What are the classifications of ovarian sex cord stromal tumors?

What are the classifications of ovarian sex cord stromal tumors?

Ovarian sex cord stromal tumors are manifested by changes in the patient's endocrine function, and the types are also different. For example, they may show symptoms of granulosa cell tumor, theca cell tumor, fibroid tumor, etc. Therefore, it is important to focus on treatment.

Ovarian sex cord-stromal tumors originate from the sex cords and stromal tissues in the primitive gonads, and evolve into different types of cells in males and females, respectively, and form a certain tissue structure. The sex cord interstitial cells of females are called granulose cells and theca cells, while those of males are called Sertoli cells and Leydig cells. They can each form granulose cell tumors and theca cell tumors in females, or Sertoli cell tumors and Leydig cell tumors in males. It can also be mixed to form granulosa-thecoma cell tumor or supporting-lesion cell tumor. Because sex cord stroma can differentiate in multiple directions, tumors derived from all of these cell types can be found in the ovary and testis. The theca cells and interstitial cells can produce estrogen and androgen, respectively, and patients often have changes in endocrine function.

It is composed of granulosa cells of the sex cord, derived components of the interstitium, fibroblasts and theca cells. It can be a single component or two tissue components coexist, and the degree of differentiation is also different.

(1) Granular cell tumor is the most common functional tumor. It is a low-grade malignant tumor and is most common in women around 50 years old. Because it can secrete estrogen, it has a feminizing effect. Pseudo-precocious puberty may occur before puberty, menstrual disorders during childbearing age, and endometrial hyperplasia and even adenocarcinoma in postmenopausal women. The tumors are mostly unilateral, of varying sizes, with a smooth or lobed surface, a solid cross-section, and half of them are cystic. The prognosis is generally good. The 5-year survival rate is about 80%. In a few cases, recurrence may occur many years after treatment, so long-term follow-up is necessary.

(2) The incidence of theca cell tumor is half of that of granulosa cell tumor. It is basically benign, but 2-5% is malignant. It usually occurs after menopause and is rare before the age of 40. The tumor is unilateral, of varying sizes, generally medium in size, hard, with a smooth surface, solid cut surface, and off-white color, typically with yellow lipid areas. This tumor can secrete more estrogen, so the feminization symptoms are more significant than those of granulosa cell tumors. It is often accompanied by endometrial hyperplasia and even endometrial cancer. Malignant thecoma can directly infiltrate adjacent tissues and may metastasize to distant sites, but the prognosis is still better than that of general ovarian cancer.

(3) Fibroids are the most common solid ovarian tumors, accounting for 2-5% of ovarian tumors. They are benign tumors and are more common in middle-aged women. The tumors are mostly unilateral, medium in size, smooth or nodular in surface, grayish white in cross section, solid, and extremely hard. Occasionally, fibroma is accompanied by ascites or pleural effusion, which is called Meig's syndrome. The ascites and pleural effusion disappear on their own after surgical resection. Ascites seeps into the chest cavity through the lymphatic pathway or the diaphragmatic channel. The right side of the diaphragm is rich in lymph, so pleural effusion often occurs on the right side.

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