TriangleHEALTH PROFESSIONAL · SOURCE READING
Surgery and chemotherapy
Source: Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 21, 2025 · Captured 2026-09-09.
Selected source text with whitespace normalised. This Triangle page is not an NCI PDQ summary.
Complete surgical removal, when feasible, is the mainstay of therapy.[64]
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Evidence (surgery with or without chemotherapy):
In a series of nine patients, the following was observed:[65][Level of evidence C1]
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In a series of nine patients, the following was observed:[65][Level of evidence C1]
Four patients achieved continuous remission after complete resection.
In a series of nine patients, the following was observed:[65][Level of evidence C1]
Three patients with residual disease experienced disease recurrence but later achieved continuous remission.
In a series of nine patients, the following was observed:[65][Level of evidence C1]
One patient with metastatic disease responded to multiagent chemotherapy.
In another study of 31 patients who underwent complete surgical resection, 4 patients had local disease recurrences.[62]
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In another study of 31 patients who underwent complete surgical resection, 4 patients had local disease recurrences.[62]
Of the 4 patients with local recurrences, all patients were alive after surgical re-resection (3 patients) or adjuvant chemotherapy and resection (1 patient).
A review of German studies identified 37 patients younger than 21 years with inflammatory myofibroblastic tumors.[66][Level of evidence C1]
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A review of German studies identified 37 patients younger than 21 years with inflammatory myofibroblastic tumors.[66][Level of evidence C1]
Of 20 patients, 17 had complete resections with no recurrences. Surgical resections can be limited to those procedures that preserve form and function.
A review of German studies identified 37 patients younger than 21 years with inflammatory myofibroblastic tumors.[66][Level of evidence C1]
All other patients were treated with a combination of surgery and various chemotherapy regimens.
A review of German studies identified 37 patients younger than 21 years with inflammatory myofibroblastic tumors.[66][Level of evidence C1]
The overall 5-year EFS rate was 75%, and the OS rate was 91%.
A series of 32 patients aged 18 years and younger found that complete excision was the mainstay of therapy, although some patients were treated with steroids or cytotoxic chemotherapy.[67][Level of evidence C1]
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A series of 32 patients aged 18 years and younger found that complete excision was the mainstay of therapy, although some patients were treated with steroids or cytotoxic chemotherapy.[67][Level of evidence C1]
The OS rate was 94%.
A series of 32 patients aged 18 years and younger found that complete excision was the mainstay of therapy, although some patients were treated with steroids or cytotoxic chemotherapy.[67][Level of evidence C1]
Three patients had relapsed disease, two of whom died of the disease.
A series of 32 patients aged 18 years and younger found that complete excision was the mainstay of therapy, although some patients were treated with steroids or cytotoxic chemotherapy.[67][Level of evidence C1]
When complete excision was performed, with or without other treatments such as steroids, there was a high survival rate for these patients.
The benefit of chemotherapy has been noted in case reports.[68] A prospective registry of children with inflammatory myofibroblastic tumor from the EpSSG (2005–2016) found an EFS rate of 82.9% and an OS rate of 98.1% at 5 years in all patients. The response rate for patients who received systemic therapy (chemotherapy or ALK inhibitor therapy) was 63% when used as front-line therapy and 66% when used as second-line therapy. Eight of ten patients who received vinblastine and low-dose methotrexate and all five patients who received ALK inhibitors (all of whom had ALK-positive tumors) responded to treatment.[62]
A retrospective, international, multicenter study analyzed patients younger than 21 years with ROS1-altered inflammatory myofibroblastic tumors who were enrolled in either the EpSSG NRSTS-2005 study or the Soft Tissue Sarcoma Registry. Primary surgery was recommended if a microscopic radical resection without disfigurement was feasible. Of the 19 patients, 12 received neoadjuvant systemic therapy as first-line treatment (high-dose steroids, n = 2; vinorelbine/vinblastine with methotrexate, n = 6; ROS1 inhibitors, n = 8). With a median follow-up of 2.8 years, seven patients had an event. The 3-year EFS rate was 41% (95% CI, 11%–71%), and the OS rate was 100%. While many patients in this series received crizotinib, the specific ROS1 inhibitor used for each patient was not specified.[69]
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Preserved source evidence · Not medical advice