Endometrial stromal sarcoma (ESS) is a malignant variant of endometrial stromal tumors (ESTs) and encompasses approximately 2-5% of all neoplasms originating in the endometrium, although it is rarely encountered clinical practice [1]. Some authors established an incidence rate of 1-2 per 100,000 women [1], while others claim it to be as low as 1-2 per 1 million women in 1 year [2]. Based on the degree of proliferation, ESSs are either low-grade or high-grade tumors, but all forms penetrate the myometrium of the uterus, which is the main distinguishing feature from benign forms of ESTs (endometrial stromal nodules) [1]. However, some authors claim that all ESSs should be considered as low-grade sarcomas, and the term undifferentiated endometrial sarcomas (UES) should be used to describe high-grade tumors with an incompletely disclosed phenotype instead [2] [3]. They are primarily diagnosed in women between 40-60 years of age, with low-grade tumors being more commonly identified in younger women, whereas postmenopausal females are the primary patient population of high-grade ESS [1] [2] [3] [4] [5]. The clinical presentation is comprised of nonspecific symptoms such as abdominal pain, vaginal bleeding, and progressive metrorrhagia in premenopausal women [3] [4]. A palpable abdominal mass may be detected in up to a quarter of individuals [4].

Despite the rare appearance of endometrial stromal sarcoma, the physician must perform a comprehensive clinical assessment in women who report with undisclosed abdominal pain and vaginal bleeding. A detailed patient history should be obtained first, during which the duration of symptoms and their severity, as well as the menopausal status, need to be assessed. Because a palpable mass was observed in a small, but a significant number of cases, abdominal inspection and palpation must not be overlooked during the physical examination. Imaging studies must be employed as soon as possible. Ultrasonography, despite its benefits in terms of convenience and time consumption, is not a reliable tool for discriminating between uterine lesions, and it is not uncommon for endometrial stromal sarcomas to be misdiagnosed as leiomyomas [1] [2] [3] [4]. For this reason, magnetic resonance imaging (MRI) is the recommended study, as it can confirm the presence of a lesion invading the myometrium and enlargement of the uterus [2]. A definite diagnosis is made after obtaining a viable sample for a histopathological examination with immunohistochemistry and uterine curettage is the crucial procedure that allows proper sampling [1] [2]. Expression of CD10 on immunohistochemistry is a highly specific sign for endometrial stromal sarcomas [2] [6] [7].

Treatment for Endometrial Stromal Sarcoma often involves surgery, specifically a hysterectomy, which is the removal of the uterus. Depending on the stage and spread of the disease, additional treatments such as radiation therapy or hormone therapy may be recommended. Hormone therapy is particularly relevant for ESS because these tumors often express hormone receptors, making them responsive to treatments that block estrogen.

The prognosis for patients with ESS varies depending on the stage at diagnosis and the tumor's grade. Low-grade ESS tends to have a better prognosis and may be slow-growing, while high-grade ESS is more aggressive and may spread more quickly. Early detection and treatment are crucial for improving outcomes. Regular follow-up is important to monitor for any signs of recurrence.

The exact cause of Endometrial Stromal Sarcoma is not well understood. However, like many cancers, it is believed to result from genetic mutations that lead to uncontrolled cell growth. Some studies suggest a potential link to hormonal factors, given the tumor's responsiveness to hormone therapy, but more research is needed to clarify these associations.

Endometrial Stromal Sarcoma is a rare cancer, accounting for a small percentage of all uterine cancers. It is most commonly diagnosed in women between the ages of 40 and 60, although it can occur at any age. Due to its rarity, there is limited data on its exact incidence, but it is considered much less common than other types of uterine cancer, such as endometrial carcinoma.

ESS arises from the stromal cells of the endometrium, which are part of the supportive tissue in the uterine lining. These tumors are characterized by their ability to invade surrounding tissues and, in some cases, metastasize to distant sites. The pathophysiology involves genetic mutations that lead to the transformation of normal stromal cells into malignant ones, resulting in the formation of a tumor.

Currently, there are no specific measures known to prevent Endometrial Stromal Sarcoma due to its unclear etiology. General recommendations for reducing cancer risk include maintaining a healthy lifestyle, such as a balanced diet, regular exercise, and avoiding known carcinogens. Regular gynecological check-ups can help in early detection of any abnormalities.

Endometrial Stromal Sarcoma is a rare uterine cancer originating from the stromal tissue of the endometrium. It presents with symptoms like abnormal bleeding and pelvic pain, and diagnosis is confirmed through imaging and biopsy. Treatment typically involves surgery, with additional therapies depending on the tumor's characteristics. Prognosis varies, with early detection being key to better outcomes. The exact cause is unknown, and prevention strategies are general rather than specific.

For patients diagnosed with Endometrial Stromal Sarcoma, understanding the condition is crucial. ESS is a rare cancer of the uterus that can cause symptoms like unusual bleeding and pelvic discomfort. Diagnosis involves imaging and tissue sampling, and treatment often includes surgery. The outlook depends on the tumor's stage and grade, with early treatment offering the best chance for a positive outcome. Regular follow-up care is important to monitor health and manage any potential recurrence.

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