OTHER TYPES

Gynandroblastoma
• -sex cord–stromal tumor
• - mixture of granulosa cell and Sertoli–Leydig cell elements.
• -extremely rare.
• -On occasion the granulosa cell component is of the juvenile
• -accompanied by androgenic, estrogenic, or no hormonal
effects.
• -Mutations in DICER1( juvenile type)
Sex cord tumor with annular tubules
-combines features of a granulosa cell tumor
with a pattern of growth reminiscent of Sertoli
cells.
-morphologic hallmark: simple and complex
annular tubules containing eosinophilic hyaline
bodies, often calcified
-appearance is similar to that of
gonadoblastoma
-ambiguous or biphasic nature of the tumor
cells: features consistent with granulosa cell or
nonspecialized ovarian stroma alternate with
features of Sertoli cell differentiation, notably
the presence of Charcot–Bottcher filaments
-Tumors associated with the Peutz–Jeghers
syndrome(50%): multifocal, bilateral, small (or
even microscopic), calcified, and usually benign
-unassociated with the syndrome (22%) are
unilateral, often large, and clinically malignant
 -Grossly,solid or solid and cystic.
Wolffian tumor -Microscopically, epithelial cells are seen
growing in the form of cystic structures,
solid or hollow tubules, and diffuse sheets
-generally benign, but a few have
recurred or metastasized
-useful diagnostic feature: sieve-like
appearance on low-power examination
-PAS-positive basement membranes are
prominent around the epithelial
aggregates.
- Mucin stains are negative.
-Immunohistochemically,(+): keratin (CK7
but not CK20), androgen receptor (3/4),
estrogen and progesterone receptors
(1/4), inhibin (over half), FOXL2, calretinin
(almost all), and vimentin (always).
- (-): GATA3 (most cases) CEA.
Signet ring stromal Sex cord–stromal tumor
tumor of the ovary NOS (indeterminate or
-very rare type, easily mistaken for unclassified type)
Krukenberg tumor.
-examples may be variants of • -uncommon, ovarian tumors in
sclerosing stromal tumor. which the cytologic and/or
-(-): for keratin, PAS, and mucin. architectural features are
-benign. consistent with a sex cord–
stromal origin but which cannot
be assigned to any specific
Microcystic stromal tumor category.
-unilateral and nonfunctioning. • -behavior similar to granulosa
-Microscopically, presence of small cell tumor and Sertoli–Leydig
round to oval cystic spaces, in areas cell tumor, with a generally
coalescing to form larger irregular favorable prognosis when
channels, combined with lobulated confined to the ovaries at
cellular masses with intervening fibrous presentation.
or fibrohyaline stroma.
-Mutations in β-catenin, associated
nuclear immunoreactivity
Germ Cell–Sex Cord–Stromal Tumors
gonadoblastoma-most hyaline material reacts strongly with anti-laminin
distinctive member of the antibodies(basement membrane deposition).
group of tumors composed of a -germinoma (i.e. a tumor that would be
combination of germ cells and diagnosed as a seminoma if arising in the testis or
sex cord–stromal cells dysgerminoma if arising in the ovary) or,
-sexually abnormal individuals, exceptionally, another type of germ cell tumor.
most commonly affected by - found incidentally in normal infants and
gonadal dysgenesis and children in association with follicular
carrying all or part of the Y cysts(precursor of these tumors)
chromosome (i.e. XY gonadal - (+)PLAP, OCT4, and CD117.718
dysgenesis and XO-XY
mosaicism but not XX gonadal -hormonally active and clinically malignant.
dysgenesis).
- testis-specific protein Y-
encoded gene: locus on the Y
chromosome.
- with ataxia–telangiectasia
(36%: bilateral)
- key microscopic feature:
admixture of primitive germ
cells with sex cord–stromal
cells
-Hyalinization and calcification
are common.
Tumors Not Specific to
Ovary
Malignant Lymphoma and
Leukemia
Secondary ovarian involvement by generalized lymphoma or leukemia
-Nearly all ovarian lymphomas are of non-Hodgkin type.
-In children: Burkitt lymphomas predominate; in adults: diffuse large B-
cell lymphomas.
 Sarcoma
extremely rare.
Fibrosarcomas : separated from cellular fibromas mainly on the basis
of their mitotic activity (4 or more per 10 high-power fields) plus
moderate to severe cytologic atypia; hemorrhage and necrosis are also
common.
-large, solid, and accompanied by adhesions. Aggressive.
Endometrial (endometrioid) stromal sarcomas: present as primary
ovarian neoplasms; can closely simulate the pattern of growth of the
diffuse type of granulosa tumor
(+) plump oval cells arranged around vessels of the spiral arteriole
type. (+) CD10, (-) inhibin and FOXL2.
Müllerian adenosarcoma :more closely related to endometrial stromal
sarcoma
TX: surgical excision (“cytoreduction”) followed by chemotherapy.
Other Primary Tumors
• Hemangiomas of the ovary :usually small, found
incidentally, unilateral, and cavernous in type.
Rarely, they are bilateral and/ or associated with
hemangiomas elsewhere in the body.
• Other types of vascular tumors : infantile
hemangioendothelioma, lymphangioma (which can
be bilateral), Glomus tumor.
• Leiomyomas: can exceptionally develop in the ovary,
in association with uterine leiomyomas
• Adenomatoid tumor :occur in an intra- or juxta-
ovarian location; it can simulate the appearance of
yolk sac tumor, (+)oxyphilic cells.
Metastatic Tumors
The ovary is a common site of involvement for metastases.
7%: presenting clinically as primary ovarian tumors are of metastatic origin.
>50% B/L
most common sources: stomach, large bowel, appendix, breast, uterus
(corpus and cervix), lung, and skin (melanoma).
(+) GCDFP-15 and GATA3( primary ovarian carcinoma: (-)
Adenocarcinomas of the large bowel -mainly of the mucinous and endometrioid types but sometimes also
of the clear cell type .
ovarian metastases: cystic, well differentiated, mucin-producing, and associated with necrosis and
hemorrhage.
some cases (cecal origin): luteinization of the surrounding stroma, which may lead to masculinization and
other endocrine changes.
Features favoring metastasis from large bowel: B/L, surface involvement by tumor cells, infiltrative or
nodular pattern of invasion, ovarian hilar involvement, single cell invasion, signet ring cells, garland and
cribriform growth pattern, “dirty” necrosis, segmental destruction of glands, lack of squamous metaplasia,
and vascular invasion. CK7–/CK20+, CDX2+, and SATB2+
Features favoring a primary ovarian tumor: expansile (pushing) pattern of invasion, a complex papillary
architecture, size over 13 cm, a smooth external surface, and the presence of benign- and borderline-
appearing foci. CK7+ and variably CK20+, SATB2+, and CDX2+.
 Ovarian metastases from
gastric, biliary, or pancreatic
primaries have different
phenotypic features
pancreaticobiliary
adenocarcinomas : bland foci
that simulate a benign or
borderline ovarian mucinous
tumor. :majority: primary
appendiceal neoplasms that
have implanted on the
peritoneal surface and on the
ovaries
 Krukenberg tumor :designate an
ovarian neoplasm, usually bilateral
and nearly always of metastatic
origin
grossly: moderate solid
multinodular enlargement of the
ovaries
microscopically: diffuse infiltration
by signet ring cells containing
abundant neutral and acidic (sialo)
mucins. Marked stromal
proliferation with a storiform
pattern of growth and a variable
degree of luteinization are
common and may obscure the
diagnosis.
 tubular variant: prominent tubular
pattern, stromal luteinization, and
occasional associated virilization
(mistaken diagnosis of Sertoli–Leydig
cell tumor) ;(+) typical mucin-positive
signet ring cells should point toward
the correct diagnosis.
occur after the age of 40 years, but
younger patients can also be affected
(mistaken diagnosis of granulosa cell
tumor or steroid cell tumor).
usual primary sources: stomach, large
bowel, appendix, and breast.
diffuse gastric carcinoma (“linitis
plastic”) used to be the most common,
DX: requires the histochemical
identification of intracytoplasmic
mucin, and the immunohistochemical
detection of epithelial markers,
inasmuch as mucin
Others : pancreatobiliary ductal
adenocarcinoma (which can simulate
primary ovarian mucinous tumors and
which can arise in the pancreas,
gallbladder or extrahepatic or intrahepatic
bile ducts), pancreatic acinar cell
carcinoma,renal cell carcinoma (which
mimics ovarian clear cell
adenocarcinoma), carcinoma of the renal
pelvis, thyroid carcinoma (which can be
confused with struma
ovarii),hepatocellular carcinoma and
hepatoblastoma (not to be mistaken for
hepatoid yolk sac tumor or hepatoid
carcinoma, all of which are Hep Par1
positive), intra-abdominal desmoplastic
small cell tumor (of which the ovary can
be the only manifestation),and a variety of
sarcomas, including rhabdomyosarcoma,
leiomyosarcoma,
osteosarcoma,gastrointestinal stromal
tumor (GIST),soft tissue–type clear cell
Malignant melanomas: easy to recognize
because of their pigmentation, nodular
pattern of growth, and cytologic
features;
END OF PRESENTATION