Sex cord stromal tumor

Sex cord-stromal tumor is a group of ovarian tumors that derive from the sex cords of the embryonic gonads and the ovarian stroma. These tumors can produce sex steroids leading to various clinical manifestations. They are part of a broader category of ovarian neoplasms and represent approximately 5% to 8% of all ovarian tumors. The classification of sex cord-stromal tumors includes several types, with the most common being granulosa cell tumors and theca cell tumors, among others.

Classification

Sex cord-stromal tumors are classified based on their cell of origin and histological characteristics. Major types include:

• Granulosa cell tumor: Divided into adult and juvenile types, these tumors are characterized by their potential to secrete estrogen.
• Sertoli-Leydig cell tumor: Also known as androblastoma, these tumors may produce androgens leading to virilization.
• Thecoma: These tumors are usually benign and often produce estrogen, which can cause endometrial hyperplasia or even endometrial cancer.
• Fibroma: A benign tumor that does not produce hormones.

Symptoms

The symptoms of sex cord-stromal tumors vary depending on the type of tumor and the hormones they produce. Common symptoms include:

• Abnormal uterine bleeding
• Abdominal pain or a mass
• Signs of estrogen or androgen excess
• Postmenopausal bleeding
• Early puberty in prepubescent individuals

Diagnosis

Diagnosis of sex cord-stromal tumors involves a combination of clinical evaluation, imaging studies such as ultrasound or MRI, and histopathological examination of the tumor. Serum levels of inhibin A, inhibin B, and anti-Müllerian hormone (AMH) may also be useful markers for these tumors.

Treatment

Treatment options for sex cord-stromal tumors depend on the type and stage of the tumor, as well as the patient's desire for fertility preservation. Surgical removal of the tumor is the primary treatment, which may include unilateral oophorectomy or salpingo-oophorectomy. In advanced cases, a total hysterectomy with bilateral salpingo-oophorectomy may be necessary. Adjuvant therapy, including chemotherapy or radiation therapy, may be considered in certain cases.

Prognosis

The prognosis for patients with sex cord-stromal tumors varies depending on the specific type of tumor and stage at diagnosis. Generally, these tumors have a relatively favorable prognosis compared to other ovarian cancers, especially when detected early and treated appropriately.

Epidemiology

Sex cord-stromal tumors are rare, accounting for a small percentage of all ovarian tumors. They can occur at any age but are more commonly diagnosed in middle-aged and older women. The exact incidence rate varies by tumor type.