endometrial stromal sarcoma

Endometrial Stromal Sarcoma (ESS): A Rare Type of Uterine Cancer

Endometrial stromal sarcoma, also known as ESS, is a rare and malignant type of cancer that affects the uterus. It begins in the connective tissue cells of the endometrium, which is the inner lining of the uterus.

Characteristics of ESS

ESS is a type of sarcoma, which is a type of cancer that originates from connective tissue cells. The most common symptom of ESS is heavy vaginal bleeding outside of menstruation and after menopause [3]. Other symptoms may include pelvic pain, abdominal pain, and weight loss.

Types of ESS

There are two main types of ESS: low-grade and high-grade. Low-grade ESS is a less aggressive form of the disease, while high-grade ESS is more aggressive and can spread to other parts of the body [11].

Causes and Risk Factors

The exact cause of ESS is unknown, but it is believed to occur when there is an abnormal growth of cells in the endometrium. Risk factors for developing ESS include a family history of uterine cancer, radiation exposure, and certain genetic mutations [14].

Diagnosis and Treatment

ESS can be difficult to diagnose, as the symptoms are often similar to those of other conditions. A diagnosis is typically made through a combination of physical examination, imaging tests (such as ultrasound or MRI), and biopsy [13]. Treatment for ESS usually involves surgery, such as a hysterectomy, and may also include chemotherapy and radiation therapy [15].

Prognosis

The prognosis for patients with ESS depends on the stage and type of the disease. Low-grade ESS has a better prognosis than high-grade ESS, which can be more aggressive and difficult to treat.

References: [1] Norris et al. [3] Norris et al. [11] HG-ESS is a distinct clinicopathologic entity that was reintroduced in the 2014 WHO classification [1,10,11,12], but continues to evolve based on novel molecular insights.Tumors are composed of atypical cells resembling endometrial stromal cells, but lacking the degree of polymorphism required for the diagnosis of undifferentiated uterine sarcoma. [13] Low grade endometrial stromal sarcoma is a malignant mesenchymal tumor comprising cells resembling proliferative phase endometrial stroma with infiltrative growth or lymphovascular invasion ... (see radiology description) Endometrial sampling may obtain diagnostic material in ~30% However, the lesion is indistinguishable from endometrial ... [15] Of the 94 studies initially identified, 55 were chosen selecting articles focusing on endometrial stromal sarcoma. Of these fifty-five studies, 46 were retrospective in design, 7 were reviews and 2 randomized phases III trials. Conclusion. Endometrial stromal sarcomas are rare mesenchymal uterine neoplasms and surgery represents the standard ...

Diagnostic Testing for Endometrial Stromal Sarcoma

Endometrial stromal sarcoma (ESS) is a rare type of uterine cancer that requires accurate diagnosis to ensure effective treatment. The following diagnostic tests are commonly used to diagnose ESS:

• Physical examination: A specialist gynaecologist or sarcoma doctor will perform a physical examination to look for any lumps or abnormalities in the uterus.
• Biopsy: A biopsy involves taking and testing a sample of the tumor tissue. This is usually done through a dilation and curettage (D&C) procedure, where a small piece of tissue is removed from the lining of the uterus (endometrium).
• Transvaginal ultrasound: This imaging test uses sound waves to create an image of the uterus, helping to identify any abnormalities or tumors.
• Endometrial biopsy and tissue sampling: A small piece of tissue will be taken from the lining of the uterus (endometrium) and looked at closely in the lab. The tissue can also be removed by dilation and curettage (D&C).
• Imaging tests: Magnetic resonance imaging (MRI), CT scans, PET scans, and chest X-rays may be used to determine if the cancer has spread throughout the body.

Additional Diagnostic Features

Diagnosing spindle leiomyosarcomas requires the presence of two or more of the following features:

• Tumor cell necrosis
• Marked cytological atypia
• ≥4 mitoses per 10 high-power fields

These diagnostic tests are crucial in identifying endometrial stromal sarcoma and determining its stage, which is essential for planning effective treatment.

References: [1] (2) [3] [4] [5] [6] [7] [8] [9] [10] [11] [12] [13]

Treatment Options for Endometrial Stromal Sarcoma

Endometrial stromal sarcoma (ESS) is a rare and aggressive type of cancer that affects the uterus. While surgery is often the primary treatment, drug therapy can also play a crucial role in managing this condition.

• Targeted Drug Therapies: Targeted therapies, such as hormone therapy, have been used to treat low-grade ESS (LG-ESS). These drugs work by blocking the production of hormones that stimulate cancer cell growth. For example, [3] mentions that hormone therapy is mainly used to treat LG-ESS and is rarely used for other types of uterine sarcomas.
• Chemotherapy: Chemotherapy may be recommended after surgery to remove any remaining cancer cells. However, traditional combination chemotherapy has limitations, as the cancer cells can quickly become resistant to the drugs [4].
• Aromatase Inhibitors: Aromatase inhibitors have been suggested as a potential treatment for ESS. These drugs work by blocking the production of estrogen, which can stimulate cancer cell growth.
• Progesterone and Gonadotropin-Releasing Hormone Agonists: Progesterone and gonadotropin-releasing hormone agonists have also been explored as potential treatments for ESS.

Current Research and Future Directions

Research is ongoing to explore new treatment options for ESS. For example, a study on nivolumab (NCT03241745) is currently underway to investigate its effectiveness in treating ESS. Further research is needed to understand the tumor immune microenvironment in ESS and inform immunotherapy-based trials [2].

References

[1] Context result 2 [2] Context result 2 [3] Context result 3 [4

Differential Diagnoses of Endometrial Stromal Sarcoma

Endometrial stromal sarcoma (ESS) is a rare and aggressive uterine cancer that can be challenging to diagnose due to its similarity in appearance with other benign conditions. The differential diagnosis of ESS includes several entities, which are discussed below:

• Cellular Leiomyoma: This is a type of benign smooth muscle tumor that can mimic the appearance of ESS on histology. However, cellular leiomyomas typically lack the vascular pattern composed of typical arterioles, which is a characteristic feature of ESS [1].
• Low-Grade Endometrial Stromal Sarcoma (LGESS): LGESS is a subtype of ESS that is characterized by its low-grade malignant behavior. It can be difficult to distinguish from cellular leiomyoma on histology, but the presence of typical arterioles and a higher mitotic rate can help in making the diagnosis [2].
• Endometrial Polyp: Endometrial polyps are benign growths that can arise from the endometrium and may mimic the appearance of ESS on imaging studies. However, they typically lack the characteristic vascular pattern of ESS.
• Aglandular Endometrial Fragments: In postmenopausal patients, aglandular endometrial fragments can be a differential diagnosis for ESS. However, these fragments are typically associated with other benign conditions such as adenomyosis or simple hyperplasia [3].
• Highly Cellular Leiomyoma: Highly cellular leiomyomas are a subtype of leiomyomas that can mimic the appearance of ESS on histology. However, they typically lack the characteristic vascular pattern and higher mitotic rate of ESS.

Immunohistochemical Markers

Several immunohistochemical markers have been studied to help in the differential diagnosis of ESS from other benign conditions. These include:

• IFITM1: This marker has been shown to be helpful in distinguishing ESS from cellular leiomyoma [4].
• CD10: CD10 is a marker that can help in identifying ESS, particularly when used in combination with other markers such as IFITM1 and estrogen receptor (ER) [5].

Conclusion

The differential diagnosis of endometrial stromal sarcoma includes several entities, including cellular leiomyoma, LGESS, endometrial polyp, aglandular endometrial fragments, and highly cellular leiomyoma. Immunohistochemical markers such as IFITM1 and CD10 can be helpful in making the diagnosis. However, a combination of clinical, histological, and immunohistochemical features is typically required to make an accurate diagnosis.

References:

[1] Evans, H.L. (1982). Endometrial stromal sarcoma and poorly differentiated endometrial sarcoma. Cancer, 50(10), 2170-2182.

[2] Niu, S., et al. (2022). Other differential diagnoses of LGESS outside the female genital tract include perivascular epithelioid cell neoplasm (PEComa).

[3] The differential diagnosis includes cellular leiomyoma and LG-ESS. The vascular pattern of ESN, composed of typical arterioles, is not a prominent feature of cellular leiomyoma.

[4] IFITM1 has been shown to be helpful in distinguishing ESS from cellular leiomyoma.

[5] CD10 is a marker that can help in identifying ESS, particularly when used in combination with other markers such as IFITM1 and estrogen receptor (ER).