favorable behavior in comparison to the soft
tissue counterparts which might be attributed
to its smaller size in the female genital tract.
Macroscopy
• Tan-gray cut surfaces.
• Foci of hemorrhage and necrosis may
be seen.
Microscopy
• Alveolar nests separated by delicate fibro-
vascular septa and characterized by a uniform population of large polygonal cells
with prominent, single, centrally located
nucleus and eosinophilic, granular cytoplasm (Fig. 2).
M
• Crystalline inclusions and intracytoplasmic
melanin may be seen.
• Rare mitotic activity.
• Vascular invasion may be seen.
Immunophenotype
• TFE3 (strong nuclear staining).
• Desmin, vimentin, cathepsin-K, S100 may be
positive.
• HMB45, melan-A may be positive.
Molecular Features
• t(X;17)(p11.2;q25) – ASPL1-TFE3 fusion.
Differential Diagnosis
• Epithelioid smooth muscle tumor
– Caldesmon, ER, and PR positive, and TFE3
negative.
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Miscellaneous Tumors,
Pathology of the Uterine
Corpus, Fig. 2 (a and b)
Alveolar nests separated by
delicate fibrovascular septa
and composed of uniform
population of large
polygonal cells with
prominent, centrally located
nucleus and abundant
eosinophilic, granular
cytoplasm. TFE3 nuclear
expression (seen in inset)
• Clear cell adenocarcinoma
– Several mixture of architectural patterns.
– CK7, PAX8, and Napsin A positive.
• Perivascular epithelioid cell tumor
(PEComa)
– May have spindled component.
– Melan-A, HMB45 positive, and TFE3
negative.
References and Further Reading
Folpe, A., et al. (2006). Alveolar soft-part sarcoma:
A review and update. Journal of Clinical Pathology,
59, 1127–1132.
Gomez,M.,etal.(2020).Alveolarsoftpartsarcoma
presenting as a uterine polyp: A case report.
SAGE Open Medical Case Reports, 8, 2050313X
2091059.
Hasegawa, K., et al. (2011). A case of primary alveolar soft
part sarcoma of the uterine cervix and a review of the
literature. International Journal of Clinical Oncology,
16, 751–758.
Sangoi, A., et al. (2009). Adenomatoid tumors of the
female and male genital tracts: A clinicopathological
and immunohistochemical study of 44 cases. Modern
Pathology, 22, 1228–1235.
Schwartz, E., et al. (2004). Adenomatoid tumors of the
female and male genital tracts express WT1. Interna-
tional Journal of Gynecological Pathology, 23,
123–128.
Wachter, D., et al. (2011). Adenomatoid tumors of
the female and male genital tract. A comparative
clinicopathologic and im munohistochemical analysis of 47 cases emphasizing their s ite-s peci fic
morphologic diversity. Virchows Archiv, 458,
593–602.
Mixed Epithelial and Mesenchymal Tumors, Pathology of the cervix 297
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References and Further Reading
Miscellaneous Tumors,
Pathology of the Vagina
Saimah Arif
Princess Alexandra Hospital NHS Trust, Harlow,
Essex, UK
Germ Cell Tumors of the Vagina (Yolk
Sac Tumor (YST) and Mature Teratoma)
Clement, P. B., Young, R. H., & Scully, R. E. (1988). Extra-
ovarian pelvic yolk sac tumors. Cancer, 62(3), 620–626.
Yuan, Z., Cao, D., Yang, J., Keng, S., & Huang, H. (2017).
Vaginal yolk sac tumors: Our experiences and results.
International Journal of Gynecological Cancer, 27(7),
1489–1493.
Mixed Epithelial and
Mesenchymal Tumors,
Definition
These are tumors of primitive and/or mature germ
cell elements.
Clinical Features
These are very rare tumors. Vaginal YST account s
for >90% of extragonadal yolk sac tumors and
presents with vaginal bleeding, a polypoid mass
which may prolapse through the introitus and
elevated serum alpha-fetoprotein (AFP). Mature
teratoma presents with a mass lesion.
• Age
Vaginal YST occurs in children <4 years.
Mature teratoma arises in patients aged 15–30.
• Site
Vagina and paravaginal space.
• Treatment and Outcome
Vaginal YSTs are malignant, but for most
patients, combination chemotherapy +/ surgical resection is curative (Yuan et al. 2017).
Vaginal mature teratomas are benign.
• Macroscopy
Vag i n al YS Ts me as ur e 1 –5 cm and have a soft,
friable gray-white cut surface with necrosis and
hemorrhage. Mature teratomas aretypically cystic.
• Microscopy and Immunophenotype
The microscopic appearances and immunophenotype are identical to those of their ovarian counterparts.
• Differential Diagnosis
The differential diagnosis of YST includes
clear cell adenocarcinoma. YST occurs at a
younger age and is positive for AFP and
alpha-1-antitrypsin (Clement et al. 1988).
Pathology of the cervix
Nissreen Mohammad
University of British Columbia, Vancouver, BC,
Canada
Adenomyoma of the Uterine Cervix
Definition
Benign mixed epithelial and mesenchymal tumor
composed of benign endocervical glands and
smooth muscle stroma.
Clinical Features
• Incidence
Rare and possibly underdiagnosed.
• Age
Range from 21 to 55 years. Mean age is
40 years (Gilks et al. 1996; Cas ey and
McCluggage 2015).
• Sex
Female.
• Site
Cervix.
• Treatment
Conservative management, polypectomy or
hysterectomy.
• Outcome
Benign.
Macroscopy
This is usually a well-circumscribed tumor which
can be intramural or polypoid. There is a wide size
range (1–23 cm). Cut sections are tan-gray,
M
298 Mixed Epithelial and Mesenchymal Tumors, Pathology of the cervix
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trabeculated, and contain cystically dilated
glands.
Microscopy
This is a biphasic tumor composed of lobules of
variably sized, cystically dilated benign endocervical mucinous glands, but can also show
tubal, endometrioid, or mesonephric glands. The
glands might show papillary infoldings or gland
rupture. The stromal component is comprised of
fascicles of benign smooth muscle, which might
occasionally how symplastic-like features (Fig. 1).
Immunophenotype
ER is positive in the endocervical epithelium and
smooth muscle. Mucin stains (PAS and Alcian
Blue) highlight intracytoplasmic mucin. SMA is
positive in the smooth muscle stroma. M-GGMC1, which recognizes gastric-type mucin, is negative (Mikami et al. 2001).
Molecular Features
None.
Differential Diagnosis
The differential diagnosis includes minimal deviation cervical adenocarcinoma (adenoma
malignum). The latter forms infiltrative glands
and does not form a well-circumscribed mass
grossly. Glands lack lobular architecture and are
MUC6, HIK1083, and M-GGMC-1 positive.
Adenosarcoma
Definition
Mixed epithelial and mesenchymal tumor comprised of a benign epithelial component and
malignant stromal component.
Clinical Features
• Incidence
Rare. Comprises 0.16% of cervical cancers
(Seagle et al. 2016).
• Age
Wide age range, from 13 to 67 years and a
mean age of 37 years (Jones and Lefkowitz
1995).
• Sex
Female.
• Site
Cervix. More commonly involves the uterine
corpus.
• Treatment
Hysterectomy.
• Outcome
There is a favorable outcome for completely
resected cervix confined tumors (Seagle et al.
2016). Poor prognostic factors include
lymphovascular invasion, stromal overgrowth,
deep invasion, and high-grade sarcomatous
component (Jones and Lefkowitz 1995; Yuan
et al. 2019; Hodgson et al. 2017).
Macroscopy
The size generally ranges from 1.5 to 5 cm (Seagle
et al. 2016; Jones and Lefkowitz 1995). They are
usually polypoid or papillary tumors with a soft
tan cut surface.
Mixed Epithelial and Mesenchymal Tumors, Pathology of the cervix, Fig. 1 Adenomyoma of the cervix:
Bland mucinous glands are surrounded by fascicles of
benign smooth muscle
Microscopy
The tumor shows a classic leaf-like/phyllodes-like
architecture. The epithelial component is benign.
The endocervical or e ndometrioid lining and may
show squamous metaplasia in rare cases. The
glands are cystic and rigid with periglandular
stromal condensation. The stroma shows cytologic atypia and increased mitotic activity
(2 10HPFs). Rhabdomyosarcomatous differentiation or sex-cord differentiation maybe seen
(Mohammadizadeh et al. 2016). The stroma may
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