Ovarian tumor is a commonest malignancy in female

1. OVARIAN TUMOUR PART-2
By –DR. PRIYANKA GUPTA

2. CLASSIFICATION OF OVARIAN TUMORS

3. GERM CELL TUMORS
Constitute approximately 20% of all ovarian neoplasms.
Most of them are seen in children and young adults.
Approximately 95% are benign cystic teratomas.
Younger the patient , more likely the germ cell tumour will be malignant.

5. TYPE OF GERM CELL TUMORS
PRIMITIVE GERM CELL TUMORS
• Dysgerminoma
• Yolk sac tumour
• Embryonal carcinoma
• Polyembryoma
• Choriocarcinoma
BIPHASIC OR TRIPHASIC TERATOMAS
• Immature teratoma
• Mature teratoma

6. MONODERMAL TERATOMAS AND SOMATIC TYPE TUMORS ASSOCIATED WITH
DERMOID CYSTS
• Thyroid tumor group (Struma Ovarii)
• carcinoid group
• Neuroendocrine group
• Carcinoma group (SCC and adenocarcinoma)
• Melanocytic group
• Sarcoma group
• Sebaceous tumor group

7. DYSGERMINOMA
• Constitutes less than 1% of all ovarian tumours.
• Most patients are young.
• Approximately 5% of dysgerminomas arise in abnormal gonads:-
pure or mixed gonadal dysgenesis (from a gonadoblastoma)
or testicular feminization (androgen insensitivity) syndrome.
• Exceptionally, the tumor is associated with Hypercalcemia.
• More common on the right side and is bilateral in 15% of cases.

8. GROSS- It is often large (may reach over 1000g) and encapsulated
Smooth, often bosselated surface
CUT SURFACE- solid tan,white to gray, foci of hemorrhage and necrosis

9. MICROSCOPY- tumor cells usually group themselves in well defined nests
separated by fibrous strands infiltrated by lymphocytes(most of which are T
cells ).
• Occasionally, a pseudotubular , alveolar, or cord like arrangement may be
seen.
• Focal necrosis ,hyaline changes in vessels, germinal centers, and
granulomatous foci may be present.
• Indivisual tumour cells are uniform and have squared-off nuclei , one or
more prominent elongated purple nucleoli, and abundant clear to finely
granular cytoplasm that contains glycogen and fine droplets of fat.
• The cell membrane is prominent.

11. IMMUNOHISTOCHEMISTRY
• Tumor is consistently reactive for PLAP and CD117,variably for keratin (erratically and focally),
and sometimes for GFAP and desmin.
• OCT3/4 stains the cells of dysgerminoma , the germ cell component of gonadoblastoma , and
embryonal carcinoma, but not yolk sac tumor.
• SALL4 is typically positive.
• Negative for EMA,CEA,hCG,CD30,glypican3,AFP
• D/D-
1. Yolk sac tumor
2. Lymphoma
3. Clear cell carcinoma
4. Embryonal carcinoma

12. Ovarian dysgerminoma resembles classic seminoma of the testis.
Like testicular seminoma, ovarian dysgerminoma may exhibit signs of early
differentiation toward other types of germ cell elements. They include:-
1. Scattered HCG-positive syncytiotrophoblastic cells,often in close proximity to
blood vessels or to hemorrhagic foci. This change, seen in approximately 3% of
all dysgerminomas, may be accompanied by serum elevation of hCG and tissue
immunoreactivity for this marker.
2. Abortive yolk sac elements accompanied by serum elevation of alpha-fetoprotein
and tissue immunoreactivity for this marker.
Metastases of dysgerminoma occur more commonly in the contralateral ovary,
retroperitoneal nodes, and peritoneal cavity.
The survival rate of pure dysgerminoma is 95%.

14. YOLK SAC TUMOR (ENDODERMAL SINUS TUMOR)
 Neoplasm of children and young adults (median
age,19 years).
 Serum AFP level elevated whereas chorionic
gonadotropin level were normal.
 GROSS- Usually unilateral ovarian mass with
predilection for right ovary.
an average diameter of 15 cm.
smooth and glistening external surface,
Cut surface is variegated, partially cystic and
often
containing large foci of hemorrhage and necrosis.

15. MICROSCOPY-
 Multiple histologic patterns with predominance of 1 or 2 patterns-
 Reticular / microcystic pattern:
• Most common pattern
• Loose meshwork of anastomosing channels and variably sized cysts (macro or
microcysts) lined by flat or cuboidal cells having varying amounts of clear to
eosinophilic cytoplasm.
• Tumor cells occasionally contain lipid and have a signet ring-like morphology
• Cysts may contain eosinophilic hyaline globules and amorphous, eosinophilic
acellular basement membrane-like material.
• Formation of glomeruloid perivascular structures (Schiller-Duval bodies)
Hallmark of yolk sac tumor but their absence does not rule out the diagnosis.
Rounded to elongated papillary structures containing a central fibrovascular
core with a single central vessel, surrounded by tumor cells projecting into a cystic /
sinusoidal space (resembling immature glomeruli).
 Other patterns are-Endodermal sinus, Solid, Alveolar-glandular, Polyvesicular
vitelline, Myxomatous, Papillary, Macrocystic, Hepatoid, Glandular or primitive
endodermal (intestinal).

16. A and B, Low- and high-power views of ovarian yolk sac tumor. Numerous hyaline
globules are seen in the cytoplasm of the tumor cells lining the papillae.

17. Schiller-Duval body with a single central vessel, surrounded by
several layers of tumor cells.

18. IMMUNOHISTOCHEMISTRY
 Intracytoplasmic and extracellular PAS-positive hyaline droplets are nearly always
present, they usually stain for alpha-fetoprotein(AFP), contain α1-antitrypsin and
basement membrane components (type IV collagen and laminin).
 Yolk sac tumor also stains for pankeratin but not for keratin 7 or EMA and WT1.
 They also exhibit positivity for SALL4 and glypican 3,a property they share with
testicular and extragonadal yolk sac tumors.
 However, OCT4 is typically negative.
 D/D-
1. Clear cell carcinoma
2. Embryonal carcinoma
3. Dysgerminoma
4. Endometriod ca

19. EMBRYONAL CARCINOMA
Also occurs in a young age group (median age, 15 years).
Serum chorionic gonadotropin levels are high.
GROSS- Average diameter of tumour is 17 cm.
Their external surface is smooth and glistening .
ON CUT- predominantly solid and variegated, with
extensive areas of necrosis and hemorrhage.
MICROSCOPY- it is composed of solid sheets and
nests of large primitive cells, occasionally
forming papillae and abortive glandular
structures.

20. Microscopy of embryonal carcinoma

21. Embryonal carcinoma. The tumor shows pseudoglandular
and solid patterns

22. IMMUNOHISTOCHEMIDTRY- Syncytiotrophoblast-like tumor cells are frequently
seen scattered among the smaller cells; these are immunoreactive for hCG.
Embryonal carcinoma shows immunoreactivity for pan-keratin, CD30, OCT4, and
SALL4.
Embryonal carcinomas largely composed of embryoid bodies are referred to as
polyembryomas.

23. Polyembryoma- The embryoid body shows amniotic
cavity, embryonic disk and atypical yolk sac

24. CHORIOCARCINOMA
• Most choriocarcinomas involving the ovary represent metastases from uterine
tumors.
• Primary ovarian choriocarcinomas are very rare.
• Primary ovarian choriocarcinomas can develop from an ovarian
pregnancy(gestational type, which is most common) or as a germ cell
neoplasm(non-gestational type).
GROSS:- Pure choriocarcinoma present as solid, hemorrhagic and friable mass.
MICROSCOPY:- Biphasic pattern of syncytial and cytotrophoblastic elements in
necrotic and hemorrhagic back ground.
• Immunohistochemistry for HCG (Human Chorionic Gonadotrophin) is positive.
• Ovarian gestational choriocarcinomas have a better prognosis than nongestational
choriocarcinoma.

26. TERATOMA
Teratoma consists of tissues representing all the three germ layers.
They are classified as
1. Immature teratoma
2. Mature teratoma
3. Monodermal teratoma.
Immature teratoma- It is Malignant ovarian neoplasm usually seen in children and
adolescents and composed of embryonal and adult tissues derived from all three germ
layers.
GROSS- Mostly unilateral and size ranges from 15 to 18cms.
On cut surface shows partly cystic and solid areas. Focal areas of necrosis and hemorrhage
are seen.

28. Microscopy:- A mixture of mature and immature elements are seen.
Immature elements in the form of neuroepithelial tubules, rosettes, immature
cartilage, fat, liver tissue, endodermal glands are seen.
Grading system of immature teratoma is as follows:-
Grade 1- tumours have predominantly mature tissue. Rare foci of immature
neuroepithelial tissue that occupy <1 low power field (4X) in any slide. [LOW
GRADE]
Grade 2- tumours have admixture of mature with few foci of immature
neuroepithelium occupying 1-3 low power fields (4x) in any slide.[HIGH GRADE]
Grade 3- tumours with large amount of immature neuroepithelial tissue occupying
>3 low power fields (4X) in any slide.[HIGH GRADE]

30. MATURE SOLID TERATOMA
 predominantly solid gross appearance, but multiple small cystic areas also are
present.
 composed entirely of adult tissues derived from all three germ layers.
some authors refer to mature solid teratoma as “grade 0” immature teratoma.
 Rare neoplasm occurs in young women, predominantly in the second decade.
 The prognosis is excellent.

31. MATURE CYSTIC TERATOMA
• It accounts for 25% of all ovarian tumour.
• 90% of germ cell tumours present as mature cystic teratoma.
• They constitute the most common ovarian tumor in childhood ( more common in
20-50 years of age).
• Predominantly they are unilateral tumours (88%).
GROSS:- They present as multiloculated cyst. The cystic content is greasy and
usually contain keratin, sebum, hair and teeth sometimes an imperfectly formed
mandible or a partial human body like configuration (Homunculus/fetiform
teratoma) is found. The characteristic Rokitansky protuberances with variety of
tissue types are found. Solid areas should be grossed carefully to rule out immature
teratoma.
The teeth tend to be located in a well-defined nipple-like structure covered with hair,
known as Rokitansky’s protuberance

33. • MICROSCOPY:-A mixture of ectodermal, mesodermal, endodermal, elements are
seen. They are composed of hair follicles, epithelium, salivary gland, thyroid and
respiratory tract epithelium. Benign tumours include cutaneous adnexal tumours,
salivary gland tumours.
• mature ovarian teratomas of either the cystic or solid variety—as well as immature
teratomas—may be accompanied by peritoneal nodules exclusively composed of
mature glial and neuronal tissue, a condition known as gliomatosis peritonei.
• These nodules appear grossly as miliary grayish-white nodules in the peritoneal
surface or omentum and may be accompanied by fibrosis and chronic inflammation.
• This is a benign process, as long as the glial tissue is entirely mature and
unaccompanied by other teratomatous elements.

34. Mature cystic teratoma-The lining of the
cyst is composed
of skin with its appendages

35. Various Tissue Components of
Mature Cystic Teratoma of Ovary.
A, Skin adnexa, glial tissue, and
choroid plexus; B, gastric mucosa
of pyloric type; C, anterior
pituitary gland.

38. “Somatic-type” Tumors Developing in Mature Cystic Teratoma- Emergence of a
benign or a malignant neoplasm with somatic-type features is an uncommon
event in mature cystic teratoma, occurring in approximately 2% of all cases.
• The most common malignant change in cystic teratoma is squamous cell
carcinoma.
• These ovarian squamous cell carcinomas typically present in the 5th or 6th
decade.
• The prognosis of squamous cell carcinoma arising from a teratoma is guarded
and these tumors are characterized by aggressive locally invasive growth.
Epidermoid Cyst -The rare epidermoid cyst of the ovary arises from epithelial
cell nests, of the type encountered in Brenner tumors.
• It is distinguished from mature cystic teratoma on thorough sampling by the
absence of skin adnexa and other tissues.

39. MONODERMAL TERATOMA
It consists of tissues derived from one germ cell layer.
The most common to occur is struma ovary. Other rare entities are carcinoid tumour
and neuroectodermal elements tumour.
STRUMA OVARII
• The predominant component in this type is thyroid tissue.
• It constitutes 2 to 7% of all ovarian teratomas.
• Malignancy is rarely encountered.
• If it arises, it presents as papillary carcinoma with typical nuclear features.
• The thyroid nature of the lesion has been fully documented with biologic and
immunohistochemical studies for TTF-1 and thyroid hormones

40. Gross- they are less than 10cms in maximum extension. the
mass has the color and consistency of thyroid tissue, but it is often cystic

41. Cut surface is solid tan with glistening
surface.
Microscopy- shows numerous follicles
filled with colloid.
The tissue may show any of the pathologic
changes seen in a normally placed gland,
including diffuse or nodular hyperplasia
(which may lead to hyperthyroidism),
thyroiditis, papillary carcinoma (including
the follicular or microcarcinoma variants and
featuring either RAS or BRAF mutation),
follicular carcinoma (sometimes resulting in
peritoneal spread, so-called peritoneal strumosis)

42. CARCINOID TUMOUR
• The neuroendocrine tumours are more common in older age group.
• Carcinoid tumor can be seen in the ovary as a metastasis of a primary tumor located in the
gastrointestinal tract or elsewhere, as a component of adult cystic teratoma, or as a primary
pure neoplasm of this organ.
• The large majority of primary ovarian carcinoid tumors are unilateral, but in 16% of cases
the contralateral ovary is involved by a cystic teratoma or a mucinous neoplasm.
• The patient present with menstrual irregularities and abdominal pain.
• Gross- Pure primary carcinoid tumors have a mean diameter of 10 cm.
external surface is smooth or bosselated.
Cut surface is predominantly solid, firm, tan to yellow, and homogeneous.

44. • Microscopy-the appearance is similar to that of carcinoid tumors elsewhere, in
that they recapitulate the various patterns of this well-differentiated
neuroendocrine tumor as seen in various sites.
• Thus, there are tumors with an insular pattern of growth similar to those seen in
the appendix and small bowel, tumors with a trabecular appearance similar to
those seen in the rectum and tumors with a mucinous (goblet cell) appearance
similar to those seen primarily in the appendix.
IMMUNOHISTOCHEMISTRY
• Neuron-specific enolase (NSE), chromogranin, 5-HT, and a large variety of
peptide hormones (including peptide YY) have been demonstrated
immunohistochemically, particularly in tumors of the trabecular type.
• A potential pitfall should be mentioned here: primary ovarian carcinoid tumors
and those metastatic from the gastrointestinal tract show identical
immunoprofiles (e.g. expression of CDX2) so immunohistochemistry is not
helpful in distinguishing primary from metastatic carcinoid tumor

46. STRUMAL CARCINOID
• an ovarian neoplasm combining the features of carcinoid tumor and
struma ovarii .
A, Gross appearance of strumal carcinoid showing a variegated appearance resulting from the admixture of
carcinoid tumor and struma ovarii. B, Microscopic appearance, showing intimate admixture of thyroid
follicles and carcinoid trabecula.

47. SEX CORD-STROMAL TUMOR
• These tumors account for approximately 5% of ovarian neoplasms.
• Benign > Malignant
• All granulosa tumors have malignant potential; although most do not recur or
metastasize
• Composed of Granulosa cells, Theca cells, Sertoli cells, Leydig cells and Fibroblasts
of stromal origin

48. GRANULOSA CELL TUMOR
• 1.5% of all ovarian neoplasms
• MC malignant sex cord stromal tumor.
• Two types-
1. ADULT
2. JUVENILE
• Granulosa cell tumor is a sex cord–stromal ovarian neoplasm showing
differentiation toward follicular granulosa cells.

49. ADULT GRANULOSA TUMOR
• 95% of all granulosa cell tumors.
• Age: 50-55 years (Occurs in middle aged to postmenopausal women).
• Most common estrogenic ovarian tumor.
• Usually unilateral
• GROSS- Variable size (average diameter-10 cms),smooth,
lobulated outline
• predominantly solid cut surface
• color is usually gray, but it may be yellow in areas of luteinization .
• Cysts may be filled with straw-colored or mucoid fluid.

52. MICROSCOPIC EXAMINATION-
• Several Patterns of growth include microfollicular (m/c),
macrofollicular, trabecular, insular, watered-silk, solid, pseudopapillary,
and diffuse (sarcomatoid) patterns.
• Fibrothecomatous stroma often surround the granulosa cells.
• Tumor cells resemble normal granulosa cells, CALL-EXNER BODIES,
nuclear GROOVES and bizarre nuclei maybe seen.
• An important diagnostic feature is the presence of folds or grooves in
the nuclei, resulting in a “coffee-bean” appearance.

53. A and B, Microscopic appearance of adult granulosa cell
tumor. Call–Exner bodies are seen in B.

55. IMMUNOHISTOCHEMISTRY
• granulosa cell tumors include vimentin, FOXL2, and SF-1.
• Adult granulosa cell tumor positive for CK8 and CK18,CD99.
• ER and PR also positive .
• Approx 50% cases are reactive for S-100 and negative for EMA.
• Adult granulosa cell tumor show somatic mutation in the FOXL2 gene
(402C→G).
• The peptide hormone inhibin and follicle regulatory proteins, two substances
normally produced by ovarian granulosa cells, have been found to be elevated
in the serum of patients with granulosa cell tumor.

56. JUVENILE GRANULOSA CELL TUMOR
• Predominantly in first 3 decades (average age 15yrs).
• Associated with enchondromatosis (Ollier disease), Maffucci
syndrome, Goldenhar or Potter syndrome(bilateral)
• Presents as isosexual pseudoprecocity
• Prognosis good.
• They also show consistent trisomy for chromosome 12.
• GROSS- usually unilateral with a smooth surface.
• Mean size is 12.5 cm.
• Multiloculated, cystic and solid tumor with yellow –white solid
areas.
• May have hemorrhage and necrosis.

58. Microscopic examination-
• Macrofollicular, solid and cystic patterns
• Follicles contain mucinous material, lined by one or more layers of
granulosa cells
• Cells are polygonal to spindled shape, ample amount of
amphophilic/pink cytoplasm. Nuclei large round usually darkly
stained.
• LACK of nuclear grooves.
• Focal or extensive luteinization is a typical finding.
• CYTOMEGALY with macronuclei, multinucleation and bizzare
multilobulated nuclei occasionally seen.
• Mitotic figures average 6 /10 hpf.

59. Juvenile Granulosa Cell Tumor. The follicle-like spaces seen on low-power examination
(A) are a common feature of this neoplasm. On high power (B) the tumor cells are seen
to lack the coffee bean nuclei seen in the adult type.

60. IMMUNOHISTOCHEMISTRY
• Positive for inhibin, calretinin,SF1,WT1,FOXL2.
• Negative for SALL4,Glypican,AFP.
• DIFFERENTIAL DIAGNOSIS-
1. Adult granulosa cell tumor
2. Sertoli-Leydig cell tumor
3. Clear cell carcinoma
4. Yolk sac tumor

61. ADULT GCT
• Less than 1% prepubertal
• Usual after 30 years
• Mature follicles
• Call-Exner bodies common
• Nuclei pale, angular,
commonly grooved
• Luteinization infrequent
JUVENILE GCT
• 50% prepubertal
• Rare after 30 years
• Immature follicles with
mucin content
• Call-Exner bodies rare
• Nuclei darker, round,
ungrooved
• Luteinization frequent

62. THECOMA
• Almost always benign.
• Composed of cells resembling theca cells.
• Usually occur in postmenopausal women( mean age =59 years) presents as uterine
bleeding.
• GROSS- well defined capsule and a firm consistency usually unilateral
• Most are <5cm
• Solid , yellow and lobulated / white with focal yellow areas.
• Necrosis is rare.

63. Thecoma: the sectioned surface is lobulated and yellow
Well circumscribed, yellow-tan mass

65. • MICROSCOPIC EXAMINATION-
• it is composed of fascicles of spindle cells with ill-defined borders, centrally placed
nuclei, and a moderate amount of pale grayish-pink cytoplasm.
• The intervening tissue may show considerable collagen deposition and focal hyaline
plaque formation.
• diffuse or nodular growth pattern
• Calcification is more common in young patients.
• Absent or minimal nuclear atypia.
• Cellularity is variable considerably.

67. IMMUNOHISTOCHEMISTRY
• Positive for inhibin, calretinin, oil red O, vimentin , FOXL2, WT1, CD56.
• Reticulin stain shows a pericellular pattern.
• Negative for pancytokeratin,CD10,CD117,DOG1,CD99.

68. FIBROMA
• Benign stromal tumor composed of fibroblastic cells within a variable collagenous stroma.
• Most common ovarian stromal tumor.
• usually after puberty(mean age is 48 years).
• GORLIN syndrome should be suspected in young patients with bilateral calcified ovarian
fibroma.
• GROSS- usually unilateral, solid, lobulated, firm, uniformly white, white to yellow –white to
tan –yellow cut surface may be whorled.
• The average diameter is 6 cm.
• Myxoid or edematous changes may be seen, sometimes resulting in cystic degeneration.
• Grossly, fibromas may have an appearance similar to thecoma, Brenner tumor, and
Krukenberg tumor.

69. A and B, Outer aspect and cut surface of ovarian
fibroma. The white color contrasts with the yellow hue
of thecoma

70. MICROSCOPIC EXAMINATION-
• composed of closely packed spindle stromal cells arranged in a “feather-stitched” or
storiform pattern within a variably collagenous stroma.
• Bland spindled to ovoid nuclei with pointy ends and scant eosinophilic cytoplasm
blending with surrounding stroma.
• Hyaline bands, edema, and hyaline globules may be present.
• Cellular Fibroma(10% cases ):-resembles adult granulosa cell tumor,densely cellular
with little intercellular collagen.
• Cytogenetically, both thecomas and fibromas have been found to exhibit trisomy of
chromosome 12 in a minority of the tumor cells.
• Loss of heterozygosity at both the PTCH gene (implicated in Gorlin syndrome) and
the STK11 gene (implicated in Peutz–Jeghers syndrome) is relatively frequent in
cellular fibromas.
• Ovarian fibroma (especially if large) can be associated with ascites, sometimes in
combination with right-sided pleural effusion (Meigs syndrome)

72. IMMUNOHISTOCHEMISTRY-
Positive for WT1,SF1,FOXL2,INHIBIN(only 50% cases ),VIMENTIN,.
RETICULIN-diffentiates fibroma (indivisual pericellular reticulin staining
pattern )from diffuse type of adult granulos cell tumor (nested reticulin
staining pattern).
Differential diagnosis-
1.Diffuse adult granulosa cell tumor (GCT).
2.Thecoma.
3.Sclerosing stromal tumor.
4.Leimyoma.
5.Fibrosarcoma.

73. FIBROSARCOMA-
Most common ovarian
sarcoma
• Any age but usually older
age
• Unilateral (usually), large,
solid, hemorrhage and
necrosis common.
• Tumors with an average of
4 or more MF/10 HPF and
significant nuclear atypia
are almost always
associated with a
malignant course and
warrant the designation
fibrosarcoma.
• Poor prognosis.

74. Sclerosing stromal tumor- benign ovarian neoplasm that shares many features
with fibroma and thecoma.
occurs in a younger age group, has a less homogeneous gross appearance
M/E- a lobular pattern of growth, interlobular fibrosis, marked vascularity
the presence of a dual cell population: collagen-producing spindle cells and
lipid-containing round or oval cells .

75. SMALL CELL CARCINOMA OF HYPERCALCEMIC TYPE
• high-grade ovarian malignancy that may be confused with granulosa cell tumor.
• occurs in young females (average age, 23 years)
• often bilateral
• Familial cases have been reported.
• The tumor is associated with hypercalcemia in up to two-thirds of cases, which
disappears following removal of the tumor.
• GROSS- the tumor is large and solid, with areas of necrosis and hemorrhage.

77. Microscopic Examination-
• a diffuse proliferation of small, closely packed cells of
carcinomatous appearance with scant cytoplasm and small nuclei is
seen.
• Clusters of larger and more pleomorphic rhabdoid cells are present
in approximately half of cases.
• Cytoplasmic hyaline globules may also be seen.
• Tumors containing a large number of these larger cells have been
referred to, tongue-in-cheek, as the “large cell variant” of small cell
carcinoma.
• There may also be islands, cords, trabeculae, mucinous glands, and
follicle-like structures
• IHC-The tumor cells usually express keratin, vimentin, EMA, and
WT1 but not S-100 protein, chromogranin, inhibin, CD117, or
OCT4.

79. SERTOLI–LEYDIG CELL TUMOR
• Rare ovarian tumor composed of sex cord (Sertoli cells) and stromal (Leydig cells)
elements , accounting for <1% of all ovarian neoplasms.
• GROSS-
• almost always unilateral.
• Ranges widely from <1cm to 35 cm (mean 12-14).
• Cut surface is typically solid but cystic areas is also seen , tan –yellow

81. Microscopy- Several major patterns are seen, which may coexist in the same tumor:
1.Well differentiated (11%)-Composed of tubules lined by Sertoli-like cells separated
by variable numbers of Leydig-like cells open or closed tubules, lack nuclear atypia
,mitotic figures.
2. Moderately differentiated (intermediate grade) (54%)- Characterized by the
formation of cords, sheets, and aggregates of Sertoli-like cells, separated by spindle
stromal cells and recognizable Leydig cells. MF 5-10/hpf
3. Poorly differentiated (sarcomatoid; undifferentiated) (13%)- Composed of masses
of spindle-shaped cells arranged in a “sarcomatoid” pattern. MF ≥20/hpf
The microscopic pattern can be tubular or follicle like.
Amyloid-like material can be present, and cytoplasmic crystalline structures have
been found by electron microscopy. Some Sertoli cell tumors are composed of cells
with oxyphilic cytoplasm.

85. 4.With heterologous
elements (22%). Rare
cases are associated
with tissues such as
mucinous epithelium
of gastrointestinal
type, liver, skeletal
muscle, or cartilage.
The epithelial
component of this
neoplasm contains a
variety of endocrine
cells and can give rise
to microscopic
carcinoid tumors.

86. Gross appearance of Sertoli–Leydig cell tumor of the
retiform variant.
5. Retiform (15%). In this
category, typical elements of
Sertoli–Leydig cell tumors
coexist with formations
resembling the rete of the ovary
or testis.
These appear as irregular cleft-
like spaces lined by low cuboidal
cells; blunt papillae with
hyalinized or edematous cores
are often present.

88. Immunohistochemistry-
testosterone and estradiol are found in both Sertoli and Leydig cells and less
frequently in primitive stromal cells. The areas of Sertoli cell differentiation stain for
keratin and Sox-9.
Negative for CK7, EMA, PLAP, CEA, or S-100 protein).
Positivity for inhibin, calretinin, and WT1 is seen in most tumors.
Mutations in DICER1, which encodes an endoribonuclease involved in miRNA
processing, are found in most Sertoli–Leydig cell tumors.
D/D-
1. Endometrioid adenocarcinoma
2. Adult granulosa cell tumor.
3. Fibroma.

89. GYNANDROBLASTOMA-
• Tumor containing significant
components of both forms- sertoli
cell and granulosa cell.
• Extremely rare tumor, benign,
young adults.
• Granulosa cell component should
account for at least 10% of the
tumor in sex cord-stromal category
to warrant a diagnosis of
gynandroblastoma.
• MICROSCOPY-: Well formed
hollow tubules lined by Sertoli
cells generally admixed with
rounded islands of granulosa cells
in a MICROFOLLICULAR
pattern.
• Alpha inhibin positive

90. STEROID CELL TUMOR
• A small group of ovarian tumors is composed entirely of cells with
morphologic features indicative of steroid hormone secretion.
• These are manifested by an abundant eosinophilic or vacuolated
cytoplasm that is often positive for fat stains and that, at the
ultrastructural level, is shown to contain well-developed smooth
endoplasmic reticulum and mitochondria with tubulovesicular cristae.
• Normal steroid hormone-secreting cells can be of lutein (thecal or
stromal), Leydig (hilus), and adrenal cortical type.
• Gross- usually unilateral and are composed of yellow or yellowish-brown
nodules separated by fibrous trabeculae.
• Microscopy- characterized by masses of large rounded or polyhedral cells
with the morphologic and ultrastructural features previously described for
their normal counterparts.
• IHC- reactivity for vimentin, keratin, and for actin.
There is also consistent reactivity for inhibin and Melan-A.

92. Two cases of steroid cell tumor showing acidophilic (A) and clear (B)
appearances of the cytoplasm of the tumor cells.

93. GONADOBLASTOMA-
• The most distinctive member of the group of tumors composed of a
combination of germ cells and sex cord–stromal cells is
gonadoblastoma.
• Cells resembling dysgerminoma+ sex cord derivatives
• 1/3rd before 15 yrs; Rt>Lt; 38% B/L
• 90% cases have Y chromosome; virilized phenotypic female; gonadal
dysgenesis
• Calcification in >80% cases
• Germ cell component in nests, sex cord elements in 3 patterns-
coronal, surrounding nests, round spaces containing PAS+ve
material
• Burned out gonadoblastoma indicated by calcified bodies

95. Metastatic Tumors-
• The ovary is a common site of involvement for metastases.
Approximately 7% of lesions presenting clinically as primary ovarian
tumors are of metastatic origin.
• Over half are bilateral.
• The most common sources are stomach, large bowel, appendix, breast,
uterus (corpus and cervix), lung, and skin (melanoma).
• Adenocarcinomas of the large bowel are particularly important
because of their relatively high frequency and their ability to simulate
primary ovarian carcinomas, mainly of the mucinous and
endometrioid types but sometimes also of the clear cell type.

96. A and B, Colonic carcinoma metastatic to ovary. This may be misdiagnosed as a primary
ovarian tumor both grossly and microscopically.

98. KRUKENBERG TUMOR
• An ovarian neoplasm, usually
bilateral and nearly always of
metastatic origin.
• Gross- moderate solid multinodular
enlargement of the ovaries.
• Microscopy- diffuse infiltration by
signet ring cells containing
abundant neutral and acidic (sialo)
mucins.
Tumor emboli are found in over half
of the cases.
Marked stromal proliferation with a
storiform pattern of growth and a
variable degree of luteinization are
common and may obscure the
diagnosis.

99. Krukenberg Tumor of Ovary. A, Microscopic appearance. Numerous
signet ring cells are present in a highly fibrous stroma, either
individually or in small nests. B, Presence of intracellular mucin
evidenced by Meyer mucicarmine stain.

100. SUMMARY