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Mesothelial Tumors, Pathology of the Peritoneum 279
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Miscellaneous Tumors,
Pathology of the Broad
Ligament and Other Uterine
Ligaments
• Site
Broad ligament.
• Clinical Presentation
These tumors may present with abdominal
pain; some areas are incidental findings. Most
patients have von Hippel-Lindau (VHL) disease. Tumors may be unilateral or bilateral
(Zanotelli et al. 2010).
• Treatment
The importan ce of diagnosing this tumor is
because of the association with VHL disease
(and, hence, various tumors of other organs,
including renal cell carcinoma).
• Outcome
All lesions reported so far have been benign.
• Macroscopy
Papillary cystadenoma are up to 4 cm in diameter
and cystic with polypoid papillary protrusions.
• Microscopy
The papillae are generally short and blunted.
The stroma of the papillae varies in cellularity
and may be hyalinized or fibrous. The papillae
are usually lined by a single layer of
low-cuboidal (Fig. 1), non-ciliated cells that
have eosinophilic or clear cytoplasm. Atypia
and necrosis are absent, and mitotic figures are
rare to absent (Fig. 2).
Isabel Alvarado-Cabrero
Department of Pathology, Mexican Oncology
Hospital, Mexico City, Mexico
Papillary Cystadenoma NOS
Definition
A benign tumor of mesonephric origin that occurs
in women with von Hippel-Lindau disease
(Nogales et al. 2012).
Clinical Features
• Incidence
This tumor is rare.
• Age
Reported in women 20–64 years of age (Brady
et al. 2012).
• Sex
Female.
Miscellaneous Tumors, Pathology of the Broad Liga-
ment and Other Uterine Ligaments, Fig. 1 Papillary
cystadenoma NOS. Multiple branching papillae lined by
cells with clear cytoplasm
Miscellaneous Tumors, Pathology of the Broad Ligament and Other Uterine Ligaments 281
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Clinical Features
• Incidence
These tumors are rare; approximately 50 cases
have been reported (Ramirez et al. 2002).
• Age
The patients ranged in age from 15 to 81 years
(Daya. 1994).
• Site
Female adnexal tumor of probable wolffian origin (FATWO) occurs mainly within the leaves
of the broad ligament but may appear as pedunculated lesion arising from it. FATWO may also
be seen in the ovary and the retroperitoneum
Miscellaneous Tumors, Pathology of the Broad Ligament and Other Uterine Ligaments, Fig. 2 Papillary
cystadenoma NOS. Papillae are lined by cuboidal to
columnar, epithelium with bland nuclei and prominent
clear cytoplasm
(Kariminejad and Scully 1973).
• Clinical Presentation
Symptoms at presentation include abdominal
pain or palpable mass, and in some cases, the
tumor may be incidentally discovered (Tiltman
Immunophenotype
The cells are positive for CK7, PAX8, PAX2, and
CD10. WT1 and calretinin are variable positive.
Estrogen receptor and progesterone receptor are
negative (Cox et al. 2014; Brady et al. 2012).
and Allard 2001).
• Treatment
Most wolffian tumors are benign and
adequately treated by unilateral salpingooophorectomy . Rare FATWOs exhibit a malignant behavior, and these tumors have been
Molecular Features
Demonstration of germline VHL mutation may be
helpful (Shen et al. 2000).
treated with multiple chemotherapy regimens
(Sheyn et al. 2000).
• Outcome
Most wolffian tumors behave in benign fashion;
Differential Diagnosis
The differential diagnosis includes cystadenofibromas of mullerian type with prominent papil-
however, some cases either recur or metastasize.
Recurrences and metastases to thelung and liver
have been reported (Sheyn et al. 2000).
lary architecture that are not associated with VHL
disease. Those generally contain papillae which are
much larger and less complex and contain cilia
(Nogales et al. 2012).
Macroscopy
This tumor is typically a well-circumscribed, solid
mass with a bosselated external surface. The cut
surface is pale-yellow, solid in appearance, and
rubbery to palpation but may contain small cysts
Female Adnexal Tumor of Probable
(Kariminejad and Scully. 1973; Daya 1994).
Wolffian Origin
Microscopy
Synonyms
Wolffian adnexal tumor; Wolffian tumor.
Definition
This is a rare tumor, which is believed to originate
from mesonephric (wolffian) remnants on the
basis of their shared location in the broad ligament
(Kariminejad and Scully 1973).
The tumor shows a variable admixture of diffuse,
solid, tubular, and sieve-like cystic areas, with the
solid pattern dominating in the majority of cases.
The tubules are closely apposed but do not have a
true luminal border whereas the solid component
has a vaguely spindled appearance (Fig. 3). Most
cases do not show atypia or mitotic figures (Fig. 4)
(Kariminejad and Scully 1973; Daya 1994).
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282 Miscellaneous Tumors, Pathology of the Broad Ligament and Other Uterine Ligaments
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diagnosis, as it may closely resemble a FATWO
(Ramirez et al. 2002).
Ependymoma of the Broad Ligament
Definition
A rare, primary, neuroepithelial tumor occurring
in the broad ligament and outside of the central
nervous system (extramedullary).
Clinical Features
Miscellaneous Tumors, Pathology of the Broad Ligament and Other Uterine Ligaments, Fig. 3 Female
adnexal tumor of probably wolffian origin. Pseudotubules
(slit-like spaces) and solid areas are intimately admixed
Miscellaneous Tumors, Pathology of the Broad Ligament and Other Uterine Ligaments, Fig. 4 Female
adnexal tumor of probable wolffian origin. High-power
view, the tumor cells have scant pale eosinophilic cytoplasm and round to slightly elongated nuclei
Immunophenotype
The tumor cells are positive for calretinin,
cytokeratin, and vimentin. Epithelial membrane
antigen and carcinoembryonic antigen (CEA) are
negative (Tiltman and Allard 2001).
Molecular Features
Not clinically relevant.
• Incidence
Ependymoma of the broad ligament is
extremely rare (Zhou et al. 2015).
• Age
Patients range in age from 19 to 67 (average
32) years (Idow et al. 2008).
• Site
Broad ligament.
• Clinical Presentation
Patients present lower abdominal and pelvic
pain; however, some cases are discovered on
routine gynecologic examination (Bell et al.
1984).
• Treatment
Local excision is curative.
• Outcome
Information regarding outcome is limited;
however, in at least three patients, the tumor
had spread beyond the broad ligament (Bell
et al. 1984).
• Macroscopy
Theyvariedfrom1cmindiametertoalarge
mass filling the pelvis and extending to the umbi-
licus. Ependymomas may be solid, multicystic,
or multilobulated (Matsuyama, et al. 2010).
• Microscopy
These tumors exhibit the same microscopic fea-
tures of central nervous system ependymomas
(Fig. 5). They are composed of papillae, closely
packed tubules, and solid growth. True peri-
vascular rosettes (Fig. 6) and psammoma bodies
may be present (Matsuyama et al. 2010).
Differential Diagnosis
Endometrioid adenocarcinoma of the fallopian
tube is the main consideration in the differential
Immunophenotype
Tumor cells show immunopositivity for glial
acidic fibrillary protein (GFAP), as well as
Miscellaneous Tumors, Pathology of the Broad Ligament and Other Uterine Ligaments 283
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References and Further Reading
Bell, D. A., Woodruff, J. M., & Scully, R. E. (1984).
Ependymoma of the broad ligament. A report of two
cases. The American Journal of Surgical Pathology, 8,
203–209.
Brady, A., Nayar, A., Cross, P., et al. (2012). A detailed
immunohistochemical analysis of 2 cases of papillary
cystadenoma of the broad ligament: An extremely rare
neoplasm characteristic of patients with von Hippel
Lindau disease. International Journal of Gynecologi-
cal Pathology, 31, 133–140.
Cox, R., Vang, R., & Epstein, J. I. (2014). Papillary
cystadenoma of the epididymis and broad ligament:
Morphologic and immunohistochemical overlap with
Miscellaneous Tumors, Pathology of the Broad Ligament and Other Uterine Ligaments,
Fig. 5 Ependymoma of the broad ligament. Low-power
view, perivascular pseudorosettes and ependymal rosettes
Miscellaneous Tumors, Pathology of the Broad Ligament and Other Uterine Ligaments,
Fig. 6 Ependymoma of the broad ligament with neural
rosette formation
cytokeratin, vimentin, WT1, and CD99 (Zhou
et al. 2015).
Molecular Features
Not clinically relevant.
Differential Diagnosis
These tumors are easily confused with papillary
serous carcinoma. Being aware of its occurrence
in the adnexal structures should raise the question
of a neuroepithelial neoplasm. However,
ependymoma differentiation is evidenced by the
presence of perivascular rosettes and positive
reaction for GFAP (Idow et al. 2008).
clear cell papillary renal cell carcinoma. The American
Journal of Surgical Pathology, 38, 713–718.
Daya, D. (1994). Malignant female adnexal tumor of prob-
able wolffian origin with review of the literature. Arch
Pathol Lab Med, 118, 310–312.
Idow, M. O., Rosenblum, M. K., Wei, X. J., et al. (2008).
Ependymomas of the central nervous system and adult
extra-axial ependymomas are morphologically and
immunohistochemically distinct – A comparative
study with assessment of ovarian carcinomas for
expression of glial fibrillary acidic protein. The Amer-
ican Journal of Surgical Pathology, 32, 710–718.
Kariminejad, M. H., & Scully, R. E. (1973). Female
adnexal tumor of probable wolffian origin.
A distinctive pathologic entity. Cancer, 31, 671–677.
Matsuyama, A., Hisaoka, M., Yamamoto, I., et al. (2010).
Extraspinal ependymoma of the broad ligament.
Pathology International, 60, 241–244.
Nogales, F. F., Goyenaga, P., Preda, O., et al. (2012). An
analysis of five clear cell papillary cystadenomas of
mesosalpinx and broad ligament: Four associated with
von Hippel-Lindau disease and one aggressive sporadic
type. Histopathology, 60, 748–757.
Ramirez, P. T., Wolf, J. K., Malpica, A., et al. (2002).
Wolffian duct tumors: Case reports and review of the
literature. Gynecologic Oncology, 86, 225–230.
Shen, T., Zhuang, Z., Gersell, D. J., & Tavassoli, F. A. (2000).
Allelic deletion of VHL gene detected in papillary tumors
of the broad ligament, epididymis, and retroperitoneum in
von Hippel-Lindau disease patients. International Jour-
nal of Surgical Patholog y, 8, 207–212.
Sheyn, I., Mira, J. L., Bejarano, P. A., et al. (2000). Meta-
static female adnexal tumor of probable wolffian origin.
A case report and review of the literature. Archives of
Pathology & Laboratory Medicine, 124, 431–434.
Tiltman, A. J., & Allard, U. (2001). Female adnexal tumors of
probable wolffian origin: An immunohistochemical study
comparing tumors, mesonephric remnants and para-
mesonephric derivatives. Histopathology, 38, 237–242.
Zanotelli, D. B., Bruder, E., Wight, E., & Troeger,
C. (2010). Bilateral papillary cystadenoma of the meso-
salpinx: A rare manifestation of von Hippel-Lindau
disease. Archives of Gynecology and Obstetrics, 282,
343–346.
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284 Miscellaneous Tumors, Pathology of the Ovary
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Zhou, F., Song, J., Mikolaenko, I., et al. (2015). Pelvic
ependymoma with clinical response to GnRH analog
therapy: A case report with an overview of primary
extraneural ependymomas. International Journal of
Gynecological Pathology, 34, 450–458.
Miscellaneous Tumors,
Pathology of the Ovary
Rouba Ali-Fehmi, Andrew Kumar, Nour Abd
Almohsen and Mir Yousufuddin Ali Khan
Wayne State University, Detroit, MI, USA
Tumors of the Rete Ovarii
Synonyms
These masses may be called adenoma of rete
ovarii, rete ovarii cystadenoma (transformation
to adenocarcinoma of rete ovarii is an extremely
rare manifestation).
Definition
Tumors arising from the rete ovarii, a vestigial
structure present in the ovarian hilus that is histologically identical to its testicular homologue.
• Treatment
Surgical excision of the tumor showed
progression-free survival and overall survival,
while surgery and oncologic treatment is indi-
cated for adenocarcinomas.
• Outcomes
When benign, the prognosis is good; however,
adenocarcinomas have a more aggressive
behavior (Rutgers and Scully 1988).
Macroscopy
Usually unilateral and unilocular with a smooth
lining and filled with serous fluid, these tumors
range from 1 to 24 cm with a mean size of 8.7 cm.
Rete ovarii adenomas are not grossly evident but
may rarely be seen as a solid or mixed solid-cystic
mass. Adenocarcinomas are large tumors presenting with solid and cystic areas on cut surface.
Microscopy
Adenomas of the rete ovarii are lined by bland,
mitotically inactive, flat, cuboidal, or columnar
cells. There is scant eosinophilic cytoplasm with
rare cilia and focal transitional cell metaplasia.
These lesions tend to be circumscribed and composed of tightly packed tubules, which may
become cystic and contain simple papillae. The
tubules are similarly lined by bland, mitotically
inactive cuboidal or columnar cells.
Clinical Features
• Incidence
They represent less than 1% of ovarian tumors
(Rutgers and Scully 1988). Adenocarcinomas
are extremely rare.
• Age
Most common in postmenopausal patients.
• Clinical presentation
Patients present with abdominal discomfort
and pressure, virilization, hirsutism, and pelvic
mass. Adenomas tend to be incidental findings.
The only case of rete ovarii adenocarcinoma
presented with abdominal swelling and ascites
in a 52-year-old woman.
• Sex
Female.
• Site
Ovary.
Immunophenotype
These adenomas and adenocarcinomas are positive for CAM 5.2, cytokeratin 19, CA125, CD10,
and may occasionally stain for epithelial membrane antigen as well as estrogen and progesterone receptors.
Differential Diagnosis
The major differential diagnosis to compare the
tumors of the rete ovarii against are serous
cystadenomas and serous adenocarcinomas,
which usually contain one (but may contain
more) smooth glistening, thin-walled cyst filled
with clear, watery serous fluid. In general, the
histopathology of the serous neoplasms mimic
the epithelium of the fallopian tubes, in that the
cysts are lined by a single layer of cuboidal to low
columnar ciliated cells without nuclear atypia;
Miscellaneous Tumors, Pathology of the Ovary 285
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however, they may also be lined with non-ciliated
cuboidal to columnar secretory cells. Psammoma
bodies may be present in serous cystadenomas as
well as serous adenocarcinomas (Gattuso et al.
2015).
Wolffian Tumor
Synonyms
Female adnexal tumor of probable Wolffian origin.
Definition
A tumor of Wolffian origin arising in the ovary or
adjacent to it and expanding into it.
Clinical Features
• Incidence
This is an uncommon ovarian neoplasm
(Young and Scully 1983).
• Age
Patients range in age from 24 to 87 years old.
Most are postmenopausal.
• Clinical presentation
Patients may present with abdominal enlargement, abdominal pain, abdominal mass, postmenopausal vaginal bleeding, and urinary
frequency (Young and Scully 1983).
• Sex
Female.
• Site
Ovary.
• Treatment
The most common treatment is hysterectomy
and bilateral salpingo-oophorectomy as well as
tumor debulking. The role of adjuvant chemotherapy or radiation therapy is unknown. Tyrosine kinase inhibitor Gleevec (STI571) is a
treatment option in cases of recurrent or metastatic c-kit (CD117) expressing tumors (Steed
et al. 2004).
• Outcomes
This entity is usually benign and confined to
the ovary. There have been rare cases in which
the tumor presented with advanced stage disease or behaved in a malignant fashion after
resection. Malignant behavior is associated
with cytologic atypia and increased mitoti c
activity. Occasionally, tumors with minimal
nuclear atypia and very low mitotic rates have
recurred (Deen et al. 2007).
Macroscopy
Tumors are unilateral, solid, or a mixture of solid
and cystic. They range from gray to white, tan, or
yellow and may range in size from 2 to 20 cm.
Microscopy
This lesion consists of combinations of the following patterns: cysts of variable sizes forming a
sieve-like pattern; closely packed pattern; or
retiform, tubular, and solid foci in which the
cells are spindled. The tumor cells are cuboidal
or columnar; however, the cells lining the cyst
may be flattened. Hobnail cells may be seen. The
cells are typically bland with a low mitotic rate.
Immunophenotype
Tumor cells stain positive for a broad spectrum of
cytokeratin and vimentin, and often for calretinin.
There are variable expressions of cytokeratin 7,
estrogen and progesterone receptors, smooth muscle actin (SMA), CD10, androgen receptor,
inhibin, c-Kit, and EMA. The tumor cells are typically negative for monoclonal CEA (Fanghong
et al. 2008; Devouassoux-Shisheboran et al. 1999).
Molecular Features
No disease defining molecular alterations are
identified.
Differential Diagnosis
The main differential diagnoses are endometrioid
carcinoma, which usually has a glandular pattern
with variable squamous differentiation and
nuclear atypia; high-grade serous carcinoma,
which has papillary and micropapillary architecture with high-grade nuclear features and a high
mitotic activity, as well as diffuse positive or
completely negative p53 expression; and clear
cell carcinoma, which has high-grade nuclear features including abundant, clear cytoplasm and
clear cell mark ers, such as HNF1 beta and Napsin
A positivity. All three of these entities are positive
for PAX8, while Wolffian tumor is negative
(Lucas and Zheng 2018). Another rare differential
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diagnosis is Mullerian mesenchymal tumor with
endometrial stromal and smooth muscle differentiation. This neoplasm shows both smooth muscle
and endometrial stromal differential intermixed
together. The tumor cells express smooth muscle
actin, CD10, desmin, estrogen and progesterone
receptor.
Small Cell Carcinoma,
Hypercalcemic Type
Definition
An undifferentiated neoplasm predominantly
composed of small cells. This may occasionally
have a large cell component and may be associated with paraneoplastic hypercalcemia. The
tumor is not related to small cell carcinoma of
neuroendocrine (pulmonary) type.
Clinical Features
• Incidence
This is a rare tumor.
• Age
This typically occurs in young women within
the second and third decades, with a mean age
of 23 years old.
• Clinical presentation
This is associated with paraneoplastic hypercalcemia in 66% of cases (Dickersin et al.
1982; Young et al. 1990). Most patients present
with symptoms consistent with an ovarian
mass. Occasionally, the tumor presents with
metastatic disease. Symptoms specifically
attributable to hypercalcemia are rare. Onehalf of cases have unilateral ovarian involvement with extra-ovarian spread in the form of
peritoneal disease.
• Sex
Female.
• Site
Ovary.
• Treatment
Bilateral salpingo-oophorectomy with surgical
debridement, followed by chemotherapy with/
without radiation therapy.
• Outcomes
This is a highly aggressive neoplasm. The
tumor stage is the most important prognostic
factor. One-third of the patients with stage IA
disease were disease free 1–13 years after sur-
gery, while the other two-thirds died or had
recurrence of the primary disease (Young
et al. 1990). Almost all patients with any
stage higher than II died of disease. More
favorable outcomes are associated with
age > 30, a normal preoperative calcium
level, tumor size <10 cm, and no large cell
component. Recurrence is monitored by
serum calcium levels.
Macroscopy
The tumors are relatively large, mainly consisting
of solid components. The masses are pale and
range from white to gray. There may be necrosis,
hemorrhaging, and cystic degeneration.
Microscopy
A diffuse growth pattern is usually present, but
most cases have focal follicle-like spaces with
luminal eosinophilic (or rarely basophilic) fluid
(Dickersin et al. 1982; Young et al. 1990). Nested,
corded, or trabecular growth may be present as
well. The nuclei tend to be monotonous and
hyperchromatic with course clumped chromatin
and small nucleoli. There tends to be conspicuous
mitotic activity with areas of necrosis. If there is a
component of large cells, containing abundant
eosinophilic cytoplasm (and can be focal, predominant or exclusive) it would then be considered a large cell variant. There may be small foci
of mucin, and rarely, signet ring cells in 15% of
cases. There is usually minimal stroma (Figs. 1, 2,
and 3).
Immunophenotype
Most cases exhibit diffuse nuclear staining with
antibodies against N-terminal of WT1
(McCluggage et al. 2004). The tumor focally
stains positive with broad-spectrum cytokeratins,
epithelial membrane antigen (EMA), CD10,
calretinin, and neuroendocrine markers (Aguirre
et al. 1989; McCluggage et al. 2004). Parathyroid
hormone-related protein (PTHrP) may be positive
(Dickersin et al. 1982). The absence of
SMARCA4 and SMARCA2 is found in almost
all tumors.
Miscellaneous Tumors, Pathology of the Ovary 287
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Miscellaneous Tumors, Pathology of the Ovary,
Fig. 1 Small cell carcinoma, hypercalcemic type:
Section showing sheet of small round blue cells with overlapping nuclei
Miscellaneous Tumors, Pathology of the Ovary,
Fig. 2 Small cell carcinoma, hypercalcemic type
Section showing follicle-like space within the diffuse
sheet of cells
Molecular Features
Molecular studies via flow cytometry of paraffinembedded tissue have demonstrated that the neoplastic cells are diploid (E ichhorn et al. 1992a).
Somatic or germline mutations of SMARCA4 are
detected in most tumors (Moes-Sosnowska
et al. 2015).
Differential Diagnosis
Due to the young age and the presence of folliclelike spaces in the neoplasm, the most common
differential diagnosis include juvenile granulosa
cell tumor. Likewise, this tumor may also be confused with adult-type granulosa cell tumors,
Miscellaneous Tumors, Pathology of the Ovary,
Fig. 3 Small cell carcinoma, hypercalcemic type:
Section showing cell with scant cytoplasm, prominent
nucleoli, brisk mitotic activity, and focal area of overlapping nuclei
malignant lymphoma, and other small cell malignant neoplasm that involve the ovary (Dickersin
and Scully 1993).
Small Cell Carcinoma, Pulmonary
Type
Synonyms
Small cell carcinoma of neuroendocrine type.
Definition
A small cell carcinoma resembling pulmonary
small cell carcinoma of neuroendocrine type.
Clinical Features
• Incidence
Rare tumor.
• Age
Most patients are postmenopausal.
• Clinical presentation
Patients present with symptoms referable to a
pelvic or abdominal mass (Eichhorn et al.
1992b).
• Sex
Female.
• Site
Ovary.
• Treatment
Radical hysterectomy and bilateral salpingo-
oophorectomy with debulking if extra-ovarian
M
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extension with adjuvant chemotherapy with/
without radiation therapy.
• Outcomes
Due to the highly aggressive nature of these
tumors and the tendency to present at an
advanced stage, the prognosis is poor
(Eichhorn et al. 1992b).
Macroscopy
The tumors present as large and predominantly
solid, with frequent areas of necrosis. They are
often bilateral and commonly contain extraovarian spread.
Microscopy
The tumors predominantly grow in a diffuse pattern, but may also present with nests and rosettelike structures. Most cases contain a component
of surface epithelial-stromal tumor, most commonly endometrioid or mucinous type
(Fukunaga et al . 1997; Grandjean et al. 2007).
The cells of the tumors tend to be r ound with
slightly spindled hyperchromatic nuclei. They
may contain “salt-and-pepper” chromatin with
nuclear m olding. The nuclei usually demonstrate
abundant mitotic activity with apoptosis. The
cytoplasm is typically scant. Necrosis is common. They may rarely arise in ovarian teratoma
(Ikota et al. 2012).
Immunophenotype
The tumors are variably positive with neuroendocrine markers such as chromogranin, synaptophysin, CD56, and PGP9.5. The diagnosis
of small cell carcinoma of pulmonary type can
be made in the absence of neuroendocrine marker
positivity if the morphology is consistent. The
tumor may show nuclear staining with TTF-1 ;
however, this does not necessarily indicate a
metastasis from a pulmonary primary (Grandjean
et al. 2007).
Molecular Features
Immunohistochemical staining for neuronspecific enolase are typically positive, while a
minority of cases have chromogranin positivity
(Eichhorn et al. 1992b).
Differential Diagnosis
The most common differential diagnosis is small
cell carcinoma, hypercalcemic type that shows
large cells with abundant cytoplasm, central and
prominent nucleoli. It is associated with paraneoplastic hypercalcemia. Immunohistochemistry shows tumor cells to stain positive for
vimentin and WT1 with loss of SMARCA4.
Desmoplastic small round cell tumor (DSRCT)
may also pose a diagnostic challenge. However,
DSRT may show prominent desmoplastic stroma
and shows dual expression of epithelial markers
and characteristic paranuclear dot-like positivity
for desmin.
Wilms Tumor
Synonyms
Nephroblastoma.
Definition
A primary ovarian tumor with features similar to
those of the kidney tumor of the same name. There
are two types of extrarenal Wilms tumors (EWT):
(1) EWT as a component of a teratoma (teratoid
Wilms tumor) and (2) pure EWT.
Clinical Features
• Incidence
Extremely rare, less than five cases (Isaac et al.
2000; Oner et al. 2002; Pereira et al. 2000;
Sahin and Benda 1988).
• Age
Most commonly seen in the third to sixth
decades.
• Clinical presentation
Abdominal pain with or without distension,
pelvic or abdominal mass and ascites.
• Sex
Female.
• Site
Ovary.
• Treatment
No specific treatment guidelines currently exist
for EWT or teratoid EWT, and the guidelines
commonly reffered to traetment planning for
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