diseases of ovary ii
DESCRIPTION
Pathology of Ovary mainly neoplasms; germ cells and othersTRANSCRIPT
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Diseases of Ovary II
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..today• Germ cell tumor• Teratoma• Dysgerminoma• Yolk sac tumor• Embryonal carcinoma• Choriocarcinoma
• Sex cord stromal tumor• Granulosa theca cell tumor• Fibroma, thecoma• Sertoli-Leydig cell tumor
• Metastatic tumor
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Germ cell tumor Germ cell tumors arise from primordial germ cells of
the ovary . Of these ovarian lesions, 97% are benign
proliferations (ie, mature teratomas); the remaining 3% are malignant.
In the first two decades of life, almost 75% of ovarian tumor are germ cell origin
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Clinical Features Symptoms and signs – Grow rapidly (except for
benign cystic teratoma)
Pelvic pain related to capsular distension, hemorrhage or necrosis, torsion and rupture
Pelvic mass and pressure symptoms
Ascites in advanced cases.
– Menstrual irregularities in menarcheal patients.
– Precaucious puberty in patient whose tumor produce hCG.
–
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Germ cell tumor and their marker substances
NEOPLASM AFP hCG DYSGERMINOMA _ +/_ ENDODERMAL SINUS (Y S)TUMOR + _ IMMATURE TERATOMA _ _ MIXED GERM CELL TUMOR +/_ +/_ CHORIOCARCINOMA _ + EMBRYONAL CARCINOMA + + NOTE: PLAP and LDH are produce in 95% of
Dysgerminoma
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TeratomaTYPES-• Mature• Immature• Monodermal
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Mature (benign) Teratomas • Also known as Dermoid cyst• Women during
reproductive age group• Excellent prognosis, even
if peritoneal implants are present
C/F • Abdominal mass, pain, • Acute abdomen• May rupture into peritoneal
cavity - simulates metastatic carcinoma or miliary tuberculosis• May present with paraneoplastic
syndrome- inflammatory limbic encephalitis• Or with hemolytic anemia/
virilization
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terms..a/w..teratoma• Dermoid cyst: usually
means teratoma resembles skin
• Gliomatosis peritonei: peritoneal implants exclusively composed of mature glial tissue and other teratomatous elements are absent
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Gross features• cystic or solid• cystic content -greasy material
composed of sebaceous material • keratin, hair, calcification,
teeth, or contains partial mandible• rarely is “fetiform” (partial
human body-like structure) • Rokitansky’s protuberance-a
well defined nipple-like structure covered with hair
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Microscopical features..all three germ• ectodermal structures in 100%, • mesodermal in 93%, endodermal in
71%; • Cyst wall lined by skin (with sweat
sebaceous glands, hair shaft) or other type epithelium
• Other tissues e.g. mature cartilage, mature bone or thyroid tissue
• Presence of glial tissue common; • prostate tissue in 10%; • still considered mature if
microscopic foci of immature tissue
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• This teratoma has cartilage, adipose tissue, and
• intestinal glands at the right,
• thyroid tissue at the left.
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May be seen in associated with• Mucinous cystadenoma• Dysgerminoma
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Malignant change in cystic teratoma
• Squamous cell carcinoma• most common
• Adenocarcinoma • Thyroid carcinoma• Malignant melanoma
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• Teratoma with squamous cell carcinoma show flakes of keratin, cellular tissue fragments • Inset: atypical
cells with individual cell keratinization
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Immature (malignant) teratoma• Mixture of adult & embryonal/ fetal tissue, mainly
immature neuroepithelial component.• Usually prepubertal or young women (mean 18 years)
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Gross
• Bulky • Smooth external surface• Solid or predominantly
solid or cystic with areas of necrosis, hemorrhage• Hair, sebaceous material,
cartilage, bone or calcification +/-
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Micro:• Premature
neurogenic elements (GFAP+);
• mesodermal elements common;
• some tumors derived primarily of esophageal, liver and intestinal structures (endodermal)
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Grading: histologic grade is based on proportion of tissue containing immature neuroepithelium• Norris grading system (correlates best with extraovarian
spread, survival)• 1 - rare foci neuroepithelium occuping <1 low power
fields in any one slide, abundant mature tissue, loose mesenchymal tissue with occasional mitoses, immature cartilage, tooth anlage• 2 - less mature tissue than grade 1, foci of
neuroepithelium with mitoses, < 4 low power fields in any one slide• 3 - little/no mature tissue; numerous neuroepithelial
elements merging with cellular stroma occupying 4+ low power fields
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Monodermal teratomas• Strauma ovarii• Carcinoids• Ependymomas
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Struma ovarii • Rare monodermal teratoma composed predominantly of mature
thyroid tissue
• May show pathologic changes of thyroid gland including hyperfunctioning; malignancies are usually papillary thyroid carcinoma
• Associated with • mucinous cystadenoma, • Brenner tumor, • carcinoid tumor
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Strauma ovariiGross:
• resembles red-brown thyroid tissue
• unilateral
• usually multilocular cystic
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• Micro: • thyroid follicles
with colloid• other
teratomatous elements may be present
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Carcinoid tumors
• 15% have cystic teratoma or mucinous neoplasm in contralateral ovary• 1/3 are associated with
carcinoid syndrome• Low malignant potential-
5%
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Dysgerminoma• Most common malignant germ cell tumor (30-40%)• Analogous to Seminoma of male testis.• 10-15% are bilateral• May arise in gonadal or • Extragonadal sites,
midline structure from pineal gland, mediastinum and the retroperitoneum.
• 5% : before the age of 10 years, 75% : between the ages of 10 and 30 years rarely occur after 50 years of age.
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Dysgerminoma 5% are discovered in abnormal gonads – Associated with pure gonadal dysgenesis (46,XY) , -- mixed gonadal dysgenesis(45,X/46,XY) -- and androgen insensitivity syndrome(46,XY)
– Therefore should be determined karyotype in premenarcheal patients
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Dysgerminoma a/w …• May coexist with • Immature teratoma,• Choriocarcinoma, • Endodermal sinus tumor
•
• 20-30% of ovarian malignancies associated with pregnancy are Dysgerminoma.
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Gross appearance• Size varies widely,
usually Φ 5-15 cm.• Capsule is slightly
bosselated • Cut surface is soft and
fleshy, pale pink or yellow-white in colors .
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Histological characteristicsLarge, round, ovoid or
polygonal cells.Clear, very pale staining
cytoplasm, centrally placed regular nuclei and prominent nucleoli,
Fibrous septa with lymphocytes
Mitotic figures are usually numerous.
Syncitiotrophoblastic cells +/-
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Immunohistochemistry Prognosis
• Oct 3• Oct 4• Nanog• c-KIT• PLAP
• Responds well to chemotherapy• Overall survival >80%
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Endodermal sinus tumor• Derive from primitive
yolk sac.(yolk sac tumor)• The second most
frequent malignant germ cell tomors.
• Median age 16-18 years.• Abdominal pain, 75%• Secrete AFP
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Endodermal sinus tumor• Soft grayish brown • 10-30cm, • capsule is intact• Cut surface gray yellow
with solid- cystic, hemorrhagic and necrotic change.• Unilateral 100%• biopsy of opposite ovary
is contraindicated
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Endodermal sinus tumor• Scattered tubules lined by single
layers of flattened cuboidal cells.
• Loose reticular stroma
• Papillary structure with a central blood vessel (Schiller Duval body)
• Polyvesicular vitelline, hepatoid, glandular
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Endodermal sinus tumor….hyaline globule• Eosinophilic intracellular
or extracellular • PAS +• Diastase-resistant • Most often found in the
reticular and endodermal sinus patterns.• Immunohistochemical
staining positive for AFP, alfa1-AT
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Embryonal carcinoma• Rare• Median age 15 yrs, aggressive
• More than 50% cases present with isosexual pseudoprecosity, painful abdominal mass, abnormal uterine bleeding, amenorrhea
• Increased serum hCG and AFP levels
• Extraovarian spread in 40% cases
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• Median diameter 17cm• External
surface: • smooth and
glistening
• Cut surface: predominantly solid and variegated
Extensive areas of necrosis and hemorrhage
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•Solid sheets and nests •Large anaplastic cells: pale vacuolated cytoplasm; large pleomorphic nuclei; prominent nucleoli.
•Occasionally forming papillae and abortive glandular structures
•Frequently syncytiotrophoblast-like tumor cells scattered
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Polyembryoma• consists of small embryoid bodies having a central
‘germ disc’ composed of embryonal carcinoma like epithelium and two cavities
• Tumor marker: Increased hCG, AFP
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Embryoidbody having a
core of embryonal carcinoma like cell, a
dorsal amniotic
cavity, and a ventral
component of yolk sac
tumor
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Choriocarcinoma• Rare• Below age 20 yrs• Clinical Features: abdominal mass, pain isosexual
pseudoprecosity, menstural irregularities.• Component of mixed germ cell tumor• Very high hCG
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Gross • Soft• Hemorrhagic• Necrotic• pink tan in color
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Histopathology• Clusters of cytotrophoblast
separated by streaming masses of syncytiotrophoblast:
• Cytotrophoblast: centrally placed hyperchromatic nuclei, prominent nucleoli, clear cytoplasm, well defined cell border
• Syncytiotrophoblast: multinucleated
• characteristic dimorphic plexiform pattern 15%
• hemorrhage
• necrosis
• Villi characteristically absent
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Metastases IHCLungbrainliverkidneybowel
Choriocarcinoma cells: positive for: • hCG• keratin
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Granulosa theca cell tumor• varying proportions of granulosa and theca cell
differentiation• postmenopausal women
• elaborate large amounts of estrogen • distinct hazard of malignancy
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Gross • usually unilateral • vary in size• solid, and cystic encapsulated masses• Tumors that are hormonally active have a yellow coloration to their cut surfaces, due to intracellular lipids• The pure thecomas are solid, firm tumors
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Microscopy • The small, cuboidal to
polygonal cells • Cells have scant cytoplasm
and grooved nucleus(coffee bean)• may grow in anastomosing
cords, sheets, or strands• Small, distinctive, gland-like
structures filled with an acidophilic material similar to immature follicles (Call-Exner bodies)
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Immunohistohemistry • Inhibin
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Fibromas, Thecomas, Fibrothecomas• Tumors arising in the ovarian stroma - fibroblasts
(fibromas) or• Plump spindle cells with lipid droplets (thecomas) • Many tumors contain a mixture of these cells and are
termed fibromathecomas
• Meigs syndrome• Fibroma ovary• Hydrothorax• Ascitis
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Sartoli leydig cell tumorAlso known as Androblstoma
Second and third decade
Virilisation
Tubules composed of Sertoli cells or Leydig cells
interspersed with stroma
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Other sex cord stromal tumor• Hilus cell tumor-• Reinke crystal• Virilizing tumor
• Gonadoblastoma• Both germ cell and sex cord stromal derivatives• Coexsisting Dysgerminoma in 50% cases
• Pregnancy leuteoma
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Metastatic tumor• Uterus• Fallopian tubes• c/l ovary• Pelvic peritoneum
• Ca breast• GIT• Krukenberg tumor
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Krukenberg tumor…
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Thank
you