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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