Materiały źródłowe

• #1 Epithelioid Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK532911/

  Epithelioid sarcoma is a rare and aggressive soft tissue sarcoma characterized by both epithelial and mesenchymal differentiation. […] Diagnosis is typically made through tissue biopsy, while imaging studies are essential for assessing the extent of the primary tumor and identifying metastatic disease. […] A tissue biopsy is necessary, as soft tissue swellings have many possible causes. Core needle biopsies are the preferred method. […] Epithelioid sarcoma is associated with a high rate of distant metastases, with recent studies suggesting a distant metastasis rate of up to 50%. […] Diagnosing epithelioid sarcoma can be challenging due to its mixed mesenchymal and epithelial differentiation. […] The most accurate diagnosis can be made by considering the appropriate clinical context, such as the presence of a soft tissue mass on the extremity of a young male, with immunohistochemical staining providing diagnostic support.

• #2 Epithelioid Sarcoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2924131/

  Epithelioid sarcoma is a rare, high-grade, soft tissue tumor that has a known propensity for local recurrence, regional lymph node involvement, and distant metastases. […] Because epithelioid sarcoma presents innocuously, it is often mistaken as a benign process, which can result in insufficient treatment. Therefore, we emphasize the need for clinicians to consider this diagnosis when a slow-growing tumor is found on the distal extremity of a young male as the malignancy inherently portends a poor prognosis. […] The diagnosis of ES is made via histopathological examination. Prior to obtaining a biopsy of a subcutaneous tumor, some clinicians may prefer to obtain imaging in order to assess for subclinical involvement and the best means of biopsy. Because of its ability to visualize soft tissue detail, magnetic resonance imaging (MRI), as opposed to computed tomography scans and X-rays, is the preferred modality for imaging.

• #3 3 Things You Should Know About Identifying and Treating Epithelioid Sarcoma

  https://www.cancernetwork.com/view/3-things-you-should-know-about-identifying-and-treating-epithelioid-sarcoma

  Experts discuss epithelioid sarcoma diagnosis, management, and targeted therapies, and highlight 3 things everyone should know about treatment. […] Epithelioid sarcoma (ES) is a rare form of soft tissue sarcoma (STS) that accounts for fewer than 1% of STS cases. The disease is known for being difficult to diagnose and treat and for being associated with high rates of local recurrence (34%-77%) and metastasis (40%). […] Given the ambiguous clinical features of ES, the diagnosis often hinges on microscopic appearance and genetic analysis. The distinctive mutation in ES is a loss of INI1 function, which is present in over 90% of tumors regardless of subtype. […] As with all suspected cases of STS, it is recommended that possible ES lesions be evaluated by collaborating members of a multidisciplinary team who are experienced in the management of sarcomas.

• #4 Epithelioid Sarcoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2924131/

  The three variants of ES are epithelioid, spindled, and mixed, with the principal form being epithelioid. […] Elucidation of the diagnosis is aided by consistently positive staining with the immunoreactants vimentin, epithelial membrane antigen, and cytokeratin. […] Aggressive treatment with preoperative or postoperative radiation therapy combined with wide local excision appears to be indicated once diagnosis is established via histopathological confirmation.

• #5 Epithelioid sarcoma | Sarcoma UK

  https://sarcoma.org.uk/about-sarcoma/what-is-sarcoma/types-of-sarcoma/epithelioid-sarcoma/

  Epithelioid sarcoma is a very rare type of soft tissue sarcoma. […] A specialist doctor will diagnose epithelioid sarcoma through a series of tests. These may include: […] A biopsy taking and testing a sample of the tumour. Using this biopsy, a doctor will look to see if the biopsy looks like epithelioid sarcoma and whether it contains the SMARCB1 error. […] There are two types of epithelioid sarcoma that you may be diagnosed with: Distal The most common type that commonly affects the hands, feet, legs and arms; Proximal A less common type that mostly affects the central trunk, such as the chest, abdomen, pelvis and back.

• #6 Distal and proximal epithelioid sarcoma — differences in diagnosis and similarities in treatment | Krotewicz | Oncology in Clinical Practice

  https://journals.viamedica.pl/oncology_in_clinical_practice/article/view/99119

  Epithelioid sarcoma (ES) comprises two subtypes, distal and proximal. […] The differences in clinical presentation underscore the necessity for tailored treatment approaches for each ES subtype. […] Tazemetostat, an oral selective inhibitor of the histone methyltransferase enhancer of zeste homolog 2 (EZH2), has recently gained FDA approval as a first-line treatment for ES patients. […] Epithelioid sarcoma: Diagnostic Features and Genetics. […] Epithelioid sarcoma: a review and update.

• #7 Navigating Diagnostic Challenges in Epithelioid Sarcoma: Multidisciplinary Strategies to Optimize Diagnosis

  https://www.cancernetwork.com/view/navigating-diagnostic-challenges-in-epithelioid-sarcoma-multidisciplinary-strategies-to-optimize-diagnosis

  Diagnosing epithelioid sarcoma (ES) is often challenging due to its rarity, variable presentation, and histologic overlap with other conditions. […] Common diagnostic pitfalls include misinterpreting ES as benign lesions, such as granulomatous inflammation or reactive processes, especially in the early stages. […] A key diagnostic marker is loss of INI1 (SMARCB1), which should be assessed in cases with ambiguous histology. […] Awareness of these diagnostic nuances and incorporating molecular testing early in the workup can significantly improve diagnostic accuracy.

• #8 Epithelioid Sarcoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2924131/

  Epithelioid sarcoma is a rare, high-grade, soft tissue tumor that has a known propensity for local recurrence, regional lymph node involvement, and distant metastases. […] Because epithelioid sarcoma presents innocuously, it is often mistaken as a benign process, which can result in insufficient treatment. Therefore, we emphasize the need for clinicians to consider this diagnosis when a slow-growing tumor is found on the distal extremity of a young male as the malignancy inherently portends a poor prognosis. […] The diagnosis of ES is made via histopathological examination. Prior to obtaining a biopsy of a subcutaneous tumor, some clinicians may prefer to obtain imaging in order to assess for subclinical involvement and the best means of biopsy. Because of its ability to visualize soft tissue detail, magnetic resonance imaging (MRI), as opposed to computed tomography scans and X-rays, is the preferred modality for imaging.

• #9 Navigating Diagnostic Challenges in Epithelioid Sarcoma: Multidisciplinary Strategies to Optimize Diagnosis

  https://www.cancernetwork.com/view/navigating-diagnostic-challenges-in-epithelioid-sarcoma-multidisciplinary-strategies-to-optimize-diagnosis

  Diagnosing epithelioid sarcoma (ES) is often challenging due to its rarity, variable presentation, and histologic overlap with other conditions. […] Common diagnostic pitfalls include misinterpreting ES as benign lesions, such as granulomatous inflammation or reactive processes, especially in the early stages. […] A key diagnostic marker is loss of INI1 (SMARCB1), which should be assessed in cases with ambiguous histology. […] Awareness of these diagnostic nuances and incorporating molecular testing early in the workup can significantly improve diagnostic accuracy.

• #10 Epithelioid sarcoma // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/epithelioid-sarcoma

  Epithelioid sarcoma can be hard to diagnose. It looks like problems that are much more common. Often healthcare professionals consider those more common problems first. For example, a sore on the skin that is not healing could be mistaken for a skin infection. […] Tests and procedures used in the diagnosis of epithelioid sarcoma include: […] Imaging tests take pictures of the body. They can show the location and size of an epithelioid sarcoma. Tests might include X-ray, MRI, CT and positron emission tomography, which is also called a PET scan. […] A biopsy is a procedure to remove a sample of tissue for testing in a lab. The tissue might be removed using a needle that is put through the skin and into the cancer. Sometimes surgery is needed to get the tissue sample. The sample is tested in a lab to see if it is cancer. Other special tests give more details about the cancer cells. Your healthcare team uses this information to make a treatment plan.

• #11 Epithelioid Sarcoma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/449

  Diagnosing epithelioid sarcoma can be challenging due to its mixed mesenchymal and epithelial differentiation. This unique characteristic can cause the tumor to appear similar to various other reactive, benign, and malignant processes, making histologic diagnosis alone difficult. Immunohistochemical labeling may be used to confirm the diagnosis, although it is essential to consider other more common diagnostic entities with similar histologic and immunophenotypic characteristics. […] The differential diagnosis based on histomorphology generally includes reactive and benign lesions such as granulomatous diseases, nodular fasciitis, fibrohistiocytic lesions, fibromatosis, and tenosynovial giant cell tumors. Malignant lesions in the differential diagnosis include metastatic carcinoma, melanoma, synovial sarcoma, vascular neoplasms, spindle cell squamous cell carcinoma, malignant peripheral nerve sheath tumor (MPNST), and extrarenal rhabdoid tumor.

• #12 Epithelioid Sarcoma | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/e/epithelioid-sarcoma

  Epithelioid sarcoma is rare and commonly misdiagnosed. That is why it is so important for people who have this condition to receive care from specialists who have extensive experience in diagnosing and treating it. […] Patients will need a physical exam, blood work and imaging tests to diagnose epithelioid sarcoma. These tests help the doctor learn the exact size and location of the tumor and help to see if the cancer has spread. […] Patients will also have a biopsy where the doctor removes a small piece of the tumor. A specialist examines the tissue under a microscope to check for the SMARCB1 or INI-1 marker. The results may guide the team as it develops a treatment plan. […] Prompt diagnosis and treatment can greatly improve the outcome for patients with epithelioid sarcoma. That is why it is vitally important for patients to receive care at a hospital such as Cincinnati Childrens, whose sarcoma team has experience in diagnosing and treating this rare type of cancer.

• #13 Recommendations from the Epithelioid Sarcoma Collaborative: A White Paper – Journal of Oncology Navigation & Survivorship

  https://www.jons-online.com/issues/2021/september-2021-vol-12-no-9/recommendations-from-the-epithelioid-sarcoma-collaborative-a-white-paper

  A key factor identified by the collaborative that impacted patient outcomes is a lengthy or prolonged journey from disease presentation to disease diagnosis, to treatment and care, otherwise referred to as a diagnostic delay as described in other sarcomas and rare malignancies. Prolonged time to diagnosis and treatment is recognized to impact patient outcomes based on the assumption that early diagnosis leads to better local control and overall survival. The diagnostic journey overall is recognized to be prolonged among patients diagnosed with a sarcoma compared with other cancers. Greater diagnostic delay has also been recognized to be associated with patients with limitations in healthcare coverage, older populations, and lower socioeconomic profiles (ie, Medicare and Medicaid). […] Using the model developed by Olesen and colleagues, the diagnosis journey can be subdivided into the time periods between symptoms and treatment, each of which has potential for delay in the diagnosis journey. The patient delay describes the period where the patient experiences and responds to symptoms. The GP delay describes the period when the symptoms are first described by a patient to a clinician, often a GP, to the consideration of the symptoms as cancer. This delay may be compounded by inadequate imaging (ultrasound imaging) or the misinterpretation of appropriate MRI imaging by radiologists unfamiliar with ES and STSs. The third delay is characterized as a system delay or healthcare provider delay, the clinical pathway from GP referral until diagnosis and treatment.

• #14 Recommendations from the Epithelioid Sarcoma Collaborative: A White Paper – Journal of Oncology Navigation & Survivorship

  https://www.jons-online.com/issues/2021/september-2021-vol-12-no-9/recommendations-from-the-epithelioid-sarcoma-collaborative-a-white-paper

  A key factor identified by the collaborative that impacted patient outcomes is a lengthy or prolonged journey from disease presentation to disease diagnosis, to treatment and care, otherwise referred to as a diagnostic delay as described in other sarcomas and rare malignancies. Prolonged time to diagnosis and treatment is recognized to impact patient outcomes based on the assumption that early diagnosis leads to better local control and overall survival. The diagnostic journey overall is recognized to be prolonged among patients diagnosed with a sarcoma compared with other cancers. Greater diagnostic delay has also been recognized to be associated with patients with limitations in healthcare coverage, older populations, and lower socioeconomic profiles (ie, Medicare and Medicaid). […] Using the model developed by Olesen and colleagues, the diagnosis journey can be subdivided into the time periods between symptoms and treatment, each of which has potential for delay in the diagnosis journey. The patient delay describes the period where the patient experiences and responds to symptoms. The GP delay describes the period when the symptoms are first described by a patient to a clinician, often a GP, to the consideration of the symptoms as cancer. This delay may be compounded by inadequate imaging (ultrasound imaging) or the misinterpretation of appropriate MRI imaging by radiologists unfamiliar with ES and STSs. The third delay is characterized as a system delay or healthcare provider delay, the clinical pathway from GP referral until diagnosis and treatment.

• #15 Recommendations from the Epithelioid Sarcoma Collaborative: A White Paper – Journal of Oncology Navigation & Survivorship

  https://www.jons-online.com/issues/2021/september-2021-vol-12-no-9/recommendations-from-the-epithelioid-sarcoma-collaborative-a-white-paper

  Overcoming delays inherent in each interval of the diagnosis journey is recommended. A first step of increasing awareness of STS overall among patients and providers may be to reduce the patient delay in recognizing symptoms of ES. Increased awareness coupled with providing ES resources and educating healthcare providers about the different types of STS may also reduce the GP delay in considering the presentation as a possible cancer and improving the patient-care partner-physician dialogue. Finally, overcoming system delays to increase timely medical care and reduce misdiagnosis or delays in diagnosis necessitates solutions to the cascade of delays. One proposed solution is to establish an ES standard of diagnosis for widespread adoption among healthcare providers that includes a recommended timeline, such as a 6-week follow-up and imaging, followed by a second opinion, followed by diagnosis confirmed by an expert pathologist.

• #16 Recommendations from the Epithelioid Sarcoma Collaborative: A White Paper – Journal of Oncology Navigation & Survivorship

  https://www.jons-online.com/issues/2021/september-2021-vol-12-no-9/recommendations-from-the-epithelioid-sarcoma-collaborative-a-white-paper

  A key factor identified by the collaborative that impacted patient outcomes is a lengthy or prolonged journey from disease presentation to disease diagnosis, to treatment and care, otherwise referred to as a diagnostic delay as described in other sarcomas and rare malignancies. Prolonged time to diagnosis and treatment is recognized to impact patient outcomes based on the assumption that early diagnosis leads to better local control and overall survival. The diagnostic journey overall is recognized to be prolonged among patients diagnosed with a sarcoma compared with other cancers. Greater diagnostic delay has also been recognized to be associated with patients with limitations in healthcare coverage, older populations, and lower socioeconomic profiles (ie, Medicare and Medicaid). […] Using the model developed by Olesen and colleagues, the diagnosis journey can be subdivided into the time periods between symptoms and treatment, each of which has potential for delay in the diagnosis journey. The patient delay describes the period where the patient experiences and responds to symptoms. The GP delay describes the period when the symptoms are first described by a patient to a clinician, often a GP, to the consideration of the symptoms as cancer. This delay may be compounded by inadequate imaging (ultrasound imaging) or the misinterpretation of appropriate MRI imaging by radiologists unfamiliar with ES and STSs. The third delay is characterized as a system delay or healthcare provider delay, the clinical pathway from GP referral until diagnosis and treatment.

• #17 Epithelioid Sarcoma: Symptoms, Treatment & Prognosis

  https://my.clevelandclinic.org/health/diseases/24331-epithelioid-sarcoma

  Epithelioid sarcoma is a rare subtype of soft tissue sarcoma. […] A healthcare provider will run several tests to diagnose ES, which may include: […] A physical examination. […] Biopsy. […] Blood tests for cancer. […] Magnetic resonance imaging (MRI). […] Computed tomography (CT) scans. […] Positron emission tomography (PET) scans. […] Epithelioid sarcoma is treatable, especially when detected early. […] Your healthcare provider will run several tests to determine how big the tumor is and whether it has spread to other areas of your body. […] The only way to cure ES is to completely remove the tumor. […] If a surgeon is unable to completely remove a tumor, theyll likely recommend a combination of treatments to manage your condition. […] Many research studies have reported on epithelioid sarcoma survival rates through the years. […] The five-year survival rate for epithelioid sarcoma ranges from 25% to 92%, depending on which study you read.

• #18 Epithelioid Sarcoma | Oncology | Bon Secours

  https://www.bonsecours.com/health-care-services/cancer-care-oncology/conditions/epithelioid-sarcoma

  Doctors use imaging tests, biopsy and physical exams to diagnose epithelioid sarcoma. […] Your oncologist may use one or more of the following tests to diagnose this condition: […] Physical exam your doctor will perform a complete physical exam, including asking questions about your health history, symptoms and related risk factors. […] Biopsy in this test, your doctor removes a biopsy (small sample) from the suspicious area. This sample is sent to the laboratory, where a specialist closely checks the biopsy for epithelioid sarcoma. […] Computerized tomography (CT) scan this type of imaging test provides a 3D image of the inside of the body that your doctor can use to determine the size and location of the epithelioid sarcoma. […] Magnetic resonance imaging (MRI) this type of imaging test uses high-powered magnets to create detailed images of the inside of your body. Your doctor can closely examine these images to determine the size and location of the epithelioid sarcoma.

• #19 What is Epithelioid Sarcoma? – Surgical Oncology

  https://surgicaloncology.co.uk/epithelioid-sarcoma-collaborative-calls-for-earlier-diagnosis/

  A new white paper has been published by Epithelioid Sarcoma Collaborative, addressing the challenges of those with this rare type of cancer. Currently, there is very little awareness of this type of cancer, mostly known to affect younger adults. […] Patients with Epithelioid Sarcoma face a lot of challenges compared to those diagnosed with more common cancers. The main challenges include: The time it takes to receive a diagnosis, A lack of awareness of the cancer. […] As, it may be difficult to diagnose, in particular for primary carers, you should see advice if you have any of these symptoms: a lump that is 5cm or bigger (the short side of credit card), rapidly growing, deep, painful or recurrent. […] There is very little awareness about this type of cancer. While it may be rare, awareness does need to be raised to ensure patients get the help they need in a timely manner. […] These are the main challenges patients face when getting diagnosed and treated for this rare form of cancer. When it is diagnosed, there are effective treatment options available.

• #20 Epithelioid Sarcoma: Symptoms, Treatment & Prognosis

  https://my.clevelandclinic.org/health/diseases/24331-epithelioid-sarcoma

  Epithelioid sarcoma is a rare subtype of soft tissue sarcoma. […] A healthcare provider will run several tests to diagnose ES, which may include: […] A physical examination. […] Biopsy. […] Blood tests for cancer. […] Magnetic resonance imaging (MRI). […] Computed tomography (CT) scans. […] Positron emission tomography (PET) scans. […] Epithelioid sarcoma is treatable, especially when detected early. […] Your healthcare provider will run several tests to determine how big the tumor is and whether it has spread to other areas of your body. […] The only way to cure ES is to completely remove the tumor. […] If a surgeon is unable to completely remove a tumor, theyll likely recommend a combination of treatments to manage your condition. […] Many research studies have reported on epithelioid sarcoma survival rates through the years. […] The five-year survival rate for epithelioid sarcoma ranges from 25% to 92%, depending on which study you read.

• #21 Epithelioid Sarcoma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/449

  Epithelioid sarcoma is locally invasive and frequently metastasizes to regional lymph nodes and distant sites. Diagnosis is determined by tissue biopsy, while imaging studies are essential for assessing the extent of the primary tumor and identifying metastatic disease. […] A tissue biopsy is necessary, as soft tissue swellings have many possible causes. Core needle biopsies are the preferred method. Based on clinical examination, biopsies should also be taken from any suspicious lymph nodes. Magnetic resonance imaging (MRI) is the preferred diagnostic tool for most soft-tissue sarcomas, as it provides detailed images of the extent of tumor invasion into the surrounding fascial planes. […] Epithelioid sarcoma is associated with a high rate of distant metastases, with recent studies suggesting a distant metastasis rate of up to 50%. The most common sites of metastases are the lungs, lymph nodes, and skin. As a result, staging imaging with a chest computed tomography (CT) scan is typically performed.

• #22 Epithelioid Sarcoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2924131/

  Epithelioid sarcoma is a rare, high-grade, soft tissue tumor that has a known propensity for local recurrence, regional lymph node involvement, and distant metastases. […] Because epithelioid sarcoma presents innocuously, it is often mistaken as a benign process, which can result in insufficient treatment. Therefore, we emphasize the need for clinicians to consider this diagnosis when a slow-growing tumor is found on the distal extremity of a young male as the malignancy inherently portends a poor prognosis. […] The diagnosis of ES is made via histopathological examination. Prior to obtaining a biopsy of a subcutaneous tumor, some clinicians may prefer to obtain imaging in order to assess for subclinical involvement and the best means of biopsy. Because of its ability to visualize soft tissue detail, magnetic resonance imaging (MRI), as opposed to computed tomography scans and X-rays, is the preferred modality for imaging.

• #23 Epithelioid sarcoma | Applied Radiology

  https://www.appliedradiology.com/Articles/epithelioid-sarcoma

  Epithelioid sarcoma (conventional type). […] Imaging features are best delineated with contrast-enhanced MRI to evaluate extent prior to biopsy and/or surgical resection. […] Epithelioid sarcoma is an aggressive soft-tissue sarcoma, and the most common sarcoma involving the distal upper extremity in adolescents and young adults. When presented with a tumor in this location, epithelioid sarcoma should be included as a primary consideration, particularly in a tumor that demonstrates a characteristic infiltrating pattern along the volar fascial planes.

• #24 Epithelioid Sarcoma | Oncology | Bon Secours

  https://www.bonsecours.com/health-care-services/cancer-care-oncology/conditions/epithelioid-sarcoma

  Doctors use imaging tests, biopsy and physical exams to diagnose epithelioid sarcoma. […] Your oncologist may use one or more of the following tests to diagnose this condition: […] Physical exam your doctor will perform a complete physical exam, including asking questions about your health history, symptoms and related risk factors. […] Biopsy in this test, your doctor removes a biopsy (small sample) from the suspicious area. This sample is sent to the laboratory, where a specialist closely checks the biopsy for epithelioid sarcoma. […] Computerized tomography (CT) scan this type of imaging test provides a 3D image of the inside of the body that your doctor can use to determine the size and location of the epithelioid sarcoma. […] Magnetic resonance imaging (MRI) this type of imaging test uses high-powered magnets to create detailed images of the inside of your body. Your doctor can closely examine these images to determine the size and location of the epithelioid sarcoma.

• #25 Epithelioid sarcoma // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/epithelioid-sarcoma

  Epithelioid sarcoma can be hard to diagnose. It looks like problems that are much more common. Often healthcare professionals consider those more common problems first. For example, a sore on the skin that is not healing could be mistaken for a skin infection. […] Tests and procedures used in the diagnosis of epithelioid sarcoma include: […] Imaging tests take pictures of the body. They can show the location and size of an epithelioid sarcoma. Tests might include X-ray, MRI, CT and positron emission tomography, which is also called a PET scan. […] A biopsy is a procedure to remove a sample of tissue for testing in a lab. The tissue might be removed using a needle that is put through the skin and into the cancer. Sometimes surgery is needed to get the tissue sample. The sample is tested in a lab to see if it is cancer. Other special tests give more details about the cancer cells. Your healthcare team uses this information to make a treatment plan.

• #26 The diagnosis and treatment of the epithelioid sarcomas involving the peripheral nerves | Scientific Reports

  https://www.nature.com/articles/s41598-024-82357-z

  Epithelioid sarcomas are rare soft tissue tumors and have possibility to involve the peripheral nerve and present as sensory and motor disorders. […] This situation is extremely rare in clinic and was only reported as several case reports in literature. It can be easily ignored and misdiagnosed in clinic and may bring out severe outcomes. […] Early diagnosis and treatment are the keys to better prognosis. We recommended high-resolution ultrasound as a standard diagnostic tool for nerve compression syndromes not only reveal the morphological structure of the peripheral nerve, but also discover the tumor involving the nerve. […] High-resolution ultrasound is recommended to avoid misdiagnosis and mistreatment in clinic. […] It should be emphasized that excluding tumors is necessary for the patients whose symptoms resembled those of the nerve compression diseases. High-resolution ultrasound can not only reveal the morphological structure of the peripheral nerve, but also find the nerve originated tumor or the tumor which compresses the peripheral nerve.

• #27 The diagnosis and treatment of the epithelioid sarcomas involving the peripheral nerves | Scientific Reports

  https://www.nature.com/articles/s41598-024-82357-z

  Epithelioid sarcomas are rare soft tissue tumors and have possibility to involve the peripheral nerve and present as sensory and motor disorders. […] This situation is extremely rare in clinic and was only reported as several case reports in literature. It can be easily ignored and misdiagnosed in clinic and may bring out severe outcomes. […] Early diagnosis and treatment are the keys to better prognosis. We recommended high-resolution ultrasound as a standard diagnostic tool for nerve compression syndromes not only reveal the morphological structure of the peripheral nerve, but also discover the tumor involving the nerve. […] High-resolution ultrasound is recommended to avoid misdiagnosis and mistreatment in clinic. […] It should be emphasized that excluding tumors is necessary for the patients whose symptoms resembled those of the nerve compression diseases. High-resolution ultrasound can not only reveal the morphological structure of the peripheral nerve, but also find the nerve originated tumor or the tumor which compresses the peripheral nerve.

• #28 Epithelioid Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK532911/

  Epithelioid sarcoma is a rare and aggressive soft tissue sarcoma characterized by both epithelial and mesenchymal differentiation. […] Diagnosis is typically made through tissue biopsy, while imaging studies are essential for assessing the extent of the primary tumor and identifying metastatic disease. […] A tissue biopsy is necessary, as soft tissue swellings have many possible causes. Core needle biopsies are the preferred method. […] Epithelioid sarcoma is associated with a high rate of distant metastases, with recent studies suggesting a distant metastasis rate of up to 50%. […] Diagnosing epithelioid sarcoma can be challenging due to its mixed mesenchymal and epithelial differentiation. […] The most accurate diagnosis can be made by considering the appropriate clinical context, such as the presence of a soft tissue mass on the extremity of a young male, with immunohistochemical staining providing diagnostic support.

• #29 Epithelioid Sarcoma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/449

  Epithelioid sarcoma is locally invasive and frequently metastasizes to regional lymph nodes and distant sites. Diagnosis is determined by tissue biopsy, while imaging studies are essential for assessing the extent of the primary tumor and identifying metastatic disease. […] A tissue biopsy is necessary, as soft tissue swellings have many possible causes. Core needle biopsies are the preferred method. Based on clinical examination, biopsies should also be taken from any suspicious lymph nodes. Magnetic resonance imaging (MRI) is the preferred diagnostic tool for most soft-tissue sarcomas, as it provides detailed images of the extent of tumor invasion into the surrounding fascial planes. […] Epithelioid sarcoma is associated with a high rate of distant metastases, with recent studies suggesting a distant metastasis rate of up to 50%. The most common sites of metastases are the lungs, lymph nodes, and skin. As a result, staging imaging with a chest computed tomography (CT) scan is typically performed.

• #30 Pathology Outlines – Epithelioid sarcoma

  https://www.pathologyoutlines.com/topic/softtissueepithelioidsarcoma.html

  A malignant mesenchymal neoplasm that exhibits epithelioid cytomorphology and a predominantly epithelial phenotype […] Diagnosis […] Core needle biopsy for deep tumors and punch biopsy for dermal tumors […] Followed by wide resection […] The course of epithelioid sarcoma is often unpredictable and it is common for patients to present with extensive disease, lymph node metastases, or distant metastases.

• #31 Epithelioid Sarcoma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/449

  Epithelioid sarcoma is locally invasive and frequently metastasizes to regional lymph nodes and distant sites. Diagnosis is determined by tissue biopsy, while imaging studies are essential for assessing the extent of the primary tumor and identifying metastatic disease. […] A tissue biopsy is necessary, as soft tissue swellings have many possible causes. Core needle biopsies are the preferred method. Based on clinical examination, biopsies should also be taken from any suspicious lymph nodes. Magnetic resonance imaging (MRI) is the preferred diagnostic tool for most soft-tissue sarcomas, as it provides detailed images of the extent of tumor invasion into the surrounding fascial planes. […] Epithelioid sarcoma is associated with a high rate of distant metastases, with recent studies suggesting a distant metastasis rate of up to 50%. The most common sites of metastases are the lungs, lymph nodes, and skin. As a result, staging imaging with a chest computed tomography (CT) scan is typically performed.

• #32 Pathology Outlines – Epithelioid sarcoma

  https://www.pathologyoutlines.com/topic/softtissueepithelioidsarcoma.html

  A malignant mesenchymal neoplasm that exhibits epithelioid cytomorphology and a predominantly epithelial phenotype […] Diagnosis […] Core needle biopsy for deep tumors and punch biopsy for dermal tumors […] Followed by wide resection […] The course of epithelioid sarcoma is often unpredictable and it is common for patients to present with extensive disease, lymph node metastases, or distant metastases.

• #33 Epithelioid sarcoma // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/epithelioid-sarcoma

  Epithelioid sarcoma can be hard to diagnose. It looks like problems that are much more common. Often healthcare professionals consider those more common problems first. For example, a sore on the skin that is not healing could be mistaken for a skin infection. […] Tests and procedures used in the diagnosis of epithelioid sarcoma include: […] Imaging tests take pictures of the body. They can show the location and size of an epithelioid sarcoma. Tests might include X-ray, MRI, CT and positron emission tomography, which is also called a PET scan. […] A biopsy is a procedure to remove a sample of tissue for testing in a lab. The tissue might be removed using a needle that is put through the skin and into the cancer. Sometimes surgery is needed to get the tissue sample. The sample is tested in a lab to see if it is cancer. Other special tests give more details about the cancer cells. Your healthcare team uses this information to make a treatment plan.

• #34 Epithelioid Sarcoma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/449

  Epithelioid sarcoma is locally invasive and frequently metastasizes to regional lymph nodes and distant sites. Diagnosis is determined by tissue biopsy, while imaging studies are essential for assessing the extent of the primary tumor and identifying metastatic disease. […] A tissue biopsy is necessary, as soft tissue swellings have many possible causes. Core needle biopsies are the preferred method. Based on clinical examination, biopsies should also be taken from any suspicious lymph nodes. Magnetic resonance imaging (MRI) is the preferred diagnostic tool for most soft-tissue sarcomas, as it provides detailed images of the extent of tumor invasion into the surrounding fascial planes. […] Epithelioid sarcoma is associated with a high rate of distant metastases, with recent studies suggesting a distant metastasis rate of up to 50%. The most common sites of metastases are the lungs, lymph nodes, and skin. As a result, staging imaging with a chest computed tomography (CT) scan is typically performed.

• #35 Epithelioid Sarcoma: An overview with emphasis on its differentiation from morphologic mimics – JDPO

  https://www.jdpo.org/html-article/15946

  Epithelioid sarcoma (ES) is a malignant mesenchymal neoplasm that exhibits epithelioid cytomorphology and a predominantly epithelial phenotype. The aim of the study was to analyze our cases of ES regarding clinical, pathological and immunohistochemical features. On immunohistochemistry (IHC), all the tumors were reactive for CK, EMA, Vimentin, Ca 125 and showed loss of nuclear expression of INI1. Co-expression of epithelial and mesenchymal markers along with reactivity for CD34 and Ca 125 and loss on INI1 expression on IHC substantiate the diagnosis of ES. ES is often misdiagnosed because of nonspecific clinical features at presentation. Helpful clues in diagnosis are tumors in young males with epithelioid and/or spindle cell morphology, Rhabdoid cells and granuloma like central necrosis. […] On IHC, both CES and PES were typically positive for cytokeratin (CK), epithelial membrane antigen (EMA), vimentin. CA 125 was expressed in all 7 cases and CD34 in 6 cases (except in case 4). Loss of nuclear expression of SMARC B1 protein/INI 1 was noted in all 7 cases.

• #36 Epithelioid sarcoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/epithelioid-sarcoma?embed_domain=Navimumbaihouses.com%2Fblog%2Fnews%2Fhow-youll-know-if-your-client-is-truly-motivated-to-buy%2F&lang=us

  Epithelioid sarcomas are diagnosed based on typical histological and molecular pathological features. […] Diagnostic criteria according to the WHO classification of soft tissue and bone tumors (5th edition): soft tissue mass (distal or proximal presentation), epithelioid to spindled cell morphology, infiltrative growth, SMARCB1/INI1 loss, immunoreactivity for epithelial membrane antigen (EMA) and keratin.

• #37 Epithelioid Sarcoma: An overview with emphasis on its differentiation from morphologic mimics – JDPO

  https://www.jdpo.org/html-article/15946

  Epithelioid sarcoma (ES) is a malignant mesenchymal neoplasm that exhibits epithelioid cytomorphology and a predominantly epithelial phenotype. The aim of the study was to analyze our cases of ES regarding clinical, pathological and immunohistochemical features. On immunohistochemistry (IHC), all the tumors were reactive for CK, EMA, Vimentin, Ca 125 and showed loss of nuclear expression of INI1. Co-expression of epithelial and mesenchymal markers along with reactivity for CD34 and Ca 125 and loss on INI1 expression on IHC substantiate the diagnosis of ES. ES is often misdiagnosed because of nonspecific clinical features at presentation. Helpful clues in diagnosis are tumors in young males with epithelioid and/or spindle cell morphology, Rhabdoid cells and granuloma like central necrosis. […] On IHC, both CES and PES were typically positive for cytokeratin (CK), epithelial membrane antigen (EMA), vimentin. CA 125 was expressed in all 7 cases and CD34 in 6 cases (except in case 4). Loss of nuclear expression of SMARC B1 protein/INI 1 was noted in all 7 cases.

• #38 Epithelioid Sarcoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK532911/

  The differential diagnosis based on histomorphology generally includes reactive and benign lesions such as granulomatous diseases, nodular fasciitis, fibrohistiocytic lesions, fibromatosis, and tenosynovial giant cell tumors. […] Immunohistochemical labeling may be used to confirm the diagnosis, although it is essential to consider other more common diagnostic entities with similar histologic and immunophenotypic characteristics.

• #39 Epithelioid Sarcoma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/449

  Diagnosing epithelioid sarcoma can be challenging due to its mixed mesenchymal and epithelial differentiation. This unique characteristic can cause the tumor to appear similar to various other reactive, benign, and malignant processes, making histologic diagnosis alone difficult. Immunohistochemical labeling may be used to confirm the diagnosis, although it is essential to consider other more common diagnostic entities with similar histologic and immunophenotypic characteristics. […] The differential diagnosis based on histomorphology generally includes reactive and benign lesions such as granulomatous diseases, nodular fasciitis, fibrohistiocytic lesions, fibromatosis, and tenosynovial giant cell tumors. Malignant lesions in the differential diagnosis include metastatic carcinoma, melanoma, synovial sarcoma, vascular neoplasms, spindle cell squamous cell carcinoma, malignant peripheral nerve sheath tumor (MPNST), and extrarenal rhabdoid tumor.

• #40 Epithelioid Sarcoma: An overview with emphasis on its differentiation from morphologic mimics – JDPO

  https://www.jdpo.org/html-article/15946

  Epithelioid sarcoma (ES) is a malignant mesenchymal neoplasm that exhibits epithelioid cytomorphology and a predominantly epithelial phenotype. The aim of the study was to analyze our cases of ES regarding clinical, pathological and immunohistochemical features. On immunohistochemistry (IHC), all the tumors were reactive for CK, EMA, Vimentin, Ca 125 and showed loss of nuclear expression of INI1. Co-expression of epithelial and mesenchymal markers along with reactivity for CD34 and Ca 125 and loss on INI1 expression on IHC substantiate the diagnosis of ES. ES is often misdiagnosed because of nonspecific clinical features at presentation. Helpful clues in diagnosis are tumors in young males with epithelioid and/or spindle cell morphology, Rhabdoid cells and granuloma like central necrosis. […] On IHC, both CES and PES were typically positive for cytokeratin (CK), epithelial membrane antigen (EMA), vimentin. CA 125 was expressed in all 7 cases and CD34 in 6 cases (except in case 4). Loss of nuclear expression of SMARC B1 protein/INI 1 was noted in all 7 cases.

• #41 Epithelioid Sarcoma: A Review and Update | JCAD – The Journal of Clinical and Aesthetic Dermatology

  https://jcadonline.com/epithelioid-sarcoma-a-review-and-update/

  Epithelioid sarcoma is a rare, high-grade, soft tissue tumor that has a known propensity for local recurrence, regional lymph node involvement, and distant metastases. […] The diagnosis of ES is made via histopathological examination. Prior to obtaining a biopsy of a subcutaneous tumor, some clinicians may prefer to obtain imaging in order to assess for subclinical involvement and the best means of biopsy. Because of its ability to visualize soft tissue detail, magnetic resonance imaging (MRI), as opposed to computed tomography scans and X-rays, is the preferred modality for imaging. […] The three variants of ES are epithelioid, spindled, and mixed, with the principal form being epithelioid. […] Elucidation of the diagnosis is aided by consistently positive staining with the immunoreactants vimentin, epithelial membrane antigen, and cytokeratin. […] Aggressive treatment with preoperative or postoperative radiation therapy combined with wide local excision appears to be indicated once diagnosis is established via histopathological confirmation. […] SLNB and lymphadenectomy may also be helpful in certain subsets of patients.

• #42 Epithelioid Sarcoma: An overview with emphasis on its differentiation from morphologic mimics – JDPO

  https://www.jdpo.org/html-article/15946

  Epithelioid sarcoma (ES) is a malignant mesenchymal neoplasm that exhibits epithelioid cytomorphology and a predominantly epithelial phenotype. The aim of the study was to analyze our cases of ES regarding clinical, pathological and immunohistochemical features. On immunohistochemistry (IHC), all the tumors were reactive for CK, EMA, Vimentin, Ca 125 and showed loss of nuclear expression of INI1. Co-expression of epithelial and mesenchymal markers along with reactivity for CD34 and Ca 125 and loss on INI1 expression on IHC substantiate the diagnosis of ES. ES is often misdiagnosed because of nonspecific clinical features at presentation. Helpful clues in diagnosis are tumors in young males with epithelioid and/or spindle cell morphology, Rhabdoid cells and granuloma like central necrosis. […] On IHC, both CES and PES were typically positive for cytokeratin (CK), epithelial membrane antigen (EMA), vimentin. CA 125 was expressed in all 7 cases and CD34 in 6 cases (except in case 4). Loss of nuclear expression of SMARC B1 protein/INI 1 was noted in all 7 cases.

• #43 Epithelioid sarcoma of the extremities – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/epithelioid-sarcoma-of-the-extremities/

  Histopathology of the excised specimen confirmed the diagnosis of epithelioid sarcoma. […] The paucity of symptoms, a benign appearance in early-stage imaging and indistinctive histopathology in some cases makes the diagnosis challenging. […] Biopsy is the diagnostic modality of choice. […] The tumor consists of epithelioid cells well blended with fusiform cells. […] It exhibits immunohistochemical reactivity for keratins and epithelial membrane antigen, vimentin and CD34 (in more than half of the cases). […] One of our patients, in addition, had a high level of CA 125. Serum CA 125 level could be a useful marker for diagnosis and for monitoring the clinical course. […] Histopathological differential diagnoses for epithelioid sarcoma include both benign and malignant conditions such as granuloma annulare, necrobiosis lipoidica, fibrous histiocytoma, nodular fasciitis, melanoma, clear-cell sarcoma of the tendon and aponeurosis, metastatic squamous cell carcinoma, synovial sarcoma, epithelioid hemangioendothelioma and malignant extra-renal rhabdoid tumors of the soft tissue. […] Epithelioid sarcoma should be considered in the differential diagnosis of chronic nodules, especially on the extremities of young men.

• #44 Navigating Diagnostic Challenges in Epithelioid Sarcoma: Multidisciplinary Strategies to Optimize Diagnosis

  https://www.cancernetwork.com/view/navigating-diagnostic-challenges-in-epithelioid-sarcoma-multidisciplinary-strategies-to-optimize-diagnosis

  Diagnosing epithelioid sarcoma (ES) is often challenging due to its rarity, variable presentation, and histologic overlap with other conditions. […] Common diagnostic pitfalls include misinterpreting ES as benign lesions, such as granulomatous inflammation or reactive processes, especially in the early stages. […] A key diagnostic marker is loss of INI1 (SMARCB1), which should be assessed in cases with ambiguous histology. […] Awareness of these diagnostic nuances and incorporating molecular testing early in the workup can significantly improve diagnostic accuracy.

• #45 3 Things You Should Know About Identifying and Treating Epithelioid Sarcoma

  https://www.cancernetwork.com/view/3-things-you-should-know-about-identifying-and-treating-epithelioid-sarcoma

  Experts discuss epithelioid sarcoma diagnosis, management, and targeted therapies, and highlight 3 things everyone should know about treatment. […] Epithelioid sarcoma (ES) is a rare form of soft tissue sarcoma (STS) that accounts for fewer than 1% of STS cases. The disease is known for being difficult to diagnose and treat and for being associated with high rates of local recurrence (34%-77%) and metastasis (40%). […] Given the ambiguous clinical features of ES, the diagnosis often hinges on microscopic appearance and genetic analysis. The distinctive mutation in ES is a loss of INI1 function, which is present in over 90% of tumors regardless of subtype. […] As with all suspected cases of STS, it is recommended that possible ES lesions be evaluated by collaborating members of a multidisciplinary team who are experienced in the management of sarcomas.

• #46 Epithelioid sarcoma – Wikipedia

  https://en.wikipedia.org/wiki/Epithelioid_sarcoma

  Epithelioid sarcoma is a rare soft tissue sarcoma arising from mesenchymal tissue and characterized by epithelioid-like features. It accounts for less than 1% of all soft tissue sarcomas. […] Tissue biopsy is the diagnostic modality of choice. Due to a high incidence of lymph node involvement, a sentinel lymph node biopsy may be performed. A common characteristic of epithelioid sarcoma (observed in 80% of all cases) is the loss of function of the SMARCB1 gene (whose protein product is termed BAF47, INI1, or hSNF5). Immunohistochemical staining of INI1 is available and helps to diagnose of epithelioid sarcoma. MRI is the diagnostic modality of choice for imaging prior to biopsy and pathologic diagnosis for most patients.

• #47 Soft Tissue Sarcomas | Choose the Right Test

  https://arupconsult.com/content/soft-tissue-sarcomas

  Molecular genetic testing may be useful to support the diagnosis and classification of soft tissue sarcomas. […] Laboratory testing for soft tissue sarcomas is appropriate in individuals with a biopsy specimen that is suspicious for sarcoma and requires additional information beyond what can be provided by a morphologic evaluation. […] For many soft tissue sarcomas, diagnosis and classification are based on histology and supported by immunohistochemical and molecular findings. […] Immunohistochemistry testing aids in the diagnosis and classification of soft tissue sarcomas. […] Genetic tests on tumor samples may be useful in addition to histology and immunohistochemistry in diagnosis and classification, and may be particularly beneficial in unusual cases or when the diagnosis is not definitive.

• #48 Recommendations from the Epithelioid Sarcoma Collaborative: A White Paper – Journal of Oncology Navigation & Survivorship

  https://www.jons-online.com/issues/2021/september-2021-vol-12-no-9/recommendations-from-the-epithelioid-sarcoma-collaborative-a-white-paper

  Epithelioid sarcoma (ES) is a rare soft tissue sarcoma (STS) first described in the 1970s by Enzinger. The rarity of ES leads to multiple challenges for patients diagnosed with ES beginning with but not limited to the diagnosis journey, confounded by the subdivision of patients into 2 subtypes distal (classic) ES and proximal-type ES, and by the presentation. The suite of nonspecific symptoms of ES necessitates pathologic diagnosis, which is done by examining a piece of tissue from the lump or growth. A confident diagnosis of ES is defined by the loss of INI1 expression. Improved ES patient staging and imaging would improve patients initial diagnoses and their subsequent treatment assessment. Currently, there are no consensus guidelines for the systemic treatment, staging, and imaging of ES patients.

• #49 Epithelioid Sarcoma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/449

  Epithelioid sarcoma is locally invasive and frequently metastasizes to regional lymph nodes and distant sites. Diagnosis is determined by tissue biopsy, while imaging studies are essential for assessing the extent of the primary tumor and identifying metastatic disease. […] A tissue biopsy is necessary, as soft tissue swellings have many possible causes. Core needle biopsies are the preferred method. Based on clinical examination, biopsies should also be taken from any suspicious lymph nodes. Magnetic resonance imaging (MRI) is the preferred diagnostic tool for most soft-tissue sarcomas, as it provides detailed images of the extent of tumor invasion into the surrounding fascial planes. […] Epithelioid sarcoma is associated with a high rate of distant metastases, with recent studies suggesting a distant metastasis rate of up to 50%. The most common sites of metastases are the lungs, lymph nodes, and skin. As a result, staging imaging with a chest computed tomography (CT) scan is typically performed.

• #50 Epithelioid sarcoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/epithelioid-sarcoma?embed_domain=Navimumbaihouses.com%2Fblog%2Fnews%2Fhow-youll-know-if-your-client-is-truly-motivated-to-buy%2F&lang=us

  Epithelioid sarcomas are diagnosed based on typical histological and molecular pathological features. […] Diagnostic criteria according to the WHO classification of soft tissue and bone tumors (5th edition): soft tissue mass (distal or proximal presentation), epithelioid to spindled cell morphology, infiltrative growth, SMARCB1/INI1 loss, immunoreactivity for epithelial membrane antigen (EMA) and keratin.

• #51 3 Things You Should Know About Identifying and Treating Epithelioid Sarcoma

  https://www.cancernetwork.com/view/3-things-you-should-know-about-identifying-and-treating-epithelioid-sarcoma

  Experts discuss epithelioid sarcoma diagnosis, management, and targeted therapies, and highlight 3 things everyone should know about treatment. […] Epithelioid sarcoma (ES) is a rare form of soft tissue sarcoma (STS) that accounts for fewer than 1% of STS cases. The disease is known for being difficult to diagnose and treat and for being associated with high rates of local recurrence (34%-77%) and metastasis (40%). […] Given the ambiguous clinical features of ES, the diagnosis often hinges on microscopic appearance and genetic analysis. The distinctive mutation in ES is a loss of INI1 function, which is present in over 90% of tumors regardless of subtype. […] As with all suspected cases of STS, it is recommended that possible ES lesions be evaluated by collaborating members of a multidisciplinary team who are experienced in the management of sarcomas.

• #52 Epithelioid Sarcoma | Symptoms, Diagnosis & Treatment

  https://www.cincinnatichildrens.org/health/e/epithelioid-sarcoma

  Epithelioid sarcoma is rare and commonly misdiagnosed. That is why it is so important for people who have this condition to receive care from specialists who have extensive experience in diagnosing and treating it. […] Patients will need a physical exam, blood work and imaging tests to diagnose epithelioid sarcoma. These tests help the doctor learn the exact size and location of the tumor and help to see if the cancer has spread. […] Patients will also have a biopsy where the doctor removes a small piece of the tumor. A specialist examines the tissue under a microscope to check for the SMARCB1 or INI-1 marker. The results may guide the team as it develops a treatment plan. […] Prompt diagnosis and treatment can greatly improve the outcome for patients with epithelioid sarcoma. That is why it is vitally important for patients to receive care at a hospital such as Cincinnati Childrens, whose sarcoma team has experience in diagnosing and treating this rare type of cancer.

• #53 Recommendations from the Epithelioid Sarcoma Collaborative: A White Paper – Journal of Oncology Navigation & Survivorship

  https://www.jons-online.com/issues/2021/september-2021-vol-12-no-9/recommendations-from-the-epithelioid-sarcoma-collaborative-a-white-paper

  Increasing access to expert, experienced sarcoma centers is an essential component of improving outcomes among patients with ES and includes partnerships with advocacy groups to support the cost of patient travel. We propose employing technological solutions to help close the geographical gap to help ensure ES patients have a sarcoma specialist and an expert pathologist involved in the diagnosis. The use of virtual tumor boards and telemedicine in diagnosis and management can make great inroads in closing the geographical gap. […] In conclusion, increasing awareness and understanding of ES is a necessary first step to improve the diagnosis journey by (1) decreasing the time to correct diagnosis; (2) reducing barriers to timely treatment and access to expert, experienced sarcoma centers; and (3) raising awareness among GPs and other care providers who may be the first point of contact for these patients. In addition, establishing a standard for ES diagnosis will create a consistent, reliable clinical evaluation threshold.

• #54 Recommendations from the Epithelioid Sarcoma Collaborative: A White Paper – Journal of Oncology Navigation & Survivorship

  https://www.jons-online.com/issues/2021/september-2021-vol-12-no-9/recommendations-from-the-epithelioid-sarcoma-collaborative-a-white-paper

  Increasing access to expert, experienced sarcoma centers is an essential component of improving outcomes among patients with ES and includes partnerships with advocacy groups to support the cost of patient travel. We propose employing technological solutions to help close the geographical gap to help ensure ES patients have a sarcoma specialist and an expert pathologist involved in the diagnosis. The use of virtual tumor boards and telemedicine in diagnosis and management can make great inroads in closing the geographical gap. […] In conclusion, increasing awareness and understanding of ES is a necessary first step to improve the diagnosis journey by (1) decreasing the time to correct diagnosis; (2) reducing barriers to timely treatment and access to expert, experienced sarcoma centers; and (3) raising awareness among GPs and other care providers who may be the first point of contact for these patients. In addition, establishing a standard for ES diagnosis will create a consistent, reliable clinical evaluation threshold.

• #55 EPITHELIOID SARCOMA

  https://www.seyitaligumustas.com/en/epithelioid-sarcoma

  Epithelioid sarcoma is a very rare (less than 1% of all soft tissue sarcomas) malignant soft tissue tumor. […] The diagnosis of epithelioid sarcoma is confirmed with a biopsy after physical examination and imaging studies have been completed. The biopsy is often performed using a closed procedure with special needles. […] After the diagnosis of epithelioid sarcoma, PET-CT or tomography (chest, abdomen, and pelvis) is performed for grading. […] The primary treatment for epithelioid sarcoma is removal of the tumor with clean margins (wide resection). […] Adding radiotherapy (high dose and large area) to surgical treatment before or after surgery reduces the chance of tumor recurrence. […] In patients with epithelioid sarcoma, systemic chemotherapy is recommended for locally advanced or metastatic cases.

• #56 EPITHELIOID SARCOMA

  https://www.seyitaligumustas.com/en/epithelioid-sarcoma

  Epithelioid sarcoma is a very rare (less than 1% of all soft tissue sarcomas) malignant soft tissue tumor. […] The diagnosis of epithelioid sarcoma is confirmed with a biopsy after physical examination and imaging studies have been completed. The biopsy is often performed using a closed procedure with special needles. […] After the diagnosis of epithelioid sarcoma, PET-CT or tomography (chest, abdomen, and pelvis) is performed for grading. […] The primary treatment for epithelioid sarcoma is removal of the tumor with clean margins (wide resection). […] Adding radiotherapy (high dose and large area) to surgical treatment before or after surgery reduces the chance of tumor recurrence. […] In patients with epithelioid sarcoma, systemic chemotherapy is recommended for locally advanced or metastatic cases.

• #57 Epithelioid Sarcoma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/449

  Epithelioid sarcoma is locally invasive and frequently metastasizes to regional lymph nodes and distant sites. Diagnosis is determined by tissue biopsy, while imaging studies are essential for assessing the extent of the primary tumor and identifying metastatic disease. […] A tissue biopsy is necessary, as soft tissue swellings have many possible causes. Core needle biopsies are the preferred method. Based on clinical examination, biopsies should also be taken from any suspicious lymph nodes. Magnetic resonance imaging (MRI) is the preferred diagnostic tool for most soft-tissue sarcomas, as it provides detailed images of the extent of tumor invasion into the surrounding fascial planes. […] Epithelioid sarcoma is associated with a high rate of distant metastases, with recent studies suggesting a distant metastasis rate of up to 50%. The most common sites of metastases are the lungs, lymph nodes, and skin. As a result, staging imaging with a chest computed tomography (CT) scan is typically performed.

• #58 EPITHELIOID SARCOMA

  https://www.seyitaligumustas.com/en/epithelioid-sarcoma

  The average 5-year survival of patients with epithelioid sarcoma is 70% for patients with local disease and 50% for patients with regional lymph node involvement. […] The prognosis is worse in the presence of tumor diameter 5cm, regional lymphadenopathy, vascular invasion, necrosis rate 30%, local symptoms, positive surgical margins and metastases.

• #59 Epithelioid sarcoma: A diagnostic challenge – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/epithelioid-sarcoma-a-diagnostic-challenge/

  Epithelioid sarcoma is an uncommon slow-growing soft tissue malignancy, associated with a high incidence of local recurrence and metastasis. […] The tumor is remarkable for the diagnostic difficulties it poses, both clinically and histologically, resulting in a high frequency of initial misdiagnosis. […] Epithelioid sarcoma has a tendency to spread locally by way of lymphatics or along fascial planes and may give rise to multiple local nodules. […] Diagnosis is made by typical histological features of distinct nodular aggregates of epithelioid and spindle cells with zonal necrosis. […] Epithelioid sarcoma can be differentiated from synovial sarcoma by the absence of pseudoglandular structures and tendency to ulcerate. […] The present case was misdiagnosed as cutaneous tuberculosis elsewhere and the superficial biopsy of the lesion in our hospital was reported as epithelioid hemangioendothelioma. Diagnosis of epithelioid sarcoma was however established by a deeper biopsy because of its typical histopathological features. […] Prognosis is dependent on the depth of the tumor in relation to the deep fascia, local recurrence and regional lymph node involvement. […] The treatment of primary disease is wide local excision, followed by adjuvant radiotherapy, in an attempt to lower the risk of local recurrence.

• #60 EPITHELIOID SARCOMA

  https://www.seyitaligumustas.com/en/epithelioid-sarcoma

  The average 5-year survival of patients with epithelioid sarcoma is 70% for patients with local disease and 50% for patients with regional lymph node involvement. […] The prognosis is worse in the presence of tumor diameter 5cm, regional lymphadenopathy, vascular invasion, necrosis rate 30%, local symptoms, positive surgical margins and metastases.

• #61 Epithelioid Sarcoma: An overview with emphasis on its differentiation from morphologic mimics – JDPO

  https://www.jdpo.org/html-article/15946

  Most of the studies have shown that ES has characteristic co-expression of epithelial markers like CK, EMA and mesenchymal markers like Vimentin, CD 34 (in approximately 50% cases). Interestingly Ca 125, initially supposed to be ovarian surface epithelial marker; has been consistently reported in cases of ES, irrespective of subtype. Loss of nuclear expression of SMARC B1 protein/ INI 1 has also been uniformly observed in both types of ES. […] The mainstay of treatment is surgery with adjuvant chemotherapy and radiotherapy and local recurrence as high as 70 %, often as multiple nodules, is the major cause of treatment failure. Adverse prognostic factors in both CES and PES include male sex, older age, axial or proximal extremity location, involvement of deep soft tissue, tumor size more than 5 cm, tumor multifocality, high mitotic activity, nodal involvement and extensive necrosis.

• #62 EPITHELIOID SARCOMA

  https://www.seyitaligumustas.com/en/epithelioid-sarcoma

  The average 5-year survival of patients with epithelioid sarcoma is 70% for patients with local disease and 50% for patients with regional lymph node involvement. […] The prognosis is worse in the presence of tumor diameter 5cm, regional lymphadenopathy, vascular invasion, necrosis rate 30%, local symptoms, positive surgical margins and metastases.

• #63 Epithelioid Sarcoma: Symptoms, Treatment & Prognosis

  https://my.clevelandclinic.org/health/diseases/24331-epithelioid-sarcoma

  Epithelioid sarcoma is a rare subtype of soft tissue sarcoma. […] A healthcare provider will run several tests to diagnose ES, which may include: […] A physical examination. […] Biopsy. […] Blood tests for cancer. […] Magnetic resonance imaging (MRI). […] Computed tomography (CT) scans. […] Positron emission tomography (PET) scans. […] Epithelioid sarcoma is treatable, especially when detected early. […] Your healthcare provider will run several tests to determine how big the tumor is and whether it has spread to other areas of your body. […] The only way to cure ES is to completely remove the tumor. […] If a surgeon is unable to completely remove a tumor, theyll likely recommend a combination of treatments to manage your condition. […] Many research studies have reported on epithelioid sarcoma survival rates through the years. […] The five-year survival rate for epithelioid sarcoma ranges from 25% to 92%, depending on which study you read.

• #64 EPITHELIOID SARCOMA

  https://www.seyitaligumustas.com/en/epithelioid-sarcoma

  Epithelioid sarcoma is a very rare (less than 1% of all soft tissue sarcomas) malignant soft tissue tumor. […] The diagnosis of epithelioid sarcoma is confirmed with a biopsy after physical examination and imaging studies have been completed. The biopsy is often performed using a closed procedure with special needles. […] After the diagnosis of epithelioid sarcoma, PET-CT or tomography (chest, abdomen, and pelvis) is performed for grading. […] The primary treatment for epithelioid sarcoma is removal of the tumor with clean margins (wide resection). […] Adding radiotherapy (high dose and large area) to surgical treatment before or after surgery reduces the chance of tumor recurrence. […] In patients with epithelioid sarcoma, systemic chemotherapy is recommended for locally advanced or metastatic cases.

• #65 3 Things You Should Know About Identifying and Treating Epithelioid Sarcoma

  https://www.cancernetwork.com/view/3-things-you-should-know-about-identifying-and-treating-epithelioid-sarcoma

  Wide surgical excision of the tumor with neoadjuvant or adjuvant radiation treatment of the tumor bed is the primary treatment of choice in most cases of ES. […] Disease that recurs, metastasizes, or is diagnosed at an advanced stage is often treated with some form of systemic therapy (eg, traditional chemotherapy or targeted medication). […] The use of systemic treatment for STS has traditionally been controversial, and it is reserved for advanced cases. […] Mechanistic insight into the root cause of ES has led to the development of a promising targeted treatment, however. As mentioned above, over 90% of ES tumors demonstrate a loss of INI1 function. […] This has led to NCCN guidelines listing tazemetostat as the preferred treatment for recurrent, metastatic, or locally advanced and unresectable ES.

• #66 Distal and proximal epithelioid sarcoma — differences in diagnosis and similarities in treatment | Krotewicz | Oncology in Clinical Practice

  https://journals.viamedica.pl/oncology_in_clinical_practice/article/view/99119

  Epithelioid sarcoma (ES) comprises two subtypes, distal and proximal. […] The differences in clinical presentation underscore the necessity for tailored treatment approaches for each ES subtype. […] Tazemetostat, an oral selective inhibitor of the histone methyltransferase enhancer of zeste homolog 2 (EZH2), has recently gained FDA approval as a first-line treatment for ES patients. […] Epithelioid sarcoma: Diagnostic Features and Genetics. […] Epithelioid sarcoma: a review and update.

• #67 3 Things You Should Know About Identifying and Treating Epithelioid Sarcoma

  https://www.cancernetwork.com/view/3-things-you-should-know-about-identifying-and-treating-epithelioid-sarcoma

  Wide surgical excision of the tumor with neoadjuvant or adjuvant radiation treatment of the tumor bed is the primary treatment of choice in most cases of ES. […] Disease that recurs, metastasizes, or is diagnosed at an advanced stage is often treated with some form of systemic therapy (eg, traditional chemotherapy or targeted medication). […] The use of systemic treatment for STS has traditionally been controversial, and it is reserved for advanced cases. […] Mechanistic insight into the root cause of ES has led to the development of a promising targeted treatment, however. As mentioned above, over 90% of ES tumors demonstrate a loss of INI1 function. […] This has led to NCCN guidelines listing tazemetostat as the preferred treatment for recurrent, metastatic, or locally advanced and unresectable ES.

• #68 Epithelioid sarcoma: A diagnostic challenge – Indian Journal of Dermatology, Venereology and Leprology

  https://ijdvl.com/epithelioid-sarcoma-a-diagnostic-challenge/

  Epithelioid sarcoma is an uncommon slow-growing soft tissue malignancy, associated with a high incidence of local recurrence and metastasis. […] The tumor is remarkable for the diagnostic difficulties it poses, both clinically and histologically, resulting in a high frequency of initial misdiagnosis. […] Epithelioid sarcoma has a tendency to spread locally by way of lymphatics or along fascial planes and may give rise to multiple local nodules. […] Diagnosis is made by typical histological features of distinct nodular aggregates of epithelioid and spindle cells with zonal necrosis. […] Epithelioid sarcoma can be differentiated from synovial sarcoma by the absence of pseudoglandular structures and tendency to ulcerate. […] The present case was misdiagnosed as cutaneous tuberculosis elsewhere and the superficial biopsy of the lesion in our hospital was reported as epithelioid hemangioendothelioma. Diagnosis of epithelioid sarcoma was however established by a deeper biopsy because of its typical histopathological features. […] Prognosis is dependent on the depth of the tumor in relation to the deep fascia, local recurrence and regional lymph node involvement. […] The treatment of primary disease is wide local excision, followed by adjuvant radiotherapy, in an attempt to lower the risk of local recurrence.

• #69 Recommendations from the Epithelioid Sarcoma Collaborative: A White Paper – Journal of Oncology Navigation & Survivorship

  https://www.jons-online.com/issues/2021/september-2021-vol-12-no-9/recommendations-from-the-epithelioid-sarcoma-collaborative-a-white-paper

  Epithelioid sarcoma (ES) is a rare soft tissue sarcoma (STS) first described in the 1970s by Enzinger. The rarity of ES leads to multiple challenges for patients diagnosed with ES beginning with but not limited to the diagnosis journey, confounded by the subdivision of patients into 2 subtypes distal (classic) ES and proximal-type ES, and by the presentation. The suite of nonspecific symptoms of ES necessitates pathologic diagnosis, which is done by examining a piece of tissue from the lump or growth. A confident diagnosis of ES is defined by the loss of INI1 expression. Improved ES patient staging and imaging would improve patients initial diagnoses and their subsequent treatment assessment. Currently, there are no consensus guidelines for the systemic treatment, staging, and imaging of ES patients.

• #70 Navigating Diagnostic Challenges in Epithelioid Sarcoma: Multidisciplinary Strategies to Optimize Diagnosis

  https://www.cancernetwork.com/view/navigating-diagnostic-challenges-in-epithelioid-sarcoma-multidisciplinary-strategies-to-optimize-diagnosis

  Diagnosing epithelioid sarcoma (ES) is often challenging due to its rarity, variable presentation, and histologic overlap with other conditions. […] Common diagnostic pitfalls include misinterpreting ES as benign lesions, such as granulomatous inflammation or reactive processes, especially in the early stages. […] A key diagnostic marker is loss of INI1 (SMARCB1), which should be assessed in cases with ambiguous histology. […] Awareness of these diagnostic nuances and incorporating molecular testing early in the workup can significantly improve diagnostic accuracy.

• #71 Recommendations from the Epithelioid Sarcoma Collaborative: A White Paper – Journal of Oncology Navigation & Survivorship

  https://www.jons-online.com/issues/2021/september-2021-vol-12-no-9/recommendations-from-the-epithelioid-sarcoma-collaborative-a-white-paper

  Increasing access to expert, experienced sarcoma centers is an essential component of improving outcomes among patients with ES and includes partnerships with advocacy groups to support the cost of patient travel. We propose employing technological solutions to help close the geographical gap to help ensure ES patients have a sarcoma specialist and an expert pathologist involved in the diagnosis. The use of virtual tumor boards and telemedicine in diagnosis and management can make great inroads in closing the geographical gap. […] In conclusion, increasing awareness and understanding of ES is a necessary first step to improve the diagnosis journey by (1) decreasing the time to correct diagnosis; (2) reducing barriers to timely treatment and access to expert, experienced sarcoma centers; and (3) raising awareness among GPs and other care providers who may be the first point of contact for these patients. In addition, establishing a standard for ES diagnosis will create a consistent, reliable clinical evaluation threshold.

• #72 Recommendations from the Epithelioid Sarcoma Collaborative: A White Paper – Journal of Oncology Navigation & Survivorship

  https://www.jons-online.com/issues/2021/september-2021-vol-12-no-9/recommendations-from-the-epithelioid-sarcoma-collaborative-a-white-paper

  Increasing access to expert, experienced sarcoma centers is an essential component of improving outcomes among patients with ES and includes partnerships with advocacy groups to support the cost of patient travel. We propose employing technological solutions to help close the geographical gap to help ensure ES patients have a sarcoma specialist and an expert pathologist involved in the diagnosis. The use of virtual tumor boards and telemedicine in diagnosis and management can make great inroads in closing the geographical gap. […] In conclusion, increasing awareness and understanding of ES is a necessary first step to improve the diagnosis journey by (1) decreasing the time to correct diagnosis; (2) reducing barriers to timely treatment and access to expert, experienced sarcoma centers; and (3) raising awareness among GPs and other care providers who may be the first point of contact for these patients. In addition, establishing a standard for ES diagnosis will create a consistent, reliable clinical evaluation threshold.

• #73 Recommendations from the Epithelioid Sarcoma Collaborative: A White Paper – Journal of Oncology Navigation & Survivorship

  https://www.jons-online.com/issues/2021/september-2021-vol-12-no-9/recommendations-from-the-epithelioid-sarcoma-collaborative-a-white-paper

  Overcoming delays inherent in each interval of the diagnosis journey is recommended. A first step of increasing awareness of STS overall among patients and providers may be to reduce the patient delay in recognizing symptoms of ES. Increased awareness coupled with providing ES resources and educating healthcare providers about the different types of STS may also reduce the GP delay in considering the presentation as a possible cancer and improving the patient-care partner-physician dialogue. Finally, overcoming system delays to increase timely medical care and reduce misdiagnosis or delays in diagnosis necessitates solutions to the cascade of delays. One proposed solution is to establish an ES standard of diagnosis for widespread adoption among healthcare providers that includes a recommended timeline, such as a 6-week follow-up and imaging, followed by a second opinion, followed by diagnosis confirmed by an expert pathologist.

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