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. This condition most commonly affects young adults and typically presents as a painless and slow-growing mass, which is often located in the distal extremities. Despite its slow growth, epithelioid sarcoma is highly aggressive, posing a significant risk of recurrence, lymph node involvement, and distant metastases. […] Epithelioid sarcoma presents as a slow-growing, nodular lesion, most commonly involving the distal upper extremity, particularly the fingers, hands, and forearm. Lesions are usually painless and enlarge slowly, developing a multinodular appearance. Ulceration of the overlying skin and invasion of surrounding structures are common. […] 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.

• #1 Epithelioid Sarcoma: Symptoms, Treatment & Prognosis

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

  Epithelioid sarcoma is slow growing. A person can have this type of cancer for months (or even years) before noticing any symptoms. […] In the early stages, ES may not cause symptoms. As the tumor continues to grow, you may begin to notice these epithelioid sarcoma symptoms: A new lump or area of swelling (which can range from small to large). Open wounds (ulcerations) on your skin above the lump. Pain (if the tumor puts pressure on muscles or nerves). An existing lump that increases in size. […] 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. Then, theyll design a personalized treatment plan. […] The only way to cure ES is to completely remove the tumor. But complete removal isnt possible in all cases. […] Because ES is slow growing, many people with the disease can still lead long, fulfilling lives.

• #1 Epithelioid Sarcoma: Symptoms, Causes, Treatment | Qwark

  https://qwarkhealth.com/conditions/epithelioid-sarcoma

  Epithelioid Sarcoma is a rare type of soft tissue cancer that can occur in different parts of the body, most commonly in the hands and feet. Some of the common symptoms associated with Epithelioid Sarcoma include the development of a small, painless lump or nodule on the skin or soft tissue, which can gradually grow in size and become painful. Other symptoms may include stiffness or limited mobility in the affected area, swelling, and ulceration of the skin over the tumor. In advanced stages, the cancer can also cause fatigue, weight loss, and other non-specific symptoms. […] The symptoms of Epithelioid Sarcoma can take months or even years to appear after initial exposure. This is because the cancer develops slowly over time and may not cause any noticeable symptoms until it has grown to a certain size. In some cases, the tumor may be present for several years before it becomes symptomatic.

• #1 Epithelioid sarcoma | Altru Health System

  https://www.altru.org/health-library/conditions/epithelioid-sarcoma

  Epithelioid sarcoma symptoms include a small, firm growth under the skin called a nodule. The nodule usually isn’t painful or tender. Sometimes there is more than one nodule. Sometimes the nodule forms a sore on the skin that won’t heal. […] The nodules caused by epithelioid sarcoma most often grow on the fingers, hands or forearms. They also may grow on the knee or lower leg. […] Epithelioid sarcoma tends to grow slowly. It can come back after treatment.

• #1 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-9/3914-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. Classic ES is more frequent in adolescent and young adult (AYA) males, commonly affects the upper extremity, and is typically present as superficial, nonpainful, indolent growths on the hands and arms followed by chronic nonhealing ulcers with raised margins. Proximal-type ES is less frequently diagnosed than classic ES and can affect young and middle-aged adults and is also slightly more common among males. Proximal-type ES tends to present as deep soft tissue masses found in locations such as the trunk, pelvis, proximal limbs, and limb girdles presenting as deep or visceral tumors. The painless quality of the lump or growth/soft tissue mass can lead patients and clinicians to initially characterize the growth as benign. 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. Unfortunately, ES frequently recurs, with classic ES recurring as multinodular soft tissue masses, whereas proximal-type ES is associated with spread of the disease to distant locations (metastatic disease). Unlike other STSs, ES has a high incidence of lymphatic or regional metastases as it metastasizes via the lymphatic system. Improved ES patient staging and imaging would improve patients initial diagnoses and their subsequent treatment assessment. Wide surgical resection is the most recommended treatment modality for localized disease. Adjuvant radiation therapy has not had a role for assisting with local control in addition to surgical resection. Traditional systemic therapy with cytotoxic agents has demonstrated limited efficacy, with no single chemotherapy regimen identified as the preferred treatment. Currently, there are no consensus guidelines for the systemic treatment, staging, and imaging of ES patients. Current treatment options provide little hope for patients with advanced disease, who face inevitable progression and death.

• #1 Epithelioid Sarcoma: Symptoms, Causes & Treatment

  https://www.medicoverhospitals.in/diseases/epithelioid-sarcoma/

  Epithelioid sarcoma is a rare and aggressive soft tissue cancer that primarily affects young adults. […] Early detection of epithelioid sarcoma symptoms can significantly impact the treatment and prognosis. Therefore, understanding the presenting signs is essential for timely intervention. […] Patients may notice firm nodules or masses in the affected area. These growths can be painful or painless and are often mistaken for benign conditions. […] The skin overlying the nodules may ulcerate, leading to open sores that can become infected. […] As the tumor progresses, swelling and redness may occur due to inflammation and tissue reaction. […] Tumors located near joints may restrict movement, causing discomfort and functional impairment. […] Symptoms may include a firm, painless mass in the soft tissues, potential local pain, and swelling, often requiring prompt evaluation for potential malignancy. […] Prognosis varies; many factors influence outcomes, including tumor size, location, and response to treatment, making early diagnosis and intervention crucial.

• #1 New Soft Tissue Sarcoma treatments 2025 | Everyone.org

  https://everyone.org/explore/treatment/?id=18

  In cases where the tumor compresses major nerves, patients may experience significant functional impairment, including difficulty walking, holding objects, or performing routine activities. Neurological symptoms often prompt patients to seek medical attention, leading to earlier diagnosis in some cases. […] Large or rapidly growing soft tissue sarcomas can cause systemic symptoms, especially if they affect surrounding tissues or organs. Patients may experience fatigue, unexplained weight loss, or a general feeling of illness or malaise. These symptoms are often nonspecific and may be overlooked or attributed to other conditions initially. […] Rapidly enlarging tumors may also cause noticeable changes in body shape or symmetry, particularly if the tumor is located in a limb or visible area. Patients may notice clothing fitting differently or difficulty performing certain movements or activities due to tumor size and location.

• #1 Epithelioid sarcoma | Czarnecka | Nowotwory. Journal of Oncology

  https://journals.viamedica.pl/nowotwory_journal_of_oncology/article/view/NJO.a2023.0021

  Epithelioid sarcoma (ES) is a very rare sarcoma characterized by loss of INI1. […] ES symptoms and signs are not specific and depend on tumor localization. […] Epithelioid sarcoma symptoms and signs are not specific and depend on tumor location, therefore include a lump or swelling in the area, with masses greater than 20 cm, slightly mobile tumors, painful on palpation and without skin changes, or ulcerated and indurated lesions, but also rectum bleeding, vaginal bleeding, epistaxis, hemoptysis, nausea, vomiting, abdominal pain, abdominal fullness, ptosis, headaches, neck pain, eye pain and swelling, diarrhea or constipation, depression, anorexia, weight loss, or fever. […] Most often, at first, ES presents as a slowly growing, painless, and firm nodule, but the course of ES is unpredictable, including rapid progression with extensive lymph node or distant metastasis development.

• #1 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. The symptoms are similar to those of nerve compression diseases. […] The symptoms and signs are similar to those of the nerve compression diseases. The relative cases are extremely rarely reported in literature. These situations are easy to be ignored and misdiagnosed in clinic and may bring out severe outcomes. […] The first case involved the median nerve around the elbow. The symptoms of the patient presented mimic as carpal tunnel syndrome. […] The second case involved the ulnar nerve at the forearm. The patients symptoms were presented mimic cubital tunnel syndrome and misdiagnosed at the local hospital and underwent ulnar nerve decompression at the elbow.

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

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

  The third and the fourth case involved the ulnar nerve around the elbow, and the symptoms presented as cubital tunnel syndrome at first. […] The fifth case involved the deep branch of the radial nerve at the forearm. The patient presented as the muscle strength decline of finger extension. […] The sixth case involved the radial nerve at the arm. The symptom of the patient presented as lacking the strength of extension the wrist and fingers and feeling numbness of the dorsal radial side of the hand. […] The seventh case involved the branches of the brachial plexus, including ulnar nerve, median nerve and radial nerve. The patient felt numbness of his fingers and went to the local hospital. […] The eighth case involved the lateral cord of brachial plexus. The symptoms of the patient presented as the weakness of the elbow flexion and the numbness of the radial side of the hand.

• #1 Epithelioid Sarcoma – StatPearls – NCBI Bookshelf

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

  Local recurrence rates of 40% to 60% have been reported, with a median time to recurrence of 1 to 2 years. However, recurrences have been reported as late as 20 years after the initial operation. As a result, close surveillance with cross-sectional imaging of the primary site and lungs is critical, typically with imaging studies every 6 months to 1 year. […] Prognosis, as with most malignancies, is primarily determined by the clinical stage of the disease. Epithelioid sarcoma prognosis is most closely associated with tumor size, vascular invasion, resectability, and metastases. Large tumor size and early metastases are independently associated with poor outcomes.

• #1 Epithelioid sarcoma | Czarnecka | Nowotwory. Journal of Oncology

  https://journals.viamedica.pl/nowotwory_journal_of_oncology/article/view/NJO.a2023.0021

  The 5-year risk of recurrence after radical treatment is high, up to 70%. […] The proximal subtype of ES is more aggressive, has higher rates of recurrence and metastases, and generally worse prognosis and higher mortality compared to classical ES. […] Local recurrences are common in ES and most often develop within six months after radical treatment. Up to 75% of cases with local recurrence also develop distant metastases. […] However, most patients have an advanced stage at first diagnosis with lymph node and / or lung metastases. […] Epithelioid sarcoma metastasizes most frequently to the lungs or pleura. […] High-risk epithelioid sarcomas are patients with large tumors, high tumor grade, inadequate tumor resection, and metastatic disease, predicting a relatively poor clinical outcome.

• #1 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. Epithelioid sarcoma has an aggressive course, which means there is a high risk of recurrence after surgery and a high risk of spreading to other parts of the body (often lymph nodes and lungs). The average risk of recurrence after surgery is reported to be 50% (30-70) and the risk of metastasis is 40%. About half of recurrent tumors are reported to require amputation. There is also a risk of recurrence in patients who initially underwent amputation. […] Because epithelioid sarcoma is slow-growing and usually painless, the average time from onset to diagnosis is 10 (9-30) months. One third of patients have symptoms for more than two years. […] Due to the delay in diagnosis of epithelioid sarcoma, lymph node metastases are detected in approximately 1/3 of patients and lung metastases in 1/4 of patients at the time of initial admission.

• #1 Epithelioid Sarcoma—From Genetics to Clinical Practice

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7463637/

  Epithelioid sarcoma is a mesenchymal soft tissue sarcoma often arising in the extremities, usually in young adults with a pick of incidence at 35 years of age. […] ES is known for aggressive behavior, including a high recurrence rate and regional lymph node metastases. […] At presentation, the disease typically manifests as a painless, slow-growing, firm nodule deep in soft tissues with a glistening and gray-tan appearance characterized by superficial bleeding, necrosis, and ulcerations. […] Pain is reported by some patients, mainly if tumors localize in proximity to joints. […] Various morphology, symptoms, and signs lead to difficulties in ES diagnostics and often delay appropriate treatment. […] ES often metastasize to lymph nodes (up to 30%). […] Patients suffer from in-transit metastases and lymphatic spread.

• #1 Epithelioid Sarcoma—From Genetics to Clinical Practice

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7463637/

  Distant metastases most often develop in the lungs, bones, and brain. […] ES metastatic spread is reported in the course of the disease in 20% to 50% of patients. […] Around 20% of patients present with distant metastases already at the primary diagnosis. […] The survival of patients with ES is unsatisfactory. […] Depending on the study, the 5-year overall survival rates from 25% to 70%. […] Five years on from primary tumor diagnosis, metastatic spread is found in 30% to 75% of patients. […] The presence of lymph nodes metastases at the primary diagnosis and the presence of distant metastases is significantly correlated with the poor prognosis.

• #1 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. In patients with pulmonary metastases, the one-year survival rate is 50% and the five-year survival rate is unfortunately close to zero. […] 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.

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

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

  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. […] Local recurrence rates of 40% to 60% have been reported, with a median time to recurrence of 1 to 2 years. However, recurrences have been reported as late as 20 years after the initial operation. As a result, close surveillance with cross-sectional imaging of the primary site and lungs is critical, typically with imaging studies every 6 months to 1 year. […] Prognosis, as with most malignancies, is primarily determined by the clinical stage of the disease. Epithelioid sarcoma prognosis is most closely associated with tumor size, vascular invasion, resectability, and metastases. Large tumor size and early metastases are independently associated with poor outcomes.

• #1 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. These intervals can be broken into discrete stages to create a standardized framework of comparison, of which several models exist, but collectively will be referred to as the diagnosis journey in this white paper. 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 patient delay describes the period where the patient experiences and responds to symptoms. The nonspecific, nonpainful indolent growths, in the case of classic ES, and the deep, visceral location of growth in the case of proximal ES, adds an additional layer of delay for ES patients.

• #1 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-9/3914-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. These intervals can be broken into discrete stages to create a standardized framework of comparison, of which several models exist, but collectively will be referred to as the diagnosis journey in this white paper. 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 patient delay describes the period where the patient experiences and responds to symptoms. The nonspecific, nonpainful indolent growths, in the case of classic ES, and the deep, visceral location of growth in the case of proximal ES, adds an additional layer of delay for ES patients. The warning signs of STSs, which include a nontender, soft tissue mass that is bigger than a golf ball, are not widely known by patients and family members.

• #1 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-9/3914-recommendations-from-the-epithelioid-sarcoma-collaborative-a-white-paper

  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. In the case of ES patients, the clinicians who are first consulted tend to be primary care physicians, sports medicine doctors, or dermatologists who are typically not familiar with ES and sarcoma symptoms, and this can significantly delay diagnosis or lead to misdiagnosis, contributing to an extended GP or diagnostic delay. […] The cascade of delays and consequences along the diagnosis journey culminates in more aggressive malignancies and limited treatment options due to the growth of the tumor and/or metastasis, necessitating systemic treatment rather than surgical resection. […] 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 patientcare partnerphysician dialogue. […] Finally, overcoming system delays to increase timely medical care and reduce misdiagnosis or delays in diagnosis necessitates solutions to the cascade of delays.

• #1 An Overview of Epithelioid Sarcoma

  https://www.pharmacytimes.com/view/an-overview-of-epithelioid-sarcoma

  Epithelioid sarcoma (ES) is a rare, slow-growing subtype of STS that accounts for approximately 1% of all STS cases annually. […] Initially, ES forms as a hard lump in the soft tissue under the skin. […] There are 2 forms of ES: distal-type and proximal-type. Distal-type ES (also referred to as classic ES) is the more common, typically affecting teenagers and young adults. […] Proximal-type ES, the rarer variant, typically affects older adults and is associated with less favorable survival rates; it usually occurs in the pelvic area or abdomen. […] The 5-year overall survival for ES varies from 25% to 78%, dependent upon staging at the time of initial diagnosis.

• #1 Epithelioid Sarcoma | Symptoms, Diagnosis & Treatment

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

  Epithelioid sarcoma is a type of soft tissue sarcoma that typically appears in the extremities (especially in the arms and hands). It also can develop in the main part of the body. It can affect both children and adults but is most common in young adulthood. […] People who have epithelioid sarcoma usually notice a lump or mass somewhere in the soft tissue of their body. The mass can range in size from small to large. You can feel and sometimes see it. Sores or ulcers may appear over the mass or lump. […] Epithelioid sarcoma can be present for months or even years before a person notices a mass. […] In most cases, epithelioid sarcoma does not spread. If it does spread, lymph nodes, the lungs and bones are the most common sites. […] 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. […] Most people who are treated for epithelioid sarcoma before the cancer spreads fare well. But the disease is more challenging to treat if it has spread. Other factors, such as the gender or age of the patient, do not seem to affect the outcome significantly.

• #1 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

  The prognosis for patients who qualify for systemic treatment is generally poor, with a median life expectancy of 8 months for metastatic ES. Historically, systemic treatment for advanced cases of ES has not been particularly promising. […] 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. INI1 is a tumor suppressor that inhibits the EZH2 enzyme, indirectly stimulating the transcription of tumor suppressor genes. Tazemetostat is a novel EZH2 inhibitor that was developed to treat ES.

• #1 Epithelioid sarcoma | Czarnecka | Nowotwory. Journal of Oncology

  https://journals.viamedica.pl/nowotwory_journal_of_oncology/article/view/NJO.a2023.0021

  The analysis of 74 patients with ES has shown that patients receiving first-line systemic therapy have ORR of 15%, disease control rate (DCR) of 20%, and a median duration of response (DOR) of 3.3 months. […] Epithelioid sarcoma is built by pleomorphic epithelioid cells and the proximal subtype is more aggressive than the classical subtype, as it has higher recurrence and metastasis rates, and shorter overall survival.

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

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

  Epithelioid sarcoma is a rare and aggressive soft tissue sarcoma characterized by both epithelial and mesenchymal differentiation. This condition most commonly affects young adults and typically presents as a painless and slow-growing mass often located in the distal extremities. 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. […] Epithelioid sarcoma presents as a slow-growing, nodular lesion, most commonly involving the distal upper extremity, particularly the fingers, hands, and forearm. Lesions are usually painless and enlarge slowly, developing a multinodular appearance. Ulceration of the overlying skin and invasion of surrounding structures are common.

• #2

  https://content.irisoncology.com/library/epithelioid-sarcoma

  Epithelioid sarcoma is a rare type of cancer that starts as a growth of cells in the soft tissue. It can happen anywhere on the body. It often starts under the skin on the finger, hand, forearm, knee or lower leg. […] Epithelioid sarcoma tends to grow slowly. It can come back after treatment. […] Epithelioid sarcoma symptoms include a small, firm growth under the skin called a nodule. The nodule usually isn’t painful or tender. Sometimes there is more than one nodule. Sometimes the nodule forms a sore on the skin that won’t heal. […] The nodules caused by epithelioid sarcoma most often grow on the fingers, hands or forearms. They also may grow on the knee or lower leg.

• #2 What Is Epithelioid Sarcoma? – Support finding a pediatric cancer cure

  https://sarcomafree.org/epithelioid-sarcoma/

  Epithelioid sarcoma may not cause noticeable symptoms in its early stages. As the tumor grows, common symptoms include: […] A new lump or swelling, which may be small or large. […] Open wounds (ulcerations) on the skin over the lump. […] Pain, especially if the tumor presses on nerves or muscles. […] An existing lump that increases in size. […] Because ES grows slowly, many people live fulfilling lives even with the condition.

• #2 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. […] Symptoms of epithelioid sarcoma can vary depending on the size and location of your tumour. You may experience all, some or none of these symptoms before you are diagnosed with epithelioid sarcoma: A lump or swelling within the soft tissue of the body under the skin, often in the hands, arms, feet and legs. This lump is usually hard but slow-growing and painless. These lumps often result in open sores called ulcers on the skin surface, which can be painful. […] Epithelioid sarcoma can reappear in the same area after the treatment of a previous tumour; this is called a local recurrence. If the cancer does reappear, it is important to get treated as quickly as possible. This could involve further surgery and/or radiotherapy; your treatment will be assessed on an individual basis.

• #2 Types of Soft Tissue Sarcoma | Memorial Sloan Kettering Cancer Center

  https://www.mskcc.org/cancer-care/types/soft-tissue-sarcoma/types

  Epithelioid sarcoma: These tumors are more common in young adults. The classic form of the disease grows slowly and occurs in the feet, arms, legs, or forearms of younger men. Epithelioid tumors can also begin in the groin, and these tumors tend to be more aggressive.

• #2 :: JKSR :: Journal of the Korean Society of Radiology

  https://jksronline.org/DOIx.php?id=10.3348/jksr.2015.72.6.405

  The two clinicopathological subtypes have different clinical and histological manifestations. The distal-type, which accounts for twice as many cases as the proximal-type, usually shows slow growing painless nodules. […] Even though, the patient in our case was confirmed histopathologically to have the classic-type, the patient had a 3-month history of progressing pain in the lower leg with multiple bone, lymph node and lung metastases. […] Metastases from epithelioid sarcomas develop in approximately 40-50% of cases, with involvement of the lung, lymph nodes and, less frequently, the scalp, adrenal gland and stomach. […] Our patient had a classic-type tumor, showing ipsilateral popliteal, inguinal and external iliac lymph node enlargement with FDG uptake. Additional multiple metastases were noted in the lung and bones.

• #2 Epithelioid Sarcoma: Symptoms, Causes & Treatment

  https://www.medicoverhospitals.in/diseases/epithelioid-sarcoma/

  Epithelioid sarcoma is a rare and aggressive soft tissue cancer that primarily affects young adults. […] Early detection of epithelioid sarcoma symptoms can significantly impact the treatment and prognosis. Therefore, understanding the presenting signs is essential for timely intervention. […] Patients may notice firm nodules or masses in the affected area. These growths can be painful or painless and are often mistaken for benign conditions. […] The skin overlying the nodules may ulcerate, leading to open sores that can become infected. […] As the tumor progresses, swelling and redness may occur due to inflammation and tissue reaction. […] Tumors located near joints may restrict movement, causing discomfort and functional impairment. […] Symptoms may include a firm, painless mass in the soft tissues, potential local pain, and swelling, often requiring prompt evaluation for potential malignancy. […] Prognosis varies; many factors influence outcomes, including tumor size, location, and response to treatment, making early diagnosis and intervention crucial.

• #2 New Soft Tissue Sarcoma treatments 2025 | Everyone.org

  https://everyone.org/explore/treatment/?id=18

  Symptoms can vary significantly based on the specific location of the sarcoma. Tumors located in the limbs may cause noticeable swelling or a visible lump beneath the skin, often without pain initially. As the tumor enlarges, it may cause discomfort, limited mobility, or difficulty using the affected limb. Patients may notice stiffness or difficulty moving joints near the tumor site. […] When soft tissue sarcoma develops in the abdomen, symptoms may be less obvious initially. Patients may experience abdominal pain or discomfort, bloating, or a sensation of fullness even after eating small amounts of food. Tumors in the abdomen can also lead to nausea, vomiting, or changes in bowel habits, such as constipation or diarrhea. In some cases, abdominal sarcomas may grow significantly before being detected, due to their deep location and absence of early symptoms.

• #2 Epithelioid sarcoma | Czarnecka | Nowotwory. Journal of Oncology

  https://journals.viamedica.pl/nowotwory_journal_of_oncology/article/view/NJO.a2023.0021

  Epithelioid sarcoma (ES) is a very rare sarcoma characterized by loss of INI1. […] ES symptoms and signs are not specific and depend on tumor localization. […] Epithelioid sarcoma symptoms and signs are not specific and depend on tumor location, therefore include a lump or swelling in the area, with masses greater than 20 cm, slightly mobile tumors, painful on palpation and without skin changes, or ulcerated and indurated lesions, but also rectum bleeding, vaginal bleeding, epistaxis, hemoptysis, nausea, vomiting, abdominal pain, abdominal fullness, ptosis, headaches, neck pain, eye pain and swelling, diarrhea or constipation, depression, anorexia, weight loss, or fever. […] Most often, at first, ES presents as a slowly growing, painless, and firm nodule, but the course of ES is unpredictable, including rapid progression with extensive lymph node or distant metastasis development.

• #2 New Soft Tissue Sarcoma treatments 2025 | Everyone.org

  https://everyone.org/explore/treatment/?id=18

  In cases where the tumor compresses major nerves, patients may experience significant functional impairment, including difficulty walking, holding objects, or performing routine activities. Neurological symptoms often prompt patients to seek medical attention, leading to earlier diagnosis in some cases. […] Large or rapidly growing soft tissue sarcomas can cause systemic symptoms, especially if they affect surrounding tissues or organs. Patients may experience fatigue, unexplained weight loss, or a general feeling of illness or malaise. These symptoms are often nonspecific and may be overlooked or attributed to other conditions initially. […] Rapidly enlarging tumors may also cause noticeable changes in body shape or symmetry, particularly if the tumor is located in a limb or visible area. Patients may notice clothing fitting differently or difficulty performing certain movements or activities due to tumor size and location.

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

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

  The third and the fourth case involved the ulnar nerve around the elbow, and the symptoms presented as cubital tunnel syndrome at first. […] The fifth case involved the deep branch of the radial nerve at the forearm. The patient presented as the muscle strength decline of finger extension. […] The sixth case involved the radial nerve at the arm. The symptom of the patient presented as lacking the strength of extension the wrist and fingers and feeling numbness of the dorsal radial side of the hand. […] The seventh case involved the branches of the brachial plexus, including ulnar nerve, median nerve and radial nerve. The patient felt numbness of his fingers and went to the local hospital. […] The eighth case involved the lateral cord of brachial plexus. The symptoms of the patient presented as the weakness of the elbow flexion and the numbness of the radial side of the hand.

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

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

  The ninth case involved the lower trunk of brachial plexus and the symptoms presented as thoracic outlet syndrome. […] The tenth case involved the sciatic nerve and presented as sciatica. The patient was misdiagnosed as lumbar disc herniation and took conservative treatment at the clinic in rural areas. […] Epithelioid sarcomas involving the peripheral nerves are extremely rare in both clinic and literature. The symptoms presented were similar to those of the nerve compression diseases, which usually led to misdiagnosis and mistreatment. Early diagnosis and early treatment are the keys to better prognosis.

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

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

  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. […] Local recurrence rates of 40% to 60% have been reported, with a median time to recurrence of 1 to 2 years. However, recurrences have been reported as late as 20 years after the initial operation. As a result, close surveillance with cross-sectional imaging of the primary site and lungs is critical, typically with imaging studies every 6 months to 1 year. […] Prognosis, as with most malignancies, is primarily determined by the clinical stage of the disease. Epithelioid sarcoma prognosis is most closely associated with tumor size, vascular invasion, resectability, and metastases. Large tumor size and early metastases are independently associated with poor outcomes.

• #2 Epithelioid Sarcoma of the Spine: A Review of Literature and Case Report

  https://www.mdpi.com/2077-0383/12/17/5632

  Epithelioid sarcoma is a rare malignant mesenchymal tumor that represents less than 1% of soft-tissue sarcomas. Despite its slow growth, the overall prognosis is poor with a high rate of local recurrence, lymph-node spread, and hematogenous metastasis. A 54 year old male presented with a 1-month history of progressive left upper-limb weakness and numbness. Magnetic resonance imaging (MRI) of the spine showed an enhancing intramedullary mass at the level of T1 also involving the left T1 nerve root. […] The classic type of ES typically presents as a slow-growing painless soft-tissue swelling in the distal extremities of young adults, often associated with ulceration of the overlying skin. […] The proximal subtype also tends to have a have a more aggressive clinical behavior from the outset, with higher rates of local recurrence and earlier metastatic spread.

• #2 Epithelioid Sarcoma—From Genetics to Clinical Practice

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7463637/

  Distant metastases most often develop in the lungs, bones, and brain. […] ES metastatic spread is reported in the course of the disease in 20% to 50% of patients. […] Around 20% of patients present with distant metastases already at the primary diagnosis. […] The survival of patients with ES is unsatisfactory. […] Depending on the study, the 5-year overall survival rates from 25% to 70%. […] Five years on from primary tumor diagnosis, metastatic spread is found in 30% to 75% of patients. […] The presence of lymph nodes metastases at the primary diagnosis and the presence of distant metastases is significantly correlated with the poor prognosis.

• #2 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. In patients with pulmonary metastases, the one-year survival rate is 50% and the five-year survival rate is unfortunately close to zero. […] 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.

• #2 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-9/3914-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. These intervals can be broken into discrete stages to create a standardized framework of comparison, of which several models exist, but collectively will be referred to as the diagnosis journey in this white paper. 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 patient delay describes the period where the patient experiences and responds to symptoms. The nonspecific, nonpainful indolent growths, in the case of classic ES, and the deep, visceral location of growth in the case of proximal ES, adds an additional layer of delay for ES patients. The warning signs of STSs, which include a nontender, soft tissue mass that is bigger than a golf ball, are not widely known by patients and family members.

• #2 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-9/3914-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. Classic ES is more frequent in adolescent and young adult (AYA) males, commonly affects the upper extremity, and is typically present as superficial, nonpainful, indolent growths on the hands and arms followed by chronic nonhealing ulcers with raised margins. Proximal-type ES is less frequently diagnosed than classic ES and can affect young and middle-aged adults and is also slightly more common among males. Proximal-type ES tends to present as deep soft tissue masses found in locations such as the trunk, pelvis, proximal limbs, and limb girdles presenting as deep or visceral tumors. The painless quality of the lump or growth/soft tissue mass can lead patients and clinicians to initially characterize the growth as benign. 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. Unfortunately, ES frequently recurs, with classic ES recurring as multinodular soft tissue masses, whereas proximal-type ES is associated with spread of the disease to distant locations (metastatic disease). Unlike other STSs, ES has a high incidence of lymphatic or regional metastases as it metastasizes via the lymphatic system. Improved ES patient staging and imaging would improve patients initial diagnoses and their subsequent treatment assessment. Wide surgical resection is the most recommended treatment modality for localized disease. Adjuvant radiation therapy has not had a role for assisting with local control in addition to surgical resection. Traditional systemic therapy with cytotoxic agents has demonstrated limited efficacy, with no single chemotherapy regimen identified as the preferred treatment. Currently, there are no consensus guidelines for the systemic treatment, staging, and imaging of ES patients. Current treatment options provide little hope for patients with advanced disease, who face inevitable progression and death.

• #2 Epithelioid sarcoma | Czarnecka | Nowotwory. Journal of Oncology

  https://journals.viamedica.pl/nowotwory_journal_of_oncology/article/view/NJO.a2023.0021

  The 5-year risk of recurrence after radical treatment is high, up to 70%. […] The proximal subtype of ES is more aggressive, has higher rates of recurrence and metastases, and generally worse prognosis and higher mortality compared to classical ES. […] Local recurrences are common in ES and most often develop within six months after radical treatment. Up to 75% of cases with local recurrence also develop distant metastases. […] However, most patients have an advanced stage at first diagnosis with lymph node and / or lung metastases. […] Epithelioid sarcoma metastasizes most frequently to the lungs or pleura. […] High-risk epithelioid sarcomas are patients with large tumors, high tumor grade, inadequate tumor resection, and metastatic disease, predicting a relatively poor clinical outcome.

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

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

  The ninth case involved the lower trunk of brachial plexus and the symptoms presented as thoracic outlet syndrome. […] The tenth case involved the sciatic nerve and presented as sciatica. The patient was misdiagnosed as lumbar disc herniation and took conservative treatment at the clinic in rural areas. […] Epithelioid sarcomas involving the peripheral nerves are extremely rare in both clinic and literature. The symptoms presented were similar to those of the nerve compression diseases, which usually led to misdiagnosis and mistreatment. Early diagnosis and early treatment are the keys to better prognosis.

• #3 Epithelioid sarcoma | Czarnecka | Nowotwory. Journal of Oncology

  https://journals.viamedica.pl/nowotwory_journal_of_oncology/article/view/NJO.a2023.0021

  The 5-year risk of recurrence after radical treatment is high, up to 70%. […] The proximal subtype of ES is more aggressive, has higher rates of recurrence and metastases, and generally worse prognosis and higher mortality compared to classical ES. […] Local recurrences are common in ES and most often develop within six months after radical treatment. Up to 75% of cases with local recurrence also develop distant metastases. […] However, most patients have an advanced stage at first diagnosis with lymph node and / or lung metastases. […] Epithelioid sarcoma metastasizes most frequently to the lungs or pleura. […] High-risk epithelioid sarcomas are patients with large tumors, high tumor grade, inadequate tumor resection, and metastatic disease, predicting a relatively poor clinical outcome.

• #3 :: JKSR :: Journal of the Korean Society of Radiology

  https://jksronline.org/DOIx.php?id=10.3348/jksr.2015.72.6.405

  The two clinicopathological subtypes have different clinical and histological manifestations. The distal-type, which accounts for twice as many cases as the proximal-type, usually shows slow growing painless nodules. […] Even though, the patient in our case was confirmed histopathologically to have the classic-type, the patient had a 3-month history of progressing pain in the lower leg with multiple bone, lymph node and lung metastases. […] Metastases from epithelioid sarcomas develop in approximately 40-50% of cases, with involvement of the lung, lymph nodes and, less frequently, the scalp, adrenal gland and stomach. […] Our patient had a classic-type tumor, showing ipsilateral popliteal, inguinal and external iliac lymph node enlargement with FDG uptake. Additional multiple metastases were noted in the lung and bones.

• #4 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. […] ES is a rare aggressive malignant tumor with dismal prognosis. It 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. […] 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.

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