Comprehensive treatment approaches
Early, comprehensive treatment may be necessary when treating ES
ES presents unique challenges in diagnosis and treatment1-3 which often require early, aggressive intervention to potentially improve outcomes and survival.4-8
As with other soft tissue sarcomas, treatments that may be used for ES are classified into two main categories: localized therapies – such as surgical resection or radiotherapy, which targets the tumor at its site of origin – and systemic therapies, which target ES through systemic mechanisms of action (MOAs), addressing disease that may have spread beyond the primary site.9-14
Multiple treatment modalities and a multidisciplinary team (MDT) approach are recommended for optimal treatment of soft tissue sarcomas such as ES.1,2,10-12 A clear understanding of these treatment modalities is crucial for advancing care and improving outcomes in this rare malignancy.
The aim of localized therapies used in ES, such as surgery or radiotherapy, is to directly target the tumor at its primary location.1–3,11,13
Surgery in the multimodal management of ES
Surgery is considered the only potentially curative option2 and surgical resection, with or without radiotherapy, remains a cornerstone in the multimodal management of nonmetastatic ES.1,2,4,5,10-11
- Surgical treatment can be complex due to the tendency of ES to invade multiple tissue planes and involve critical neurovascular structures.5
- While margins of 2 to 5 cm are commonly reported, the extent of resection should be tailored to each patient, prioritizing limb function preservation whenever possible.1,11
- Affected lymph nodes are removed when involved, with amputation considered only in cases of extensive disease spread.1-3
- Given the aggressive nature of ES and its tendency to recur,1-3 surgical intervention may require a multidisciplinary approach involving oncologists, surgeons, and radiologists to ensure optimal outcomes.1,10
Radiotherapy in the multimodal management of ES
Radiotherapy plays a key role in the treatment of ES, particularly as an adjunct to surgery or in cases where surgery alone may not achieve optimal control.1-3
- Radiotherapy is commonly employed to reduce local recurrence, though its precise indications are not well established.1-3
- Radiotherapy is typically recommended for patients with narrow or microscopically positive margins or recurrent disease, or as a palliative treatment for large tumors.1
- Radiotherapy is most effective when combined with surgery in a comprehensive, multidisciplinary treatment plan aimed at improving local control and overall patient outcomes.3,9,11,12
Systemic therapies work throughout the body to target both primary and distant disease.14
- Systemic therapy may be used in the management of ES in a neoadjuvant and adjuvant setting.2,3,11,12
- Neoadjuvant therapy is used before surgery to reduce the tumor size.15
- Adjuvant therapy is used after surgery to reduce recurrence risk and may be considered in advanced or metastatic disease.16
- If a tumor is unresectable, systemic therapy may be used on its own without surgery.3,9,11,12
Multidisciplinary management
Managing ES as an MDT
Managing soft tissue sarcomas such as ES often involves complex decision-making that benefits from the input of various specialists.1,8 As such, current guidelines strongly advise a coordinated, multidisciplinary approach.9-12
- The MDT typically involves specialists such as surgeons, oncologists, radiologists, and pathologists.1
The importance of follow-up in ES
Long-term monitoring
Patients with ES may require continuous follow-up due to the tumor’s aggressive nature, high recurrence rates, metastatic potential, possible treatment-related complications, and the possible need for ongoing assessment of therapy response.1,8,10,11
Talking to your patients with ES about monitoring:
- It’s important to be open with patients about long-term expectations and the benefits of ongoing monitoring.17
- It can be critical that patients are educated on possible post-treatment complications and encouraged to report any new or recurrent symptoms promptly.1
- It is helpful to explain the critical importance of attending all scheduled follow-up appointments to ensure the best possible outcome.1
Follow-up appointments can be an opportunity to:10,11
- Assess treatment efficacy.
- Explore symptoms or treatment side effects.
- Assess patients for recurrence or metastasis via imaging studies (may include MRI, CT, PET-CT, or X-ray).
After the start of treatment, consensus guidelines recommend follow-up appointments every 3 to 6 months for 2 to 3 years and then every 6 to 12 months from then on.10,11
After treatment is initiated, current guidelines recommend follow-up every 3 to 6 months for 2 to 3 years and then every 6 to 12 months from then on.10,11
- Needs T, Fillman EP. Epithelioid sarcoma. Updated July 2, 2024. Available at https://www.ncbi.nlm.nih.gov/books/NBK532911/. Accessed February 2025.
- Czarnecka AM. Epithelioid sarcoma. NOWOTWORY J Oncol. 2023;73:154-161.
- Czarnecka AM, Sobczuk P, Kostrzanowski M, et al. Epithelioid sarcoma – from genetics to clinical practice. Cancers. 2020;12:2112.
- Zegarra Buitron E, Vidal Panduro DA, Morales Luna D. Clinicopathological characteristics, treatment, and survival in patients diagnosed with proximal-type epithelioid sarcoma: a case report and systematic review. Cureus. 2022;14(12):e32962.
- Alexander L. Epithelioid sarcoma of upper extremity: diagnostic dilemma with therapeutic challenges. Cureus. 2021;13(3):e14156.
- 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.
- 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.
- 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.
- Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Soft Tissue Sarcoma V.4.2024. © National Comprehensive Cancer Network, Inc. 2024. All rights reserved. Accessed February 17, 2025. To view the most recent and complete version of the guideline, go to NCCN.org.
- von Mehren M, Kane JM III, Bui MM, et al. NCCN Guidelines insights: soft tissue sarcoma, version 1.2021. J Natl Compr Canc Netw. 2020;18(12):1604-1612.
- Gronchi A, Miah AB, Dei Tos AP, et al. Soft tissue and visceral sarcomas: ESMO-EURACAN-GENTURIS Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2021;32(11):1348-1365.
- Hayes AJ, Nixon IF, Strauss DC, et al. UK guidelines for the management of soft tissue sarcomas. Br J Cancer. 2025;132(1):11-31.
- National Cancer Institute. Localized therapy. Available at https://www.cancer.gov/publications/dictionaries/cancer-terms/def/localized-therapy#. Accessed February 2025.
- National Cancer Institute. Systemic therapy. Available at https://www.cancer.gov/publications/dictionaries/cancer-terms/def/systemic-therapy. Accessed February 2025.
- National Cancer Institute. Neoadjuvant therapy. Available at https://www.cancer.gov/publications/dictionaries/cancer-terms/def/neoadjuvant-therapy#. Accessed February 2025.
- National Cancer Institute. Adjuvant therapy. Available at https://www.cancer.gov/publications/dictionaries/cancer-terms/def/adjuvant-therapy. Accessed February 2025.
- Weaver R, O'Connor M, Carey Smith R, et al. "We're on a merry-go-round": reflections of patients and carers after completing treatment for sarcoma. Curr Oncol. 2021;28(4):3003-3014.
- Gerrand CH, Billingham LJ, Woll PJ, Grimer RJ. Follow up after primary treatment of soft tissue sarcoma: a survey of current practice in the United Kingdom. Sarcoma. 2007;2007:34128.