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Sex Cord Stromal Ovary Tumor Pathology

Sex Cord-Stromal Tumors

Definition

Sex cord-stromal tumors are groups of tumors composed of granulosa cells, theca cells, Sertoli cells, Leydig cells, and fibroblasts of stromal origin, singly or in various combinations.
 These tumors have variable clinicopathologic fetaures and biologic behavior, which may pose a diagnostic challenge.

According to the World Health Organization (WHO),
sex cord-stromal tumors are classified into the following categories.

Granulosa-stromal cell tumors

Granulosa cell tumor group

Adult

Juvenile

Thecoma-fibroma group

Thecoma, not otherwise specified

Typical

Luteinized

Fibroma

Cellular fibroma

Fibrosarcoma

Stromal tumor with minor sex cord elements

Sclerosing stromal tumor

Signet ring cell stromal tumor

Unclassified (fibrothecoma)

Sertoli-stromal cell tumors

Sertoli-Leydig cell tumors group (androblastomas)

Well differentiated

Of intermediate differentiation

Variant with heterologous elements

Poorly differentiated (sarcomatoid)

Variant with heterologous elements

Retiform

Variant with heterologous elements

Sertoli cell tumors

Stromal-Leydig cell tumor

Sex cord-stromal tumors of mixed or unclassified cell types

Sex cord tumor with annular tubules

Gynandroblastoma

Sex cord-stromal tumor, unclassified

Steroid cell tumors

Stromal luteoma

Leydig cell tumor group: Hilus cell tumor; Leydig cell tumors nonhilar type; Leydig cell tumors, not otherwise specified

Steroid cell tumors, not otherwise specified: Well differentiated; malignant

Epidemiology

Sex cord-stromal tumors account for approximately 8% of all ovarian tumors.

Immunohistochemistry

Although various markers have been reported to stain sex cord-stromal tumors (eg, CD99, CD56, A103, müllerian inhibiting factor, vimentin), inhibin and calretinin have proven to be the most helpful to date. These tumors can also be positive for cytokeratin (CAM 5.2, AE1/AE3), CD10, ER, PR, smooth muscle actin, desmin, S100 protein, and WT-1.

CD56 is a sensitive marker of ovarian sex cord-stromal tumors and may also be useful in the diagnosis of this group of neoplasms, especially in cases that are inhibin or calretinin negative, and the differential diagnosis includes neoplasms that are CD56 negative.

Tumor spread and staging

The International Federation of Gynecology and Obstetrics (FIGO) staging of ovarian tumors is outline below.

I. Growth limited to the ovaries

A: Growth limited to one ovary; no ascites. No tumor on the external surface; capsule intact.

B: Growth limited to both ovaries; no ascites. No tumor on the external surface; capsule intact.

C: Tumor either Stage IA or Stage IB, but with tumor on the surface of one or both ovaries, or with capsule ruptured, or with ascites containing malignant cells or with positive peritoneal washings.

II. Growth involving one or both ovaries with pelvic extension

A: Extension and/or metastases to the uterus and/or tubes

B: Extension to other pelvic tissues

C: Tumor is either Stage IIA or IIB but with tumor on the surface of one or both ovaries; or with capsule ruptured; or ascites present containing malignant cells or with positive peritoneal washings.

III. Tumor involving one or both ovaries with peritoneal implants outside the pelvis and/or positive retroperitoneal or inguinal nodes. Superficial liver metastasis equals Stage III.

A. Tumor grossly limited to the true pelvis with negative nodes but with histologically confirmed microscopic seeding of abdominal peritoneal surfaces.

B. Tumor of one or both ovaries with histologically confirmed implants of abdominal peritoneal surfaces, none exceeding 2 cm in diameter. Nodes are negative.

C. Abdominal implants more than 2 cm in diameter and/or positive retroperitoneal or inguinal nodes.

IV. Growth involving one or both ovaries with distant metastasis.

If pleural effusion is present, positive cytology must be present to allot a case to stage IV. Parenchymal liver metastasis equals stage IV.

A worldwide database analysis revealed that younger age, smaller tumor size, early stage, and granulosa cell tumor histologic type appear to be independent prognostic factors for improved survival in patients with malignant sex cord-stromal tumors who undergo lymph node dissection.
The investigators indicated that stratified log odds of positive lymph nodes (LODDS) may be useful for evaluating patients’ lymph node status as well as for prediction of patient survival status.

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