Spindle Cell Lesions of Ovary

Dr Darshan Gohil

Causes
Neoplastic y Non-neoplastic
y

Neoplastic
Sex-cord stromal neoplasms a) Fibroma b) Thecoma c) Granulosa cell tumours d) Sertoli-Leydig cell tumours e) Rarer neoplasms- sclerosing stromal tumor and signet-ring stromal tumor

Others
cellular fibromatous lesions y smooth muscle neoplasms y metastatic gastrointestinal stromal tumors
y

NonNon-neoplastic
massive edema y ovarian fibromatosis y stromal hyperplasia y stromal hyperthecosis
y

Fibroma
y y y y

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 functional

y
y y y

average 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 'featherstitched' 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 cytogenetics

diffusely positive for vimentin y Trisomy 12 is a constant finding
y

Thecoma
Peri or post-menopausal women,. y symptoms of hyperestrogenism. y Most are unilateral and can measure up to 10 cm in diameter. y Endocrine associated symptoms-irregular bleeding,etc y Virilization in patients with luteinized thecomas
y

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
y

Fascicles of spindle cells with: o centrally placed nuclei o moderate amount of pale cytoplasm


y

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

y y

Bland microscopic appearance of thecoma, with some variability in cellularity.

Special Stains and Immunohistochemistry

Oil red O: (require fresh tissue) - abundant intracytoplasmic neutral fat y Silver stains: - usually reticulin fibers surrounding individual cells -may be islands devoid of reticulin, especially in areas of luteinization y Estradiol usually limited to a small number of tumor cell y positive for inhibin
y

Granulosa cell tumours

adult granulosa cell tumour y juvenile granulosa cell tumour
y

AdultAdult-GCT
y y y y y y

The tumors are usually large (>10 cm) and unilateral. The cut surface is soft and yellowtan 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. y Contain a variable amount of fibrous or thecomatous component Any tumour with >10% of granulosa cells is classified as granulosa cell tumour
y

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 tumourspolygonal to spindle shaped
y

The follicle-like spaces seen on low-power examination are a common feature of this neoplasm.

. On high power the tumor cells lack the coffeebean nuclei seen in the adult type

Immunohistochemistry
Vimentin and inhibin positive y Low molecular weight cytokeratin +ve in about half cases y CD99 membrane staining y Nuclear and cytoplasmic staining for calretinin
y

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

Types
Well differentiated(10%) y Intermediate and poorly differentiated y Retiform }90% y Sertoli cell tumour, NOS
y

Sertoli leydig cell tumor

0.1% of ovarian neoplasms y Grossly predominantly solid y Variegated appearance of cut surface of ovarian SertoliLeydig cell tumor
y

Microscopic pattern
y

Well differentiated (meyers type I) Tubules lined by sertoli like cells seperated by variable number of leydig like cells
y

Well-differentiated (Meyers type I) SertoliLeydig cell tumor.

Microscopic patterns of SLCT

Intermediate (meyers type II)
y

Formation of cords, sheets and aggregates of sertoli like cells seperated by spindle stromal cells

Microscopic patterns of SLCT

Poorly differentiared (meyers type III) Composed of masses of spindle shaped cells arranged in sacomatoid pattern

Special Stains and Immunohistochemistry of SLCT

Testosterone and estradiol both in sertoli and leydig cells y Areas of sertoli cell differentiation are Keratin+ y Gonadal stromal components- inhibin+
y

Sclerosing stromal tumour

younger average age than typical thecoma or fibroma y more than 80% of patients are younger than 30 years old y Present with clinical features of ovarian mass y estrogenic manifestations-occasionally y All the reported tumors have been unilateral and benign.
y

Gross
well-demarcated, solid white mass with yellow areas. y areas of edema and cyst formation are common y Avg.10 cm in diameter
y

Microscopy
ill-defined cellular pseudolobules y Two cell types:-a) spindle cells producing collagen, b)round to oval cells with small, dark nuclei
y

Cellular pseudolobules containing ectatic blood vessels are separated by cellular connective tissue

Signet ring stromal tuours

Rare neoplasm, occurs in adults, nonfunctioning y Stains for lipid and Mucin are negative
y

On microscopic examination, spindle cells are diffusely distributed and merge with rounded cells containing eccentric nuclei and single large vacuoles resembling signet-ring cells

Fibromatosis
13 to 39 years y menstrual abnormalities, abdominal pain, and, rarely, hirsutism or virilization y Abdominal mass on P/A y Usually unilateral
y

Fibromatosis
Pathological features: Gross y 6 to 12 cm in diameter with smooth, white external surfaces. The cut surfaces are firm, dense white tissue surrounding cystic follicles. y white, and solid or cystic
y

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 edema
6-33 years of age y abdominal or pelvic pain y menstrual irregularities y Unilateral ovarian enlargement(90%) y Rare patients-Meigs` syndrome
y

Pathology
Gross: enlarged ovary y external surface is shiny white, and smooth the cut surface is homogeneous, soft, exuding a watery fluid Microscopy: marked diffuse stromal edema that surrounds follicles and their derivatives
y

Krukenberg tumours
metastatic carcinomas with a prominent component of signet-ring cells. y usually originate in the stomach y Bilateral in 70% of cases
y

Gross
solid with a smooth or bosselated contour. y cut surfaces vary from firm, white to tan and fibroma-like to red, fleshy, and gelatinous. Necrosis and hemorrhage are common

Microscopy
y

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

Thank u