Surgery with or without chemotherapy and/or radiation therapy

In a German CWS retrospective analysis of 67 children, adolescents, and young adults (median age, 14 years) with epithelioid sarcoma, 53 patients presented with localized disease and 14 patients presented with metastatic disease.[59][Level of evidence C1] Fifty-eight of 67 patients were treated with primary resections. Resections were microscopically complete in 35 patients, microscopically incomplete in 12 patients, and macroscopically incomplete in 20 patients. Forty-nine patients received chemotherapy, and 33 patients received radiation therapy.

In a German CWS retrospective analysis of 67 children, adolescents, and young adults (median age, 14 years) with epithelioid sarcoma, 53 patients presented with localized disease and 14 patients presented with metastatic disease.[59][Level of evidence C1] Fifty-eight of 67 patients were treated with primary resections. Resections were microscopically complete in 35 patients, microscopically incomplete in 12 patients, and macroscopically incomplete in 20 patients. Forty-nine patients received chemotherapy, and 33 patients received radiation therapy.

Complete remission was achieved in 45 of 53 patients (85%) with localized disease.

In a German CWS retrospective analysis of 67 children, adolescents, and young adults (median age, 14 years) with epithelioid sarcoma, 53 patients presented with localized disease and 14 patients presented with metastatic disease.[59][Level of evidence C1] Fifty-eight of 67 patients were treated with primary resections. Resections were microscopically complete in 35 patients, microscopically incomplete in 12 patients, and macroscopically incomplete in 20 patients. Forty-nine patients received chemotherapy, and 33 patients received radiation therapy.

Twenty-seven patients with localized disease had local (n = 16), metastatic (n = 6), or combined (n = 4) relapses after a median time of 0.9 years (range, 0.1–2.3 years) after complete response of disease (45 of 63).

In a German CWS retrospective analysis of 67 children, adolescents, and young adults (median age, 14 years) with epithelioid sarcoma, 53 patients presented with localized disease and 14 patients presented with metastatic disease.[59][Level of evidence C1] Fifty-eight of 67 patients were treated with primary resections. Resections were microscopically complete in 35 patients, microscopically incomplete in 12 patients, and macroscopically incomplete in 20 patients. Forty-nine patients received chemotherapy, and 33 patients received radiation therapy.

Patients with localized disease had a 5-year EFS rate of 35% (95% CI, ±12%) and an OS rate of 48% (95% CI, ±14%).

In a German CWS retrospective analysis of 67 children, adolescents, and young adults (median age, 14 years) with epithelioid sarcoma, 53 patients presented with localized disease and 14 patients presented with metastatic disease.[59][Level of evidence C1] Fifty-eight of 67 patients were treated with primary resections. Resections were microscopically complete in 35 patients, microscopically incomplete in 12 patients, and macroscopically incomplete in 20 patients. Forty-nine patients received chemotherapy, and 33 patients received radiation therapy.

Patients with metastatic disease had a 5-year EFS rate of 7% (95% CI, ±14%) and an OS rate of 9% (95% CI, ±16%).

In a German CWS retrospective analysis of 67 children, adolescents, and young adults (median age, 14 years) with epithelioid sarcoma, 53 patients presented with localized disease and 14 patients presented with metastatic disease.[59][Level of evidence C1] Fifty-eight of 67 patients were treated with primary resections. Resections were microscopically complete in 35 patients, microscopically incomplete in 12 patients, and macroscopically incomplete in 20 patients. Forty-nine patients received chemotherapy, and 33 patients received radiation therapy.

Smaller tumor size, lower IRS group, less tumor invasiveness, negative nodal status, and microscopically complete resection correlated with a favorable prognosis in patients with localized disease.

A retrospective analysis reviewed COG and EpSSG prospective clinical trials that enrolled patients younger than 30 years with epithelioid sarcoma.[60][Level of evidence B4] The analysis identified 63 patients who were treated between July 2005 and November 2015. Patients were stratified into three risk groups using a combination of clinical features and treatment received. Low-risk patients (n = 34) underwent surgery with or without radiation therapy and included predominantly patients with nonmetastatic widely or marginally resected tumors 5 cm or smaller. The intermediate-risk group included patients (n = 16) with nonmetastatic, high-grade, and larger than 5 cm tumors or unresectable tumors. Patients with nodal or distant metastatic disease were at high risk (n = 13) , regardless of tumor grade or size.

A retrospective analysis reviewed COG and EpSSG prospective clinical trials that enrolled patients younger than 30 years with epithelioid sarcoma.[60][Level of evidence B4] The analysis identified 63 patients who were treated between July 2005 and November 2015. Patients were stratified into three risk groups using a combination of clinical features and treatment received. Low-risk patients (n = 34) underwent surgery with or without radiation therapy and included predominantly patients with nonmetastatic widely or marginally resected tumors 5 cm or smaller. The intermediate-risk group included patients (n = 16) with nonmetastatic, high-grade, and larger than 5 cm tumors or unresectable tumors. Patients with nodal or distant metastatic disease were at high risk (n = 13) , regardless of tumor grade or size.

Partial responses were observed in 11 of 22 patients (50%) who received neoadjuvant therapy.

A retrospective analysis reviewed COG and EpSSG prospective clinical trials that enrolled patients younger than 30 years with epithelioid sarcoma.[60][Level of evidence B4] The analysis identified 63 patients who were treated between July 2005 and November 2015. Patients were stratified into three risk groups using a combination of clinical features and treatment received. Low-risk patients (n = 34) underwent surgery with or without radiation therapy and included predominantly patients with nonmetastatic widely or marginally resected tumors 5 cm or smaller. The intermediate-risk group included patients (n = 16) with nonmetastatic, high-grade, and larger than 5 cm tumors or unresectable tumors. Patients with nodal or distant metastatic disease were at high risk (n = 13) , regardless of tumor grade or size.

Events were local recurrence (n = 10) and distant recurrence (n = 15).

A retrospective analysis reviewed COG and EpSSG prospective clinical trials that enrolled patients younger than 30 years with epithelioid sarcoma.[60][Level of evidence B4] The analysis identified 63 patients who were treated between July 2005 and November 2015. Patients were stratified into three risk groups using a combination of clinical features and treatment received. Low-risk patients (n = 34) underwent surgery with or without radiation therapy and included predominantly patients with nonmetastatic widely or marginally resected tumors 5 cm or smaller. The intermediate-risk group included patients (n = 16) with nonmetastatic, high-grade, and larger than 5 cm tumors or unresectable tumors. Patients with nodal or distant metastatic disease were at high risk (n = 13) , regardless of tumor grade or size.

The estimated 5-year OS rates were 86.4% for low-risk patients, 63.5% for intermediate-risk patients, and 0% for high-risk patients.

A retrospective analysis reviewed COG and EpSSG prospective clinical trials that enrolled patients younger than 30 years with epithelioid sarcoma.[60][Level of evidence B4] The analysis identified 63 patients who were treated between July 2005 and November 2015. Patients were stratified into three risk groups using a combination of clinical features and treatment received. Low-risk patients (n = 34) underwent surgery with or without radiation therapy and included predominantly patients with nonmetastatic widely or marginally resected tumors 5 cm or smaller. The intermediate-risk group included patients (n = 16) with nonmetastatic, high-grade, and larger than 5 cm tumors or unresectable tumors. Patients with nodal or distant metastatic disease were at high risk (n = 13) , regardless of tumor grade or size.

Locoregional nodal involvement, invasive tumor, high grade, and lesser extent of resection predicted poorer EFS in patients without metastases.

A review of 30 pediatric patients with epithelioid sarcoma (median age at presentation, 12 years) reported the following results:[61]

A review of 30 pediatric patients with epithelioid sarcoma (median age at presentation, 12 years) reported the following results:[61]

Responses to chemotherapy were reported in 40% of patients using sarcoma-based treatment regimens.

A review of 30 pediatric patients with epithelioid sarcoma (median age at presentation, 12 years) reported the following results:[61]

Sixty percent of patients were alive at 5 years after initial diagnosis.

A single-institution retrospective review of 20 patients, which included children and adults (median age, 27.3 years), reported the following:[57]

A single-institution retrospective review of 20 patients, which included children and adults (median age, 27.3 years), reported the following:[57]

There was no difference in the probability of recurrence between patients who received chemotherapy and those who did not receive chemotherapy.

A single-institution retrospective review of 20 patients, which included children and adults (median age, 27.3 years), reported the following:[57]

The authors suggested that radiation therapy may be useful.