Germ cell–sex cord–stromal

tumors
 Gonadoblastoma
• also known as dysgenetic gonadoma
• occurs practically always in sexually abnormal
individuals, mostcommonly affected by
gonadal dysgenesis and carrying the Y
chromosome
• testis-specific protein Y-encoded gene
– putative gene for the gonadoblastoma locus on
the Ychromosome
• risk of neoplasia – 25%
 Gonadoblastoma
• 36% of the tumors are bilateral
• seen in association with ataxia–telangiectasia
• key microscopic feature is the admixture of
primitive germ cells with sex cord–stromal
cells
• Hyalinization and calcification are common.
– reacts strongly with anti-laminin antibodies,
indicating basement membrane deposition
Ovarian gonadoblastoma. Note the sharply
outlined tumor nests and the heavy
calcification
 Gonadoblastoma
• germ cell component of gonadoblastoma may
overgrow the stromal elements and result in
the formation of a dysgerminoma or,
exceptionally, another type of germ cell tumor
 Gonadoblastoma
• analogy between gonadoblastoma and
intratubular germ cell neoplasia of the testis
on the basis of the morphology and the
immunohistochemical profile
– gonadoblastoma is an in-situ germ cell malignancy
arising in a dysgenetic gonad
Tumors not specific to ovary
Malignant lymphoma and leukemia
• Secondary ovarian involvement by generalized
lymphoma or leukemia
• Rare
• all ovarian lymphomas are of non-Hodgkin type
– In children, Burkitt lymphomas predominate; in
adults, most are diffuse large B-cell lymphomas
• Immunophenotypically all cases are of B-cell
nature
• ovarian lymphoma has been found to coexist
with a borderline serous tumor
 Sarcoma
• Rare
• Fibrosarcomas
– Aggressive
– mitotic activity (4 or more per 10 high-power fields)
plus atypia
• Endometrial (endometrioid) stromal sarcomas
– primary ovarian neoplasms
– presence of plump oval cells arranged around
vessels of the spiral arteriole type.
– reactive for CD10 but generally negative for inhibin.
 Sarcoma
• Other sarcomas of the adult ovary, all
exceptionally rare:
• Myosarcoma
• Chondrosarcoma
• Osteosarcoma
• malignant peripheral nerve sheath tumor
• angiosarcoma
• rhabdomyosarcoma
• low-grade fibromyxoid sarcoma
• Ewing sarcoma/PNET.
Other Primary Tumors
 Hemangiomas
• found incidentally, unilateral, and cavernous in
type
• distinguished from lipid cell tumors having a
prominent vascular component
 Melanoma
• Majority of cases involving ovary are
metastatic
• Primary in the ovary
– mature cystic teratoma or a struma ovarii
Metastatic tumors
 Metastatic tumor
• ovary is a common site of involvement for
metastases
• 7% of lesions presenting clinically as primary
ovarian tumors are of metastatic origin
• 50% - bilateral
• most common sources
– stomach, large bowel, appendix, breast, uterus
(corpus and cervix), lung and skin (melanoma)
 Metastatic Tumor
• Associated with very poor surviva;
• Factors influencing survival
– the site of the primary tumor,
– degree of differentiation,
– menstrual status
– type of treatment
• carcinoma in ovary
– Immunoreactivity for GCDFP-15 =NEGATIVE
– WT1, CA-125 and PAX8 = POSITIVE
• Adenocarcinomas of the large bowel
– high frequency and ability to simulate primary
ovarian carcinomas
• Primary ovarian tumors
– usually advanced (Dukes stage B or C), but
sometimes are small and clinically occult
• ovarian metastases tend to be
– cystic
– well differentiated
– mucin-producing
– associated with necrosis and hemorrhage.
Features Favoring Metastasis Features Favoring A
From Large Bowel Primary Ovarian Tumor
• bilaterality
• surface involvement by tumor • expansile (pushing) pattern
cells of invasion
• infiltrative or nodular pattern of • a complex papillary
invasion, architecture
• ovarian hilar involvement, single • size over 10 cm
cell invasion • a smooth external surface
• Signet ring cells, garland and
• the presence of benign- and
cribriform growth pattern
borderline appearing foci.
• ‘dirty’ necrosis
• segmental destruction of glands,
lack of squamous metaplasia,
and vascular invasion
 Metastatic tumor
Krukenberg tumor
• an ovarian neoplasm
• usually bilateral and nearly always of metastatic origin,
• characterized grossly by moderate solid multinodular
enlargement of the ovaries and
• microscopically by a diffuse infiltration by signet ring
cells containing abundant neutral and acidic (sialo)
mucins
• Tumor emboli – 50%
• Most occur at age 40
 Metastatic tumor
Krukenberg tumor
• Primary sources -stomach, large bowel,
appendix, and breast
• diffuse gastric carcinoma (‘linitis plastica’) –
most common
• diagnosis requires the histochemical
identification of intracytoplasmic mucin, and
the immunohistochemical detection of
epithelial markers
 Metastatic tumor
Other tumors that can metastasize to the ovary:
• pancreatobiliary ductal adenocarcinoma
• pancreatic acinar cell carcinoma
• renalcell carcinoma
• carcinoma of the renal pelvis
• thyroid carcinoma
• hepatocellular carcinoma and hepatoblastoma
• intra-abdominal desmoplastic small cell tumor
• Variety of sarcomas