diseases of ovary ii
DESCRIPTION
Pathology of Ovary mainly neoplasms; germ cells and othersTRANSCRIPT
Diseases of Ovary II
..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
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
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.
–
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
TeratomaTYPES-• Mature• Immature• Monodermal
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
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
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
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
• This teratoma has cartilage, adipose tissue, and
• intestinal glands at the right,
• thyroid tissue at the left.
May be seen in associated with• Mucinous cystadenoma• Dysgerminoma
Malignant change in cystic teratoma
• Squamous cell carcinoma• most common
• Adenocarcinoma • Thyroid carcinoma• Malignant melanoma
• Teratoma with squamous cell carcinoma show flakes of keratin, cellular tissue fragments • Inset: atypical
cells with individual cell keratinization
Immature (malignant) teratoma• Mixture of adult & embryonal/ fetal tissue, mainly
immature neuroepithelial component.• Usually prepubertal or young women (mean 18 years)
Gross
• Bulky • Smooth external surface• Solid or predominantly
solid or cystic with areas of necrosis, hemorrhage• Hair, sebaceous material,
cartilage, bone or calcification +/-
Micro:• Premature
neurogenic elements (GFAP+);
• mesodermal elements common;
• some tumors derived primarily of esophageal, liver and intestinal structures (endodermal)
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
Monodermal teratomas• Strauma ovarii• Carcinoids• Ependymomas
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
Strauma ovariiGross:
• resembles red-brown thyroid tissue
• unilateral
• usually multilocular cystic
• Micro: • thyroid follicles
with colloid• other
teratomatous elements may be present
Carcinoid tumors
• 15% have cystic teratoma or mucinous neoplasm in contralateral ovary• 1/3 are associated with
carcinoid syndrome• Low malignant potential-
5%
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.
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
Dysgerminoma a/w …• May coexist with • Immature teratoma,• Choriocarcinoma, • Endodermal sinus tumor
•
• 20-30% of ovarian malignancies associated with pregnancy are Dysgerminoma.
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 .
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 +/-
Immunohistochemistry Prognosis
• Oct 3• Oct 4• Nanog• c-KIT• PLAP
• Responds well to chemotherapy• Overall survival >80%
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
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
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
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
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
• Median diameter 17cm• External
surface: • smooth and
glistening
• Cut surface: predominantly solid and variegated
Extensive areas of necrosis and hemorrhage
•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
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
Embryoidbody having a
core of embryonal carcinoma like cell, a
dorsal amniotic
cavity, and a ventral
component of yolk sac
tumor
Choriocarcinoma• Rare• Below age 20 yrs• Clinical Features: abdominal mass, pain isosexual
pseudoprecosity, menstural irregularities.• Component of mixed germ cell tumor• Very high hCG
Gross • Soft• Hemorrhagic• Necrotic• pink tan in color
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
Metastases IHCLungbrainliverkidneybowel
Choriocarcinoma cells: positive for: • hCG• keratin
Granulosa theca cell tumor• varying proportions of granulosa and theca cell
differentiation• postmenopausal women
• elaborate large amounts of estrogen • distinct hazard of malignancy
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
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)
Immunohistohemistry • Inhibin
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
Sartoli leydig cell tumorAlso known as Androblstoma
Second and third decade
Virilisation
Tubules composed of Sertoli cells or Leydig cells
interspersed with stroma
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
Metastatic tumor• Uterus• Fallopian tubes• c/l ovary• Pelvic peritoneum
• Ca breast• GIT• Krukenberg tumor
Krukenberg tumor…
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