How ES mimics other conditions
ES mimics other conditions
As a slow-growing, indolent tumor,
ES may be overlooked due to its initially
benign appearance.6-8
ES tumors can present with a wide range of signs and symptoms, often leading clinicians to consider various benign or malignant conditions.6-8
Misdiagnosis or delays in diagnosis may lead to delayed or inappropriate treatment and negatively impact patient survival.1-4
Recognizing the signs and symptoms early and making an accurate diagnosis is essential for reducing recurrence, lowering mortality, and improving survival rates.1
The variable presentation and aggressive nature of ES have led some to call it a "great masquerader" and "a wolf in sheep's clothing".2
Patients with undiagnosed ES often delay seeking medical attention4 likely due to its slow progression and mild symptoms.6-9
In cases of clinical suspicion, encourage these patients to visit our companion patient website for practical, informative resources created with their needs in mind.
How the signs and symptoms of ES can vary
ES signs and symptoms depend on tumor location and can vary8
ES is a rare and aggressive soft tissue sarcoma with two subtypes: the distal (classical) type, which typically presents as a slow-growing mass in distal extremities (hands, forearms, or feet), and the proximal type, which is more aggressive and occurs in proximal limbs (pelvis, thighs, upper arms, or shoulders).1,6–8,10,11
Physical signs
Distal-type ES:
- Commonly occurs in distal extremities (hands, forearms, or feet).6-8,10,11
- Superficial, slow-growing nodules, typically painless;6,7,10 can also arise as deep-seated slow-growing tumors.2,6
- Typically <5 cm but can be larger.6,7
Proximal-type ES:
- Commonly occurs in proximal limbs (pelvis, thighs, upper arms, or shoulders).6-8,10,11
- Deep, infiltrating soft-tissue masses that commonly present with deeper tissue invasion compared to distal type.10,11
- Can grow up to 20 cm.6,7
Skin changes
- The overlying skin may have a shiny, gray-tan appearance.7
- Superficial bleeding, necrosis, swelling, and ulceration may occur.6-8
Symptoms
Pain or discomfort
May be painless initially but may cause pain and discomfort as it grows, depending on location (for example, near joints)1,2,6-8,11
Neurological symptoms
Localized growth can damage surrounding tissues, nerves, and blood vessels, and compression of nearby nerves or vessels can cause numbness, tingling, or weakness2,6,10,11
Tumor site-specific symptoms
Depending on the location, patients may experience bleeding, pain, or organ dysfunction1,8,12
When evaluating a suspicious lesion, remember to ask about:
Onset (sudden appearance or gradual emergence?)6-8
History of trauma to the area?6,7
Growth rate (slow or rapidly progressive?)6-8
Presence of neurovascular symptoms (paresthesia or motor deficit?)2,10
Signs of pulmonary metastasis (e.g., new-onset cough, hemoptysis)6-8
Onset (sudden appearance or gradual emergence?)6-8
Growth rate (slow or rapidly progressive?)6-8
History of trauma to the area?6,7
Presence of neurovascular symptoms (paresthesia or motor deficit?)2,10
Signs of pulmonary metastasis (e.g., new-onset cough, hemoptysis)6-8
Seeking support based on signs and symptoms
If signs and symptoms indicate ES, consider seeking expert support from a sarcoma specialist to ensure accurate assessment and management4,5,9,13-17
The evaluating physician should maintain a high level of vigilance to ensure a timely and accurate diagnosis.2,6
Why seek expert support from a sarcoma specialist
Seeking expert support and timely referral to specialist sarcoma centers can be critical for optimal diagnosis, treatment, and outcomes, but delays are common:4,9
The pathway from symptom onset to diagnosis is complex.9
Patients may delay seeking medical advice after first noticing symptoms: almost 30% may wait over 3 months, and 10% may wait over a year.4
The heterogeneity and ambiguity of symptoms may lead to longer referral times compared with more common cancers.9
Delays in referral may occur in almost 30% of cases due to misinterpretation of symptoms.4
Consider consulting a sarcoma specialist if you suspect ES.
Your decision may help minimize delays in diagnosis.
Early diagnosis through specialist referral may improve outcomes1,2,4,5,9,15
Detecting ES early is crucial as prompt, aggressive therapy may reduce the poor outcomes associated with ES,1,2,4,15 potentially leading to:
Lower recurrence rates1
Lower mortality1
Improved survival1
Consider referring patients to a specialist sarcoma center if ES is suspected based on the signs and symptoms described above14,16
Seek support from a specialist sarcoma center if you observe:
Tumor size
>5 cm16,17
Recent growth16
Deep location16,17
Pain16
Due to the rarity and heterogeneity of ES, it is important to involve sarcoma specialists working as part of a multidisciplinary team, including radiologists, oncologists, pathologists, and surgeons.6,15-17
Treatment by a soft tissue specialist may improve local control and may enhance survival rates.18-20
- 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.
- 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, Sobczuk P, Kostrzanowski M, et al. Epithelioid sarcoma – from genetics to clinical practice. Cancers. 2020;12:2112.
- Czarnecka AM. Epithelioid sarcoma. NOWOTWORY J Oncol. 2023;73:154-161.
- 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.
- Krotewicz M, Czarnecka AM, Błoński P, et al. Distal and proximal epithelioid sarcoma - differences in diagnosis and similarities in treatment. Oncol Clin Pract. 2024;99119.
- Li Y, Cao G, Tao X, et al. Clinicopathologic features of epithelioid sarcoma: report of seventeen cases and review of literature. Int J Clin Exp Pathol. 2019;12(8):3042-3048.
- Ahmad Z, Stanazai Q, Wright S, et al. Primary pleural epithelioid sarcoma of the proximal type: a diagnostic and therapeutic challenge. Transl Lung Cancer Res. 2019;8(5):700-705.
- Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Soft Tissue Sarcoma V.1.2021. © 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. Accessed February 2025.
- 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.
- 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.
- 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.
- Bhangu AA, Beard JA, Grimer RJ. Should soft tissue sarcomas be treated at a specialist centre? Sarcoma. 2004;8(1):1-6
- Blay JY, Soibinet P, Penel N, et al. Improved survival using specialized multidisciplinary board in sarcoma patients. Ann Oncol. 2017;28(11):2852-2859.
- Tirotta A , Bacon A, Collins S, et al. Primary retroperitoneal sarcoma: A comparison of survival outcomes in specialist and non-specialist sarcoma centres. Eur J Cancer. 2023;188:20-28.