Transcript
Spindle Cell Lesions of Ovary
Dr Darshan Gohil
CausesNeoplasticNon-neoplastic
Neoplastic
Sex-cord stromal neoplasmsa) Fibromab) Thecomac) Granulosa cell tumoursd) Sertoli-Leydig cell tumourse) Rarer neoplasms- sclerosing
stromal tumor and signet-ring stromal tumor
Otherscellular fibromatous lesionssmooth muscle neoplasmsmetastatic gastrointestinal
stromal tumors
Non-neoplasticmassive edemaovarian fibromatosisstromal hyperplasia stromal hyperthecosis
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)
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.
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
Immunohistochemistry and cytogeneticsdiffusely positive for vimentinTrisomy 12 is a constant finding
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
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
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.
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
Granulosa cell tumoursadult granulosa cell tumourjuvenile granulosa cell tumour
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.
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
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
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.
ImmunohistochemistryVimentin and inhibin positiveLow molecular weight cytokeratin
+ve in about half casesCD99 membrane stainingNuclear and cytoplasmic staining
for calretinin
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
TypesWell differentiated(10%)Intermediate and poorly
differentiatedRetiform
}90%Sertoli cell tumour, NOS
Sertoli leydig cell tumor
0.1% of ovarian neoplasms Grossly predominantly solid Variegated appearance of cut surface of
ovarian Sertoli–Leydig cell tumor
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.
Microscopic patterns of SLCT
Intermediate
(meyer’s type II)
Formation of cords, sheets and aggregates of sertoli like cells seperated by spindle stromal cells
Microscopic patterns of SLCT
Poorly differentiared
(meyer’s type III)
Composed of masses of spindle shaped cells arranged in “sacomatoid” pattern
Special Stains and Immunohistochemistry of SLCTTestosterone and estradiol both
in sertoli and leydig cellsAreas of sertoli cell differentiation
are Keratin+Gonadal stromal components-
inhibin+
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.
Grosswell-
demarcated, solid white mass with yellow areas.
areas of edema and cyst formation are common
Avg.10 cm in diameter
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
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
Fibromatosis13 to 39 yearsmenstrual abnormalities,
abdominal pain, and, rarely, hirsutism or virilization
Abdominal mass on P/AUsually unilateral
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.
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
Massive edema6-33 years of ageabdominal or pelvic painmenstrual irregularitiesUnilateral ovarian
enlargement(90%)Rare patients-Meigs` syndrome
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
Krukenberg tumoursmetastatic carcinomas with a
prominent component of signet-ring cells.
usually originate in the stomachBilateral in 70% of cases
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
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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