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118 Germ Cell Tumors, Pathology of the Ovary
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Germ Cell Tumors, Pathology of the Ovary,
Fig. 8 Choriocarcinoma at higher magnification
showing mixed mononucleated cytotrophoblasts and
multinucleated syncytiotrophoblasts
Molecular Features
Abnormalities in chromosome 12 are seen in 75%
of cases of YST. Most of these patients have
isochromosome 12P. In some cases, in elderly
women, yolk sac tumor may occur in association
with ovarian or endometrial carcinoma, in these
cases it is thought to be of somatic origin (Young
2014).
Embryonal Carcinoma
Definition
Embryonal c arcinoma is a primitive germ
cell tumor that shows embryonal differentiation.
The pure form is rare and mo st of the
time embryonal carcinoma presents as a component of a mixed ger m cell tumor (Figs. 9
and 10).
Clinical Features
The most common clinical presentation is pelvic
mass or abdominal pain. Embryonal carcinoma is
staged based on the Union for International Cancer Control (UICC) TNM classificati on of ovarian, fallopian tube, and primary peritoneal
carcinoma and the FIGO staging.
The presence of embryonal carcinoma in a
mixed germ cell tumor is thought to be of poor
prognosis. There are no major recent studies on
Germ Cell Tumors, Pathology of the Ovary,
Fig. 9 Mature teratoma showing a mixed squamous and
glandular epithelium, stroma, cartilage and muscle bundles
Germ Cell Tumors, Pathology of the Ovary,
Fig. 10 Mature teratoma showing a mixed squamous
and glandular epithelium, stroma, cartilage and muscle
bundles
pure ovarian embryonal carcinoma due to the fact
that this is exceedingly rare. One old study of
15 cases showed 39% survival, however this
study was before the era of immunohistochemistry and some of these cases maybe reclassified as
other forms of germ cell tumors with the current
application of immunohistochemistry (Kurman
and Norris 1976).
Macroscopy
Grossly, embryonal carcinoma usually forms a
soft pale tan friable tumor with foci of hemorrhage
and necrosis.
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Microscopy
Microscopically, embryonal carcinoma consists
of cohesive growth of tumor cells in multiple
patterns that often coexist with each other. The
most common patterns are the solid and the glandular pattern and to a lesser degree the papillary
growth pattern. Necrosis is common whether confluent solid areas of necrosis or individual cell
necrosis. Tumor cells are usually polygonal or
glandular with basophilic to amphophilic cytoplasm and indistinct cell borders. The nuclei are
large and irregular with dense clumped chromatin
and parachromatin clearing. In the papillary pattern, when the vessels are prominent and there is
prominent necrosis, a pseudo-Schiller-Duval bod-
Germ Cell Tumors, Pathology of the Ovary,
Fig. 11 Immature teratoma showing a mixture of mature
and immature elements
ies appearance may occur, however tumor cells
will be more pleomorphic than tumor cells in yolk
sac tumor. It is not uncommon to find syncytiotrophoblasts cells in embryonal carcinoma
(Figs. 11 and 12).
Immunophenotype
Immunohistochemically, embryonal carcinoma
cells are positive for EMA, CD30, OCT4,
SALL4, SOX2, and LIN28 while negative for
D2–40, AFP, beta-hCG, and GATA3.
G
Molecular Features
On the molecular level, the most common abnormalities are abnormalities involving chromosome
12 (whether isochromosome 12P or 12P amplification) (“Female Genital Tumours WHO Classification of Tumours, 5th Edition, volume 4”).
Nongestational Choriocarcinoma
Definition
Rare aggressive germ cell tumor that is composed
of trophoblastic cells that are not of gestational
origin. Only few cases of pure choriocarcinoma of
the ovary have been reported (less than 1% of
ovarian neoplasms).
Clinical Features
It usually occurs at younger age. Clinically,
patients may present with abdominal pain, pelvic
Germ Cell Tumors, Pathology of the Ovary,
Fig. 12 Immature teratoma showing a mixture of mature
and immature elements
mass, and vaginal bleeding. Serum beta-hCG is
elevated (“Female Genital Tumours WHO Classification of Tumours, 5th Edition, volume 4”).
Nongestational choriocarcinoma is staged
based on the Union for International Cancer Control (UICC) TNM classification of ovarian,
fallopian tube, and primary peritoneal carcinoma
and the FIGO staging (Figs. 13 and 14).
Nongestational choriocarcinoma has an
aggressive clinical course with a high tendency
for angiolymphatic spread and intraperitoneal
spread and it is less chemosensitive when compared with gestational choriocarcinoma (Stockton
et al. 2018).
120 Germ Cell Tumors, Pathology of the Ovary
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criterion to diagnose choriocarcinoma as it is not
unusual to identify scattered syncytiotrophoblasts
in other germ cell tumors. Syncytiotrophoblasts
usually have eosinophilic to amphophilic cytoplasm, and multiple nuclei with vesicular to hyperchromatic and sometimes smudgy chromatin.
Cytotrophoblasts usually have pale cytoplasm,
mild-to-moderate nuclear pleomorphism, and they
tend to have prominent nucleoli.
Immunoprofile
Immunohistochemically, syncytiotrophoblasts
and cytotrophoblasts are usually positive for
Germ Cell Tumors, Pathology of the Ovary,
Fig. 13 Gonadoblastoma
beta-hCG. However, to differentiate choriocarcinoma from scattered syncytiotrophoblasts in
dysgerminoma or embryonal carcinoma the best
panel would be GATA3, CD30, OCT3/4, D2–40,
and CD117. Cytotrophoblasts are positive for
GATA3 while negative for all other markers,
while embryonal carcinoma cells are positive for
OCT3/4 and CD30 while negative for GATA3 and
dysgerminoma cells are positive for OCT3/4,
D2–40, and CD117 while negative for GATA3.
Germ Cell Tumors, Pathology of the Ovary,
Fig. 14 Gonadoblastoma with adjacent dysgerminoma
Macroscopy
Grossly pure choriocarcinoma of the ovary shows
a large solid mass with marked areas of hemorrhage and necrosis (Young 2014).
Microscopy
Microscopically, choriocarcinomas are extremely
hemorrhagic and necrotic. Viable tumor areas are
usually seen at the periphery of the areas of hemorrhage and necrosis. Viable tumor areas usually
show a mixture of multinucleated syncytiotrophoblasts and mononucleated cytotrophoblasts.
The syncytiotrophoblasts usually surrounds foci of
cytotrophoblasts. Another common physical distribution of the syncytiotrophoblasts is to be stretched
around blood vessels. It is important to have a strict
Mixed GCT of the Ovary
Clinical Features
Malignant mix ed germ tumor of the ovary is
extremely rare (Goyal et al. 2019). They represent
10–20% of malignant germ cell tumors and are
usually present in children and young adults. In
the majority of cases the tumor is composed of a
mixture of dysgerminoma with yolk sac tumor,
immature teratoma, or choriocarcinoma (Goyal
et al. 2014).
The most common clinical presentations are
abdominal pain, pelvic mass, and menstrual
abnormalities. Serum markers are usually elevated depending on what germ cell components
are present.
Mixed GCTof the ovary is staged based on the
Union for International Cancer Control (UIC C)
TNM classification of ovaria n, fallopian tube,
and primary peritoneal carcinoma and the FIGO
staging.
The most important prognostic factor is stage.
Due to different sensitivity to chemotherapy and
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different behavior the type and percentage of germ
cell components present affects prognosis.
Macroscopy
Grossly the tumor is usually large with an average
size of 15 cm. The gross appearance usually
depends on what germ cell elements are present.
Microscopy
Microscopically, the different components are
usually intermixed, though you might see separate areas. The most seen combination is
dysgerminoma and yolk sac tumor. No minimal
size criterion is present for a germ cell element to
be considered as a component of a mixed germ
cell tumor.
Molecular Features
The most seen genetic abnormalities are changes
involving chromosome 12, mostly gain of 12P or
isochromosome 12P (“Female Genital Tumours
WHO Classification of Tumours, 5th Edition,
volume 4”).
Mature Teratoma
Definition
Mature teratoma is a germ cell tumor that has
mature elements originating from two or three
germ cell layers (ectoderm, mesoderm, and/or
endoderm).
Clinical Features
They represent 20% of all ovarian neoplasms in
some pathological studies. The pathogenesis of
mature teratoma is not entirely understood, however; based on the forwa rd explanation, mature
teratoma is part of the hemogenetic study which
suggests an origin from primordial germ cell
(Wang et al. 2011).
The majority of the cases are present in female
in their reproductive age. Clinically, tumors are
usually present as an abdominal mass or abdominal
pain, although in some cases may be detected
incidentally. In 10% of the cases mature teratomas
are bilateral. Rarely, young women may present
with anti-NMDAR encephalitis (Leel et al. 2018).
Mature teratomas are benign tumors with
excellent clinical course except for the rare cases
where they develop somatic malignant transformation (Biskup et al. 2006). In some instances,
usually after incomplete excision of mature teratoma, immature elements may develop in the
remaining ovarian tissue. Some women might
develop gliomatosis peritonei, however this
development is not associated with adverse outcome and these tumors should still be designated
as benign (Wang et al. 2016).
In general, mature teratomas with rare foci of
immature neural tissue are associated with an excellent prognosis and shouldnot be classified as imma-
ture teratomas (Schmidt and Kommoss 2007).
Macroscopy
Grossly, the tumor is usually cystic, hence the
name mature cystic teratoma, although in some
cases it might be entirely solid. Size usually
ranges between 5 and 10 cm, although mature
teratoma in women with anti-NMDAR tend to
be smaller in size (Leel et al. 2018). Upon opening
the cysts, we might see hair, teeth, or sebaceous
material. Along the cyst lining there is u sually a
solid nodule lined by hair-bearing skin
(Rokitansky protuberance). In solid teratomas
there are usually scattered small cysts. Solid
areas should be liberally sampled (ideally one
section per centimeter of solid areas).
Microscopy
Microscopically, there is usually a mixture of
ectodermal, endodermal, and mesodermal tissues.
Ectodermal tissue may include squamous epithelium and cutaneous adnexal structures, as well
as neuroectodermal tissues such as glia and cerebellum. Mesodermal tissue might include fat,
bone, cartilage, and smooth muscle. And endodermal tissue might include gastrointestinal
mucosa, respiratory mucosa, salivary glands, etc.
In rare instances we might find minute foci of
immature neural tissue. While it is not unusual to
find foci of fat necrosis or giant cell reaction.
Finally, in women with anti-NMDR encephalitis, there is usually neurological tissue which
tends to be surrounded by lymphoid aggregates
with germinal centers.
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Immature Teratoma of the Ovary
Definition
Immature teratoma is the second most common
malignant ovarian germ cell tumor in the USA.
Clinical Features
It tends to present in the first three decades of life
(Young 2014). The tumor tends to present as a
pelvic mass. Serum markers are usually negative,
however; immature teratoma with hepatoid components may have elevated AFP. Immature teratoma does not show gain of 12P or isochromosome
12P unless they are part of a MGCT.
Immature teratoma is staged according to the
Union of International Cancer Control (UICC)
TNM classification and FIGO staging system. One
pitfall in staging is the rare scenario of gliomatosis
peritonei. Gliomatosis peritonei is staged as stage
III, however it behaves as a benign germ cell tumor
and overtreatment should be avoided (Wang et al.
2016). For this reason, many clinicians and pathol-
ogists opt to upstage ov arian tumors only if implants
contain immature elements (grade 1 or higher).
Whichever approach is taken, it is important to
communicate the clinical picture to the surgeon.
Rarely, after chemotherapy, extraovarian
deposits of pure mature teratomatous tissue may
develop. This phenomenon is called growing teratoma syndrome (Kataria et al. 2017).
The major prognostic factors are grade and stage.
In case of metastasis, the grade of metastatic foci is a
main prognostic factor. The five-year overall survival is more tha n 90% (five-year survival for stage
I disease approaches 100%) (Gadducci et al. 2003 ).
Macroscopy
The tumor is usually unilateral, large, fleshy, tan,
and solid-cystic with hemorrhage and necrosis.
Microscopy
Microscopically, the tumor shows variable
amounts of immature elements, mostly
neuroectodermal tubules and rosettes. Usually
these elements are admixed with ectodermal and
endodermal tissue of varying maturation. The
immature elements consist of mitotically active
hyperchromatic cells and cellular mitotically
Germ Cell Tumors, Pathology of the Ovary,
Table 1 Grading of Immature Teratoma
Number of
fields
1 Grade 1 Low grade
>1to3 Grade 2 High grade
>3 Grade 3 High grade
Grade 3–tiered systems
1983
Grade 2–tiered
system
2008
active glia. Immature mesodermal and to a lesser
degree immature endodermal element may be
present. Other types of proliferation such as vascular proliferations may be seen.
Primary and metastatic foci are graded using
the same criteria. Grading depends on the number of low -power microscopic fields showing
aggregates of neuroepithelium in any individual
slides. The old grade system of 1983 divides
immature teratoma into three grades while the
recent grading system divides immature teratoma into low grade and high grade (Norris
et al. 1 976;O’Connor and Norris 1994; Young
2014). Pure gliomatosis peritonei is considered
mature (grade 0) (Wang et al. 2016)(Table1).
Immunophenotype
Rarely, immunostains may be helpful in making
the diagnosis of immature teratoma. Immature
neuroectodermal and intestinal elements tend to
be positive for SALL4. SOX2 can also be positive
in neuroectodermal elements.
Struma Ovarii
Definition
A type of mature teratoma consists predominantly
or entirely of thyroid tissue.
Clinical Features
Struma ovarii is usually and incidental finding,
although it can sometimes present as a pelvic
mass (Young 2014). It usually occurs in middleage women with a peak incidence in the fifth
decade (Smith 1946). One-third of patients may
develop ascites which can be associated with
pleural effusion and hyperthyroidism maybe
seen in less than 10% of patients (Young 2014).
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The majority of patients with struma ovarii
have benign clinical course. Some of the features
that have been reported to be associated with a
protracted clinical course include adhesions, surface defects, large size, more than 1 L of ascites,
and secondary malignancies (Young 2014). The
presence of peritoneal implants of welldifferentiated thyroid-like tissue is thought to be
a metastasis from well-differentiated follicular
carcinoma arising in struma ovarii (Roth and
Karseladze 2008).
Macroscopy
Tumor is usually unilateral, solid beefy with red to
brown cut surfaces, similar to the thyroid gland.
Although it might reach large sizes, struma ovarii
is usually less than 10 cm. In rare cases, struma
ovarii maybe cystic.
Microscopy
Microscopically, it resembles normal thyroid tissue, with follicles in different size and shape lined
by cuboidal to flat cells and filled with colloid.
When the tumor is cystic, it may resemble a serous
cystadenoma; however, the wall of the cysts and
the solid area consisting of thyroid-like follicles
are seen. Hyperplastic and adenomatous changes
may develop. In rare patients papillary thyroid
carcinoma and follicular thyroid carcinoma may
develop in struma ovarii. These patients may
show molecular changes similar to the changes
seen in papillary thyroid carcinoma and follicular
thyroid carcinoma of the thyroid. Struma ovarii is
sometimes seen alongside other teratoma components, Brenner tumor and mucinous cystadenoma.
Immunophenotype
Tumor cells have similar immunoprofile to thyroid follicular cells as they are positive for PAX8,
TTF1, and thyroglobulin.
Clinical Features
It usually presents in older women (Young 2014).
The most common presentation is of a pelvic
mass. In rare scenarios ovarian carcinoid maybe
seen as part of MEN type I syndrome. Ovarian
carcinoid is considered as a monodermal teratoma
that arises from the NE cells of the intestinal type
mucosa of mature cystic teratoma.
Prognosis of ovarian carcinoid is generally
excellent. Adenocarcinoma may rarely arise in
mucinous carcinoid and may affect the overall
prognosis (Zhai et al. 2020).
Macroscopy
Macroscopically, ovarian carcinoids are unilateral, small (average size: 3.4 cm), solid, and
homogeneous with tan-yellow cut surface. When
the patient has symptoms of carcinoid syndrome,
carcinoid tumor tends to be of larger size.
Microscopy
The most common morphologic type is insular
carcinoid (around 50%) that consists of small
nests and acini separated by fibrous stroma.
Tumor cells usually have abundant eosinophilic
cytoplasm and round nuclei with salt-and-pepper
chromatin. The second most common morphologic type of ovarian carcinoid is strumal carcinoid (around 40%) which is composed of insular
or trabecular carcinoid admixed or sitting next to
struma. Other forms of carcinoid tumor including
trabecular and mucinous carcinoid are rare.
Immunophenotype
Immunohistochemically, carcinoid tumor is positive for neuroendocrine markers including synaptophysin, chromogranin, neuron-specific
enolase (NSE), and CD56. Carcinoid tumor is
also positive for cytokeratins including
AE1/AE3 pankeratin and CK7.
G
Ovarian Carcinoid
Definition
Ovarian carcinoid is a rare well-differentiated
neuroendocrine tumor that resem bles carcinoid
tumor elsewhere in the body.
Neuroectodermal-Type Tumors
Definition
Malignant tumors that usually present as a pelvic
mass in young women with a median age at presentation of 24 years old (Liang et al. 2016).
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Clinical Features
Prognosis of these tumors is highly related to
clinical stage. And the more differentiated tumor
tends to have an overall better prognosis.
Macroscopy
These tumors are usually large and solid, though
sometimes it may show cystic areas of growth.
The cut surfaces are usually white to tan and
shows prominent areas of hemorrhage and necrosis. Teratomatous elements maybe present in the
adjacency.
Microscopy
Microscopically, these tumors show proliferation
of small round blue cells with different degrees of
differentiation.
Immunophenotype
Immunohistochemically, these tumors tend to be
positive for CD99, FLI-1, and they are often positive for CD56, NSE, and synaptophysin. Pan
cytokeratin maybe positive in some cases. Desmin
is negative which helps in differentiating these
tumors from rhabdomyosarcomas.
Monodermal Cystic Teratoma
Definition
Benign germ cell tumor that is usually seen in
children and young women.
Clinical Features
It usually presents with pelvic mass and abdominal pain. The tumor is usually cystic, and it has
elements that belong to one germ layer, either
ectoderm or endoderm. Struma ovarii, carcinoid,
and neuroectodermal-type tumors are considered
separate monodermal teratomas.
Macroscopy
Macroscopically, the tumor consists of thinwalled cysts that are filled with material
depending of the type of origin. Epidermal
cysts are usually filled with keratinaceous
white-grey material while neuroectodermal
cysts are filled with clear fluid. These tumors
have a wide size range as some of them can be
as big as 15 cm.
Microscopy
Microscopically, the tumor shows cystic appearance, and these cysts lining differ depending on
the germ layer of origin. Epidermoid cysts are
similar to their counterpart elsewhere in the
body, they are usually lined by mature
keratinizing squamous epithelium and are filled
with keratinaceous debris. Neuroectodermal cysts
tend to be lined by ependymal cells. Next to the
cyst lining we might identify other cells of neural
origin such as astrocytes and oligodendrocytes
(Fogt et al. 1994; Tiltman 1985). Pituitary and
corticotrope adenomas have been described in
association with monodermal cystic teratoma
and they have similar microscopic appearance to
prolactinomas and corticotrope adenomas of the
pituitary gland (Axiotis et al. 1987).
Somatic Neoplasms Arising in Teratomas
Definition
Somatic tumors that can arise from any of the
elements form the teratoma. They usually occur
at older age with an average age of 55 (Young
2014).
Clinical Features
The most important prognostic feature is the
stage, with tumors limited to the ovaries having
good prognosis. The early detection of somatic
malignant transformation is essential to long-term
survival and for that thorough sampling of larger
ovarian teratomas with suspicious areas is critical
(Chiang et al. 2017).
Macroscopy
These tumors are usually larger in size than
mature cystic teratoma and grossly show areas
that are suspicious for malignant transformation
(Young 2014).
Microscopy
The most common somatic malignancy to arise in
teratomas is squamous cell carcinoma (around
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80% of all somatic malignancies arising in a teratoma) followed by adenocarcinomas (Young
2014). Sarcomas represent around 8% of somatic
malignancies arising in teratomas. It is not
unusual to see extraovarian extension in somatic
malignancies arising in teratomas.
Microscopically, these tumors show overgrowth of teratoma by a pure somatic malignant
element that occupies a single, low-power
(4x objective) microscopic field. The somatic
malignant element usually resembles the
appearance of this tumor elsewhere in the
body. So, a squamous cell carcinoma arising in
a teratoma will look like a squamous cell carcinoma anywhere and it will fill a single lowpower (4x objective) microscopic field. Same
thing applies to adenocarcinoma or rhabdomyosarcoma, etc. arising in a teratoma. Identifying
the adjacent teratoma elements is essen tial in
making the correct diagnosis and distinguishing
these tumors from metastasis to the ovaries from
malignancies elsewhere in the body.
Gonadoblastoma
Definition
Gonadoblastoma is a rare in situ tumor consisting
of germ cells arranged in nests and admixed with
incompletely mature sex cord cells (Roth and
Cheng 2020).
Clinical Features
The classical gonadoblastoma is usually seen
exclusively in patients with dysgenetic gonads
who have a disorder of sex development (Roth
and Cheng 2020). The age range for
gonadoblastoma is wide with cases reported
from neonatal age up to fourth decade of life.
The majority of gon adoblastomas are incidental
findings while investigating sex development
problems. Although some cases may be present
as a germ cell tumor, gonadoblastoma can be
present as a component of Denys-Drash syndrome
and Swyer syndrome (Milewicz et al. 2016;
Tanteles et al. 2011). It might also be seen in 5%
of patients with androgen insensitivity. The vast
majority of patients with gonadoblastoma are
phenotypically females, though the involved
gonads have Y-chromosomes (Roth and Cheng
2020).
Surgical resection is consider ed curativ e
for gonadoblastoma. The optimal approach is
to perform bilateral gonadectomy as these
patients are at high risk to develop
gonadoblastoma in the contralateral gonad.
When an invasive germ cell tumor develops in
these patients, the invasive component will
guide the prognosis.
Macroscopy
Grossly, gonadoblastomas are ill-defined tan areas
with gritty texture. When an invasive germ cell
arises from a gonadoblastoma, the gross appearance would resemble the usual gross appearance
of the emerging germ cell tumor.
Microscopy
Microscopically, gonadoblastomas are composed of germ cells, immature sex cord
cells, and basement membrane material. The
germ cells and sex cord cells are usua lly
arranged in nests. The germ cells in
gonadoblastomas can be similar to th ose seen
in dysgerminomas of germ cell neoplasia in situ
(GCNIS) of the testis. In some cases, the germ
cells may look like spermatogonia. The sex cord
cells in gona doblastoma appears like immature
Sertoli cells.
In some cases of gonadoblastomas, the sex
cord cells may surround the globular basement
membrane materials forming an appearance
similar to Call-Exner bodies. In other case, the
Sertoli cells may surround the germ cell clusters
or locate the periphery of the tumor. It is commontoseelaminatedcalcifications in the glob-
ular basement membrane deposits. These
laminated calcifications maybe used to make
the diagnosis of burnt-out gonadoblastoma in
the absence of other features. In around twothird of the cases, adja cent to the nests of
dysgerminoma there might be cords of the
same cells which should be differentiated from
an invasive germ cell tumor. This phenomenon
is called dissecting gonadoblastoma (Roth and
Cheng 2018).
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It is always important to carefully look for an
invasive germ cell tumor in cases of
dysgerminoma and the most common germ
cell tumor seen in these cases is dysgerminoma.
Germ cells in dysgerminoma usually stains
positive for OCT3/4 and PLAP while sex cord
cells usually stain positive for inhibin, calretinin,
and SF1.
References and Further Reading
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Biskup, W., Calaminus, G., Schneider, D. T., Leuschner, I.,
& Gobel, U. (2006). Teratoma with malignant transfor-
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Björkholm, E. L. M., Gyftodimos, A., & Silfverswärd,
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cncr2820650110>3.0.co;2-u.
Cheng, L., Roth, L. M., Zhang, S., Wang, M., Morton,
M. J., Zheng, W., Abdul Karim, F. W., Montironi, R.,
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Seminars in Diagnos-
Germ Cell, Lymphoid,
Myeloid, and Secondary
Tumors, Pathology of the
Vulva
Aleodor Andea1and Momal Chand
1
Department of Pathology, University of
Michigan, Ann Arbor, MI, USA
2
Pathology, Ascension St. John Hospital, Detroit,
MI, USA
Germ Cell Tumors of the Vulva
Synonyms
Endodermal sinus tumor; Yolk sac tumor.
Definition
Germ cell tumors are a group of tumors that arise
from the primitive germ cell elements with yolk
sac tumor being the most common variant.
Clinical Features
• Incidence
Primarygermcelltumorsofthevulvaare
extremely rare. They are believed to arise from
the germ cells that persist during embryonic life.
• Age
Patients range in age from 1 to 52 with a mean
age of 19 years.
• Site
Vulva.
• Treatment
Local excision followed by adjuvant
chemotherapy.
• Outcome
Vulvar yolk sac tumor is a rare and aggressive form of germ cell tumor which recurs
usually within 12 months of initial diagnosis
(Mochizuki et al. 2 012) and has a high
risk of distant spread (Flanagan et al. 1997).
Inguinal lymph nodes are the most common
site of recurrence, and lungs are the most
common site of distant metastasis
(Xu et al. 2015).Tumorsizeof5cmorless
is considered favorable prognostic (Basgul
2
et al. 2006).
G
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