Sex cord-stromal neoplasms


Sex cord-stromal neoplasms

Description, Causes and Risk Factors:

Sex cord-stromal neoplasms account for ~10% of all ovarian neoplasms. They are composed of various cell types derived from gonadal stroma and sex cords. They account for most of the hormonally active ovarian tumors. Granulosa cell tumors and Sertoli-Leydig cell tumors are the most common. Unlike patients with common epithelial tumors, in which 75% are considered to be at stage III or IV at diagnosis, patients with these tumors are at stage I at diagnosis 70% of the time. Also unlike common epithelial tumors, sex cord-stromal tumors often have more specific symptoms. Granulosa cell tumors, which are most common in postmenopausal women, may cause vaginal bleeding and an elevated level of the tumor marker inhibin in the blood. Sertoli-Leydig cell tumors are rare; the average age of patients diagnosed with these tumors is 25, and only 10% of patients are over 50. About 33% of these tumors produce signs of virilism (infrequent menstrual periods, cessation of menstrual periods before menopause, hoarse voice, and appearance of facial hair).

Classification of sex cord-stromal neoplasms:

    Granulosa cell tumor.

  • Thecoma.

  • Sertoli-Leydig cell tumor.

  • Sertoli cell tumor.

  • Leydig cell tumor.

  • Fibroma.

  • Fibrosarcoma.

  • Sclerosing stromal tumor.

The morphology of these tumours varies, depending on the cell type or mixture of cell types present, and can range from entirely glandular as in well-differentiated Sertoli cell tumors to entirely spindled as in cellular fibromas. This varied appearance and the fact that some of these tumours are relatively uncommon, can lead to difficulties in diagnosis. In the great majority of cases the patterns are distinctive in routinely stained sections and as in ovarian tumour pathology in general, the importance of thorough sampling cannot be over-emphasized.

No definite etiologies have been established for granulosa cell tumours, although chromosomal abnormalities and abnormal autocrine and endocrine signalling have been suggested. All ovarian sex cord-stromal tumours are derived from the stroma of the developing ovary. The gonadal stoma is primitive, and consequently can develop in a testicular or ovarian differentiation pathway. The frequency of sex cord-stromal tumours is similar throughout the world. There does not appear to a racial predisposition, in contrast to epithelial ovarian cancers. Every year, 15,000 to 20,000 new cases of sex cord-stromal tumours are diagnosed in the USA. Sex cord-stromal tumours appear in any age group but usually in the 4th and 5th decades. Fibromas are usually detected in the fifth decade of life. The mean age of presentation of Leydig cell tumours is 50. The median age of diagnosis of adult granulosa cell tumours is 52, and is 53-years for theca cell tumours. Theca cell tumours account for 1% of ovarian neoplasms, and are rarely diagnosed in women under 30-years of age, unless they have luteinized thecoma which is more apparent in younger women. However, Sertoli-Leydig cell tumours tend to present at a younger age, usually in the third decade of life.

Granulosa cell tumours usually follow a nonaggressive clinical course. However, they may become malignant or recur (up to 30 years after the initial diagnosis). 65% of granulosa cell tumours occur in postmenopausal females. Juvenile and adult granulosa cell tumours, fibromas, and Sertoli-Leydig cell tumours are usually unilateral. Granulosa-theca cell tumours are usually large and benign, with cystic degeneration. Intra-abdominal bleeding following rupture is often the presenting symptom of patients with granulosa cell tumour. Both adult and juvenile granulosa cell tumours are indolent. Approximately 10% of granulosa cell tumours occur in pregnant patients, and should be surgically removed at 16-18 weeks of gestation.

Symptoms:

Symptoms of sex cord-stromal tumors vary depending on the type of tumor. Symptoms may include:

    Back pain.

  • Abdominal swelling or bloating.

  • Feeling full.

  • Fatigue.

  • Pelvic or abdominal pain.

  • Constipation.

  • Upset stomach.

  • Frequent need to urinate.

  • Menstrual irregularities.

  • Pain during sex.

Diagnosis:

An exact diagnosis can only be made with a biopsy (the removal of cell groups and/or tissues). A histological examination is performed following the biopsy, which is also critical in securing a proper diagnosis and a positive prognosis.

Immunohistochemical studies may be usefulin those situations when routine microscopic findingsfail to indicate a clear diagnosis. Most ovarian sexcord stromal tumours produce steroid hormones. Thediagnosis should, therefore, be suspected in patients,who present with signs of estrogen excess (precociouspuberty in a child, abnormal uterine bleeding,endometrial hyperplasia/carcinoma), or androgenexcess (virilization), especially if an adnexal mass ispresent. These tumours secreteproteins, such as inhibin (One of several proteins that participate in differentiation and growth. Two glycoproteins, inhibin A and inhibin B, are secreted by Sertoli cells in the testis and granulosa cells in the ovary, inhibiting FSH secretion by direct action on the pituitary) which can serve as diagnosticmarkers. Consideration of these characteristics, helpin clinically distinguishing sex cord-stromal tumoursfrom germ cell ovarian tumours, the more commonepithelial ovarian cancer and the rare small cellcarcinoma of the ovary.

Treatment:

The removal of sex cord-stromal tumors is conducted through surgery. The type of surgery varies, usually depending on age; for example, young women may be treated through a bilateral salpingo-oophorectomy (the removal of the fallopian tubes and ovaries). In older women, treatment can also include a complete hysterectomy (the removal of the uterus). Localized tumors may also be treated with chemotherapy and radiation.

Prognosis is typically positive. Depending on the type of tumor, survival rates are between 90 and 100% in early stages. The mortality rate for patients with juvenile granulosa cell tumors, for example, is less than 2%.

As with most cancers, survival rates drastically decrease with late-stage diagnoses. Patients with advanced stages of granulosa cell tumours, for instance, have only a 25 to 50% 5-year survival rate.

NOTE: The above information is for processing purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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