Endometrial Stromal Sarcoma

Endometrial Stromal Sarcoma (ESS) is a rare type of uterine cancer that arises from the stromal (connective tissue) component of the endometrium, the lining of the uterus. This malignancy represents a small fraction of all uterine sarcomas, which themselves are uncommon when compared to endometrial carcinoma, the most frequent type of uterine cancer. ESS is characterized by its origin in the endometrial stroma, its unique histological features, and its clinical behavior, which can range from indolent to aggressive.

Classification

ESS is classified into low-grade and high-grade categories, based on histopathological characteristics and genetic findings.

• Low-grade Endometrial Stromal Sarcoma (LG-ESS): This form is characterized by its slow growth and tendency to recur locally. It often exhibits the t(7;17)(p15;q21) translocation, resulting in the JAZF1-SUZ12 fusion gene.
• High-grade Endometrial Stromal Sarcoma (HG-ESS): HG-ESS is more aggressive, with a higher rate of distant metastases. It lacks the specific genetic alterations seen in LG-ESS and is defined by its high mitotic rate and atypical cellular morphology.

Symptoms

Symptoms of ESS may include abnormal vaginal bleeding, pelvic pain or pressure, and an enlarged uterus palpable upon physical examination. However, these symptoms are not specific to ESS and can be associated with a variety of gynecological conditions.

Diagnosis

The diagnosis of ESS is primarily based on histological examination of the tumor. Imaging studies such as ultrasound, magnetic resonance imaging (MRI), and computed tomography (CT) scan can help in assessing the extent of the disease but cannot definitively diagnose ESS. A biopsy or surgical resection of the tumor is necessary for histopathological evaluation.

Treatment

Treatment options for ESS depend on the stage of the disease, the grade of the tumor, and the patient's overall health. Surgery is the mainstay of treatment, aiming to remove the tumor and affected tissues. For LG-ESS, complete surgical resection is often curative. However, HG-ESS may require additional treatments, including radiation therapy, chemotherapy, and hormonal therapy, due to its aggressive nature and higher risk of metastasis.

Prognosis

The prognosis for patients with ESS varies depending on the grade and stage of the tumor at diagnosis. Patients with LG-ESS generally have a favorable prognosis with a high 5-year survival rate, especially when the disease is confined to the uterus and completely resected. In contrast, HG-ESS has a poorer prognosis due to its aggressive behavior and tendency to metastasize.

Epidemiology

ESS is a rare entity, accounting for less than 10% of all uterine sarcomas. It predominantly affects middle-aged women, but cases have been reported across a wide age range.

Research Directions

Ongoing research in the field of ESS is focused on understanding the molecular and genetic underpinnings of the disease, which could lead to the development of targeted therapies. The identification of specific genetic alterations in ESS has opened new avenues for targeted treatments, although these are still in the experimental phase.

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