Spindle Cell Lesions of Ovary
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Spindle Cell Lesions of Ovary
Dr Darshan Gohil
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CausesNeoplasticNon-neoplastic
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Neoplastic
Sex-cord stromal neoplasmsa) Fibromab) Thecomac) Granulosa cell tumoursd) Sertoli-Leydig cell tumourse) Rarer neoplasms- sclerosing
stromal tumor and signet-ring stromal tumor
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Otherscellular fibromatous lesionssmooth muscle neoplasmsmetastatic gastrointestinal
stromal tumors
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Non-neoplasticmassive edemaovarian fibromatosisstromal hyperplasia stromal hyperthecosis
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Fibroma The most common type of sex-cord stromal tumor
developing from specialized ovarian stroma Common Usually unilateral,bilateral in 5-10 percent of cases Almost invariably after puberty Fibromas are not hormonally
functionalaverage of 5 cm in diameter Sometimes in young women with basal cell nevus
(Gorlin's) syndrome(17%) Benign May be ascites:
◦ especially if large◦ sometimes with right-sided pleural effusion (Meigs'
syndrome)(disappears on removal of tumor)
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Gross
Solid Lobulated Firm Uniformly white Usually no adhesions Average diameter 6cm May be myxoid changes, sometimes resulting in cystic degeneration
Cut surface of ovarian fibroma.
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Microscopy
Spindle stromal cells: - closely packed - arranged in 'feather-stitched' or storiform pattern.Nuclei are fusiform and uniform.
- no atypia and few mitoses Occasional-nests/tubules of sex cord cells “fibromas with sex cord elements”
Cellular fibroma. The tumor is
hypercellular, but pleomorphism and mitotic activity are
minimal
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Immunohistochemistry and cytogeneticsdiffusely positive for vimentinTrisomy 12 is a constant finding
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ThecomaPeri or post-menopausal women,. symptoms of hyperestrogenism. Most are unilateral and can
measure up to 10 cm in diameter. Endocrine associated symptoms-
irregular bleeding,etcVirilization in patients with
luteinized thecomas
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Gross Usually unilateral Variable size Well-defined capsule Firm consistency Cut surface: * largely or entirely solid * may be cysts Yellow color
Cut surface showing predominant
Yellow areas with white foci
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THECOMA
Fascicles of spindle cells with:
o centrally placed nuclei
o moderate amount of pale
cytoplasm only mild atypia and rare
mitoses Intervening tissue may show:
-considerable collagen
deposition
- focal hyaline plaque formation
Degree of cellularity varies considerably
Some in young women are heavily calcified
Bland microscopic appearance of thecoma, with some variability in cellularity.
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Special Stains and Immunohistochemistry
Oil red O: (require fresh tissue) - abundant intracytoplasmic neutral fat Silver stains: - usually reticulin fibers surrounding
individual cells -may be islands devoid of reticulin,
especially in areas of luteinization Estradiol usually limited to a small
number of tumor cellpositive for inhibin
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Granulosa cell tumoursadult granulosa cell tumourjuvenile granulosa cell tumour
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Adult-GCT The tumors are usually large
(>10 cm) and unilateral. The cut surface is soft and
yellow-tan with cysts and hemorrhage.
encapsulated smooth, lobulated outline Cut surface: -predominantly solid May be: cystic:
◦ -filled with straw-colored or mucoid fluid
-sometimes so prominent
as to simulate appearance of a cystadenoma
Granulosa cell tumor with solid cut surface.
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The microfollicular and diffuse variants often contain characteristic Call–Exner bodies.
Contain a variable amount of fibrous or thecomatous component• Any tumour with
>10% of granulosa
cells is classified as granulosa cell tumour
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Juvenile Granulosa Cell Tumor
Fewer than 5% of granulosa cell tumours
80% during first two decades of life
more aggressive than adult more likely to produce distant metastases
Usually presents with isosexual precocity * associated with: - enchondromatosis (Ollier's disease) - Maffucci's syndrome(enchondromatosis
and multiple subcut. haemangiomas
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Juvenile Granulosa Cell Tumor
Typical morphologic features include:
diffuse or macrofollicular patterns
of growth (former predominating)
- eosinophilic mucin-positive intrafollicular secretion
macrofollicles may be surrounded by rim of spindle shaped thecal cells.
- larger tumor cells with extensive luteinization - nuclear atypia - variable but often high
mitotic activity. Granulosa cells in these
tumours-polygonal to spindle shaped
. On high power the tumor cells lack the coffee-bean nuclei seen in the
adult type
The follicle-like spaces seen on low-power examination are a common feature of this neoplasm.
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ImmunohistochemistryVimentin and inhibin positiveLow molecular weight cytokeratin
+ve in about half casesCD99 membrane stainingNuclear and cytoplasmic staining
for calretinin
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Sertoli leydig cell tumours
• Young patients (average 25 years)• 50% shows signs of androgen excess i.e
defeminisation (breast atrophy, loss of subcut. Fat)
• Later masculinisation appears
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TypesWell differentiated(10%)Intermediate and poorly
differentiatedRetiform
}90%Sertoli cell tumour, NOS
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Sertoli leydig cell tumor
0.1% of ovarian neoplasms Grossly predominantly solid Variegated appearance of cut surface of
ovarian Sertoli–Leydig cell tumor
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Microscopic pattern
Well differentiated
(meyer’s type I)
Tubules lined by sertoli like cells seperated by variable number of leydig like cells
Well-differentiated (Meyer’s type I) Sertoli–Leydig cell tumor.
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Microscopic patterns of SLCT
Intermediate
(meyer’s type II)
Formation of cords, sheets and aggregates of sertoli like cells seperated by spindle stromal cells
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Microscopic patterns of SLCT
Poorly differentiared
(meyer’s type III)
Composed of masses of spindle shaped cells arranged in “sacomatoid” pattern
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Special Stains and Immunohistochemistry of SLCTTestosterone and estradiol both
in sertoli and leydig cellsAreas of sertoli cell differentiation
are Keratin+Gonadal stromal components-
inhibin+
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Sclerosing stromal tumouryounger average age than typical
thecoma or fibromamore than 80% of patients are
younger than 30 years oldPresent with clinical features of
ovarian massestrogenic manifestations-
occasionallyAll the reported tumors have been
unilateral and benign.
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Grosswell-
demarcated, solid white mass with yellow areas.
areas of edema and cyst formation are common
Avg.10 cm in diameter
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Microscopyill-defined
cellular pseudolobules
Two cell types:-a) spindle cells producing collagen, b)round to oval cells with small, dark nuclei
Cellular pseudolobules containing ectatic blood vessels are separated by cellular connective tissue
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Signet ring stromal tuoursRare neoplasm, occurs in adults,
non-functioningStains for lipid and Mucin are negative
On microscopic examination, spindle cells are diffusely distributed and merge with rounded cells containing eccentric nuclei and single large vacuoles resembling signet-ring cells
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Fibromatosis13 to 39 yearsmenstrual abnormalities,
abdominal pain, and, rarely, hirsutism or virilization
Abdominal mass on P/AUsually unilateral
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Fibromatosis
Pathological features: Gross
6 to 12 cm in diameter with smooth, white external surfaces. The cut surfaces are firm,
white, and solid or cystic
dense white tissue surrounding cystic follicles.
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Microscopy:• Dense, hyalinized fibrous
tissue has replaced the normal ovarian stroma and surrounds a primary follicle
• proliferation of spindle cells producing variable amounts of collagen surrounding the follicle
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Massive edema6-33 years of ageabdominal or pelvic painmenstrual irregularitiesUnilateral ovarian
enlargement(90%)Rare patients-Meigs` syndrome
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PathologyGross: enlarged ovaryexternal surface is shinywhite, and smooththe cut surface is homogeneous, soft, exuding a watery fluidMicroscopy:• marked diffuse stromaledema that surrounds folliclesand their derivatives
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Krukenberg tumoursmetastatic carcinomas with a
prominent component of signet-ring cells.
usually originate in the stomachBilateral in 70% of cases
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Gross• solid with a smooth or bosselated
contour.cut surfaces vary from firm, white
to tan and fibroma-like to red, fleshy,and gelatinous. • Necrosis and hemorrhageare common
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Microscopy Rounded malignant epithelial cells, many of which have a
signet-ring-cell appearance, in small nests, cords, tiny
glands or cysts, or single cells
In typically spindle cell
stroma
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Thank u