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How to speak ES A guide to 

helpful terms

What are the next steps after diagnosis with ES?

Working with your doctor to determine the next steps

Shaking hands

Receiving a diagnosis like ES can feel daunting, but it’s important to know that you have options.

Working with a multidisciplinary team (MDT), including different types of healthcare professionals and specialists, ensures a collaborative approach to determining the next steps for your care.3,5,9

Learning more about ES and talking with your doctors about your treatment plan can help you manage your condition over time.

Discuss the best treatment pathway for your specific situation with your doctor. Asking for a referral to a sarcoma specialist center can be important because expert care from an MDT may make a difference in making sure your diagnosis is accurate and finding the most effective treatment options.3,5,9,11

Treatment overview

A tick icon

Treatment options will vary depending on whether the disease is localized, advanced, or metastatic, as these factors play a key role in determining the most appropriate approach.7-9

Depending on the stage of your disease, your medical history, and other factors, your doctor may consider localized treatment options, which focus on targeting disease in a specific area of the body, or systemic treatment, which is used to kill cancer cells throughout the body.7-9,21,22

Surgery is usually the primary treatment for localized disease and is the only approach with the potential for a cure.7-9 Radiotherapy combined with surgery is another treatment option, but there is not enough scientific data to confirm how effective this option may be.7-9

You should discuss the suitability of these treatments or other options with your doctor.

 

Please note this does not represent a comprehensive overview of treatment options for ES. Always talk to your doctor about what treatment may be most appropriate for you. Ipsen does not provide medical advice.

Tracking your progress

A bar graph showing an increase

After treatment, you will likely have regular follow-up appointments to monitor your recovery, check for any signs of recurrence, and address any ongoing concerns or side effects.3,9

This may include magnetic resonance imaging (MRI), computed tomography (CT), or positron emission tomography-computed tomography (PET-CT) scans.1,2,5,9

Always talk to your doctors about any new symptoms or health questions you may have, even between regular appointments.

Explore support and some helpful resources on living with ES
References
  1. National Cancer Institute. Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version. Available at https://www.cancer.gov/types/soft-tissue-sarcoma/patient/adult-soft-tissue-treatment-pdq. Accessed February 2025.
  2. Shakya S, Banneyake EL, Cholekho S, et al. Soft tissue sarcoma: clinical recognition and approach to the loneliest cancer. Explor Musculoskeletal Dis. 2024;2:56-68.
  3. von Mehren M, Kane JM, Bui MM, et al. NCCN Guidelines Insights: Soft Tissue Sarcoma, Version 1.2021. J Natl Compr Canc Netw. 2020;18:1604-1612.
  4. Bansal A, Goyal S, Goyal A, et al. WHO classification of soft tissue tumours 2020: an update and simplified approach for radiologists. Eur J Radiol. 2021;143:109937.
  5. National Cancer Institute. Soft Tissue Sarcoma Treatment (PDQ®)–Health Professional Version. Available at https://www.cancer.gov/types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq. Accessed February 2025.
  6. National Cancer Institute. Osteosarcoma Treatment (PDQ®)–Patient Version. Available at https://www.cancer.gov/types/bone/patient/osteosarcoma-treatment-pdq#_1. Accessed February 2025.
  7. Czarnecka AM. Epithelioid sarcoma. NOWOTWORY Journal of Oncology. 2023;73(3):154-161.
  8. Czarnecka AM, Sobczuk P, Kostrzanowski M, et al. Epithelioid sarcoma – from genetics to clinical practice. Cancers. 2020;12:2112.
  9. Needs T, Fillman EP. Epithelioid sarcoma. Updated July 2, 2024. Available at https://www.ncbi.nlm.nih.gov/books/NBK532911/. Accessed February 2025.
  10. Barrett L, Elliott E, Voorhaar M, et al. A mixed-methods study to better measure patient-reported pain and fatigue in soft tissue sarcoma. Oncol Ther. 2023;11(1):129-143.
  11. Recommendations from the Epithelioid Sarcoma Collaborative: a white paper. Available at https://www.jons-online.com/issues/2021/september-2021-vol-12-no-9/3914-recommendations-from-the-epithelioid-sarcoma-collaborative-a-white-paper. Accessed February 2025.
  12. Soomers V, Husson O, Young R, et al. The sarcoma diagnostic interval: a systematic review on length, contributing factors and patient outcomes. ESMO Open. 2020;5(1):e000592.
  13. Alves A, Constantinidou A, Thway K, et al. The evolving management of epithelioid sarcoma. Eur J Cancer Care (Engl). 2021;30(6):e13489.
  14. Martin S, Clark SE, Gerrand C, et al. Patients' experiences of a sarcoma diagnosis: a process mapping exercise of diagnostic pathways. Cancers (Basel). 2023;15(15):3946.
  15. Younger E, Husson O, Bennister L, et al. Age-related sarcoma patient experience: results from a national survey in England. BMC Cancer. 2018;18(1):991.
  16. de Visscher SA, van Ginkel RJ, Wobbes T, et al. Epithelioid sarcoma: still an only surgically curable disease. Cancer. 2006;107(3):606-612.
  17. Halling AC, Wollan PC, Pritchard DJ, et al. Epithelioid sarcoma: a clinicopathologic review of 55 cases. Mayo Clin Proc. 1996;71(7):636-642.
  18. National Cancer Institute. Recurrence. Available at https://www.cancer.gov/search/results?swKeyword=recurrence. Accessed February 2025.
  19. National Cancer Institute. Metastatic cancer: when cancer spreads. Available at https://www.cancer.gov/types/metastatic-cancer. Accessed February 2025.
  20. Null M, Arbor TC, Agarwal M. Anatomy, lymphatic system. Updated March 6, 2023. Available at https://www.ncbi.nlm.nih.gov/books/NBK513247/. Accessed February 2025.
  21. National Cancer Institute. Localized therapy. Available at https://www.cancer.gov/publications/dictionaries/cancer-terms/def/localized-therapy#. Accessed February 2025.
  22. National Cancer Institute. Systemic therapy. Available at https://www.cancer.gov/publications/dictionaries/cancer-terms/def/systemic-therapy. Accessed February 2025.

How to speak ES A guide to 

helpful terms

Cross icon 

How to speak epithelioid sarcoma (ES)

A guide to some helpful terms

Biopsy

A procedure to remove a tiny piece of tissue from the body so it can be examined under a microscope.1

Bone sarcoma

A cancer of the bone that usually affects the large bones of the arm or leg. Also referred to as osteosarcoma or osteogenic sarcoma.2

Cancer

A type of disease that happens when cells in the body divide and grow out of control or do not die when they naturally should.3

Computed tomography (CT)

A scan that uses X-rays and computer technology to take detailed pictures of the inside of the body.4

Distal ES

The most common type of ES that typically affects the hands, arms, feet, legs, and other areas further away from the center of the body.5,6

Epithelioid sarcoma (ES)

A rare type of cancer that forms in the soft tissues of the body. It may start in the soft tissue under the skin of a finger, hand, arm, leg, or foot.5,6

Gene

A piece of the DNA that contains the instructions for making a specific protein in the body.7

Immune system

The body's defense system that protects against infections and diseases by identifying and attacking harmful invaders like bacteria, viruses, and abnormal cells.8

Localized treatment

Treatment that is directed to a specific organ or limited area of the body, such as an abnormal growth on the skin.9

Lymph nodes

Small structures that filter out harmful substances and help the body fight infections.10

Lymph vessels

Thin tubes that transport lymph fluid and white blood cells throughout the body to support immune function.11

Lymphatic system

A network of vessels, tissues, and organs that help remove waste and toxins, as well as fight infections in the body.11

Magnetic resonance imaging (MRI)

A scan that uses powerful magnets and radio waves to take detailed pictures of the inside of the body.12

Malignant

A term used to describe cancer. Malignant cells grow uncontrollably, can invade nearby tissues, and may spread to other parts of the body through the bloodstream or lymphatic system.13

Metastasis

The spread of cancer from where it started to another part of the body.14

Metastatic cancer

Cancer that has spread from where it first started to another part of the body.15

Positron emission tomography-computed tomography (PET-CT)

A procedure that combines the pictures from a positron emission tomography (PET) scan and a computed tomography (CT) scan. The combined scans give more detailed pictures of areas inside the body than either scan gives by itself.16

Proximal ES

A type of ES that forms in areas close to the center of the body, such as the chest, abdomen, pelvis, and back. It is less common than distal ES.5,6

Radiotherapy

High-energy rays used to destroy cancer cells and shrink tumors.17

Recurrence

The return of cancer after successful treatment and a period when no cancer could be detected.18

Sarcoma

A type of cancer that starts in the bones or soft tissues of the body, like muscles, fat, cartilage, blood vessels, or other tissues that support or connect parts of the body.19

SMARCB1

A gene that provides instructions for a protein called INI1, which is involved in many processes, including DNA repair, DNA replication, and cell growth, division, and maturation.20 Most cases of ES involve an error or mutation in this gene.5,6

Soft tissue sarcoma

A group of different cancers that form in the soft tissues of the body, like muscles, fat, blood vessels, tendons, nerves, and lymph vessels.21

Systemic treatment

A treatment that circulates through the bloodstream, reaching cells throughout the body.22

Tumor

An abnormal clump of tissue that forms when cells divide and grow out of control or do not die when they naturally should.23

Ulcer

An open sore on the skin that fails to heal normally.24

References
  1. National Cancer Institute. Biopsy. Available at https://www.cancer.gov/publications/dictionaries/cancer-terms/def/biopsy. Accessed February 2025.
  2. National Cancer Institute. Osteosarcoma. Available at https://www.cancer.gov/publications/dictionaries/cancer-terms/def/osteosarcoma. Accessed February 2025.
  3. National Cancer Institute. What is cancer? Available at https://www.cancer.gov/about-cancer/understanding/what-is-cancer. Accessed February 2025.
  4. National Cancer Institute. Computed tomography (CT) scans and cancer. Available at https://www.cancer.gov/about-cancer/diagnosis-staging/ct-scans-fact-sheet#what-is-computed-tomography. Accessed February 2025.
  5. Needs T, Fillman EP. Epithelioid sarcoma. July 2, 2024. Available at https://www.ncbi.nlm.nih.gov/books/NBK532911/. Accessed February 2025.
  6. Czarnecka AM, Sobczuk P, Kostrzanowski M, et al. Epithelioid sarcoma – from genetics to clinical practice. Cancers. 2020;12:2112.
  7. National Cancer Institute. Gene. Available at https://www.cancer.gov/publications/dictionaries/cancer-terms/def/gene. Accessed February 2025.
  8. National Cancer Institute. Immune system. Available at https://www.cancer.gov/publications/dictionaries/cancer-terms/def/immune-system. Accessed February 2025.
  9. National Cancer Institute. Localized therapy. Available at https://www.cancer.gov/publications/dictionaries/cancer-terms/def/localized-therapy. Accessed February 2025.
  10. National Cancer Institute. Lymph node. Available at https://www.cancer.gov/publications/dictionaries/cancer-terms/def/lymph-node. Accessed February 2025.
  11. National Cancer Institute. Lymphatic system. Available at https://www.cancer.gov/publications/dictionaries/cancer-terms/def/lymphatic-system. Accessed February 2025.
  12. National Cancer Institute. Magnetic resonance imaging (MRI). Available at https://www.cancer.gov/publications/dictionaries/cancer-terms/def/magnetic-resonance-imaging. Accessed February 2025.
  13. National Cancer Institute. Malignant. Available at https://www.cancer.gov/publications/dictionaries/cancer-terms/def/malignant. Accessed February 2025.
  14. National Cancer Institute. Metastasis. Available at https://www.cancer.gov/publications/dictionaries/cancer-terms/def/metastasis. Accessed February 2025.
  15. National Cancer Institute. Metastatic cancer. Available at https://www.cancer.gov/types/metastatic-cancer. Accessed February 2025.
  16. National Cancer Institute. Positron emission tomography-computed tomography. Available at https://www.cancer.gov/publications/dictionaries/cancer-terms/def/positron-emission-tomography-computed-tomography-scan. Accessed February 2025.
  17. National Cancer Institute. Radiotherapy. Available at https://www.cancer.gov/publications/dictionaries/cancer-terms/def/radiotherapy. Accessed February 2025.
  18. National Cancer Institute. Recurrence. Available at https://www.cancer.gov/publications/dictionaries/cancer-terms/def/recurrence. Accessed February 2025.
  19. National Cancer Institute. Sarcoma. Available at https://www.cancer.gov/publications/dictionaries/cancer-terms/def/sarcoma. Accessed February 2025.
  20. MedlinePlus. SMARCB1 gene. Available at https://medlineplus.gov/genetics/gene/smarcb1/. Accessed February 2025.
  21. National Cancer Institute. Soft tissue sarcoma. Available at https://www.cancer.gov/publications/dictionaries/cancer-terms/def/sarcoma. Accessed February 2025.
  22. National Cancer Institute. Systemic therapy Available https://www.cancer.gov/publications/dictionaries/cancer-terms/def/systemic-therapy. Accessed February 2025.
  23. National Cancer Institute. Tumor. Available at https://www.cancer.gov/publications/dictionaries/cancer-terms/def/tumor. Accessed February 2025.
  24. National Cancer Institute. Ulcer. Available at https://www.cancer.gov/publications/dictionaries/cancer-terms/def/ulcer. Accessed February 2025.