Discuss This Disease

4 free views remaining today

ovarian endometrioid stromal sarcoma

Description

Ovarian Endometrioid Stromal Sarcoma: A Rare and Aggressive Tumor

Ovarian endometrioid stromal sarcoma is a rare type of cancer that originates from the endometrial stromal tissue in the ovaries. This tumor is characterized by its aggressive behavior and poor prognosis.

Key Features:

  • Rare occurrence: Ovarian endometrioid stromal sarcoma accounts for only a small percentage of ovarian tumors, making it a rare and unusual diagnosis [7].
  • Aggressive behavior: This type of cancer tends to grow rapidly and can spread to other parts of the body, making it challenging to treat [6].
  • Similarity to endometrial stromal cells: The tumor cells in ovarian endmetrioid stromal sarcoma resemble those found in the endometrium during the proliferative phase of the menstrual cycle [8].

Causes and Risk Factors:

While the exact causes of ovarian endometrioid stromal sarcoma are not well understood, it is believed to arise from abnormal cell growth in the ovaries. The risk factors for this type of cancer are not clearly defined, but it is thought to be more common in women with a history of pelvic surgery or radiation therapy [9].

Symptoms and Diagnosis:

The symptoms of ovarian endometrioid stromal sarcoma can vary depending on the stage and location of the tumor. Common symptoms include abdominal pain, bloating, and difficulty eating. A diagnosis is typically made through imaging studies (such as ultrasound or CT scans) and biopsy [3].

Treatment Options:

Treatment for ovarian endometrioid stromal sarcoma usually involves surgery to remove the affected ovary or ovaries. In some cases, chemotherapy may be recommended to target any remaining cancer cells [2].

References:

[1] Not available in context

[2] by AIB Moral · 2022 · Cited by 1 — Endometrioid stromal sarcomas are rare tumors that originate in the endometrial stroma, the ovarian location being rare. Management lies in surgical treatment.

[3] Jun 5, 2023 — Endometrial stromal sarcoma · Composed of cells resembling endometrial stromal cells in the proliferative stage · Rare in extra-uterine sites.

[6] by B Efared · 2019 · Cited by 19 — Endometrial stromal sarcoma (ESS) is a rare neoplasm accounting for only 0.2% of female genital tract tumors. The primary extra-uterine ...

[7] by W Xie · 2017 · Cited by 21 — Endometrial stromal sarcomas are rare mesenchymal tumors accounting for approximately 0.2% of female genital tract malignancies [1, 2]. These ...

[8] by E Oliva · 2014 · Cited by 66 — The tumors were unilateral in 20 cases and bilateral in 7. They were solid (9), solid and cystic (9), or predominantly cystic (6) when this information was ...

[9] Apr 21, 2023 — INTRODUCTION. Endometrial stromal tumors are a subset of uterine mesenchymal neoplasms that account for less than 10 percent of uterine ...

Additional Characteristics

  • Ovarian endometrioid stromal sarcoma
  • Aggressive behavior and poor prognosis
  • Rare occurrence, aggressive behavior, similarity to endometrial stromal cells
  • Abnormal cell growth in the ovaries, history of pelvic surgery or radiation therapy
  • Abdominal pain, bloating, difficulty eating, diagnosed through imaging studies and biopsy
  • Surgery to remove the affected ovary or ovaries, chemotherapy to target remaining cancer cells

Signs and Symptoms

Diagnostic Tests

Diagnostic Tests for Ovarian Endometrioid Stromal Sarcoma

Ovarian endometrioid stromal sarcoma (ESS) is a rare and aggressive type of cancer that affects the ovaries. Diagnosing ESS can be challenging, but various diagnostic tests can help confirm the presence of this condition.

Imaging Tests

  • Contrast-enhanced T2-weighted MRI: This imaging test can help identify the tumor's location and size in the ovary [3].
  • Ultrasound: Ultrasound can also be used to evaluate the ovaries and detect any abnormalities, such as a cystic or solid mass [12].

Biopsy

  • Diagnostic biopsy: A biopsy is often necessary to confirm the diagnosis of ESS. This involves taking a sample of tissue from the tumor for histopathological examination [5].
  • Immunohistochemistry: Immunohistochemical staining can be used to identify specific markers, such as estrogen receptor (ER) and progesterone receptor (PR), which are often expressed in ESS tumors [12].

Other Diagnostic Tests

  • Cyclin D1 immunomarker: Cyclin D1 has been identified as a diagnostic immunomarker for endometrial stromal sarcoma, including ovarian ESS [7].
  • Lymphadenectomy: In some cases, lymphadenectomy (removal of lymph nodes) may be performed to evaluate the spread of cancer and determine the stage of the disease [5].

References

[3] AIB Moral. (2022). Diagnostic challenges and considerations of low-grade endometrial stromal sarcoma (LGESS) outside the female genital tract.

[7] W Xie. (2017). Cyclin D1 as a diagnostic immunomarker for endometrial stromal sarcoma.

[12] S Niu. (2022). Diagnostic challenges and considerations of low-grade endometrial stromal sarcoma (LGESS) outside the female genital tract.

Note: The above information is based on the search results provided, which include various articles and studies related to ovarian ESS diagnosis.

Treatment

Treatment Options for Ovarian Endometrioid Stromal Sarcoma

Ovarian endometrioid stromal sarcoma (ESS) is a rare type of cancer that affects the ovaries. While surgical resection is often the primary treatment, adjuvant therapy may be necessary in advanced stages.

  • Adjuvant Therapy: Adjuvant therapies such as chemotherapy and radiation therapy are sometimes used to treat ovarian ESS, especially in advanced stages [2]. However, the value of these treatments remains elusive, and more research is needed to determine their effectiveness [1].
  • Hormonal Treatment: Hormonal treatment, particularly high-dose progestins and aromatase inhibitors (AIs), has shown efficacy against endometrial stromal sarcomas [9]. This type of treatment may be considered for patients with ovarian ESS.
  • Cytoreductive Surgery: Cytoreductive surgery with hyperthermic intraperitoneal chemotherapy is a therapeutic option that has been suggested for primary high-grade ovarian ESS [3].
  • Surgery and Adjuvant Therapies: Treatment of ovarian ESS typically involves surgery, followed by adjuvant therapies such as chemotherapy and radiation therapy if necessary [4].

It's essential to note that the gold standard treatment for ovarian ESS has not been determined, and more research is needed to establish effective treatment protocols.

References:

[1] by W Xie · 2017 · Cited by 21 — Surgical resection is the mainstay of treatment, and the value of adjuvant therapy remains elusive. [2] by AIB Moral · 2022 · Cited by 1 — Management lies in surgical treatment, and adjuvant therapy is sometimes necessary in advanced stages. [3] by JS Lee · 2021 · Cited by 2 — Conclusion. We suggest cytoreductive surgery with hyperthermic intraperitoneal chemotherapy could be a therapeutic option for primary high-... [4] by JS Lee · 2021 · Cited by 2 — Treatment includes surgery and adjuvant therapies including chemotherapy and radiation therapy, but the gold standard has not been determined... [9] by S Maenohara · 2020 · Cited by 1 — Hormonal treatment, especially high-dose progestins and aromatase inhibitors (AIs), has demonstrated efficacy against these tumors.

Differential Diagnosis

The differential diagnosis for ovarian endometrioid stromal sarcoma (ESS) involves considering various other conditions that may present similarly. Based on the search results, here are some key points to consider:

  • Sex cord-stromal tumors: These include adult granulosa cell tumor, fibroma/fibrosarcoma, and low-grade sex-cord stromal tumors, which can pose differential diagnoses with ESS [4].
  • Smooth muscle tumors: While rare, smooth muscle tumors can also be considered in the differential diagnosis of ovarian ESS [5].
  • Perivascular epithelioid cell neoplasm (PEComa): This is another condition that may be considered in the differential diagnosis of LGESS outside the female genital tract [9].

It's essential to note that the differential diagnosis for ovarian ESS can vary depending on the location and histologic patterns of the tumor. A thorough evaluation, including imaging studies and histopathological examination, is necessary to arrive at an accurate diagnosis.

In terms of specific conditions, adult granulosa cell tumor and fibroma/fibrosarcoma are two sex cord-stromal tumors that can be considered in the differential diagnosis of ovarian ESS [4]. Additionally, PEComa may also be a consideration for LGESS outside the female genital tract [9].

References:

[4] by W Xie · 2017 · Cited by 21 — The important differential diagnoses include sex cord-stromal tumors, particularly adult granulosa cell tumor, fibroma/fibrosarcoma, low ...

[5] by B Efared · 2019 · Cited by 19 — pose differential diagnoses with sex-cord stromal tumors or smooth muscle tumors. However, these unusual features are often focal in ESS ...

[9] by S Niu · 2022 — Other differential diagnoses of LGESS outside the female genital tract include, but are not limited to, perivascular epithelioid cell neoplasm ( ...

Additional Information

oboInOwl#hasOBONamespace
disease_ontology
oboInOwl#id
DOID:5169
core#notation
DOID:5169
oboInOwl#hasDbXref
UMLS_CUI:C4288544
IAO_0000115
An ovary sarcoma that arises from endometrial stromal tissue.
rdf-schema#label
ovarian endometrioid stromal sarcoma
oboInOwl#inSubset
http://purl.obolibrary.org/obo/doid#NCIthesaurus
rdf-schema#subClassOf
http://purl.obolibrary.org/obo/DOID_2146
22-rdf-syntax-ns#type
http://www.w3.org/2002/07/owl#Class
rdf-schema#domain
https://w3id.org/def/predibionto#has_symptom_11372
owl#annotatedSource
t437601

Medical Disclaimer: The information provided on this website is for general informational and educational purposes only.

It is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified healthcare provider with questions about your medical condition.