Epithelioid sarcoma in children, adolescents, and young adults: Localized, primary metastatic and relapsed disease. Treatment results of five Cooperative Weichteilsarkom Studiengruppe (CWS) trials and one registry

Abstract

Background Epithelioid sarcoma (ES) is a rare malignant soft‐tissue tumor. Little is known about the optimal treatment of primary localized (LD), metastatic (MD), and relapsed disease (RD). Methods Characteristics, treatment, and outcome of 67 patients registered within the Cooperative Weichteilsarkom Studiengruppe CWS‐81, ‐86, ‐91, ‐96, ‐2002P trials and the registry SoTiSaR were analyzed (1981–2016). Results The median age was 14 years (range, 0.7–26.9); 53 patients had localized disease (LD) and 14 metastatic disease (MD). A total of 58 of 67 patients were treated with primary resection. Resection was microscopically complete (R0) in 35, microscopically incomplete (R1) in 12, macroscopically incomplete (R2) in 20 patients. Radiotherapy (RT) was administered to 33 of 67 patients and 49 of 67 patients received chemotherapy (CHT). Complete remission (CR) was achieved in 45 of 53 (85%) patients with LD. Twenty‐seven of 53 patients relapsed after a median time of 0.9 years (range, 0.1–2.3). Relapse therapy consisted of resection (n = 19/27), RT (n = 10/27), CHT (n = 12/27), and limb perfusion (n = 3/27). The five‐year event‐free survival and overall survival of patients with LD, MD, and RD was 35% (± 12, CI 95%) and 58% (± 14, CI 95%), 7% (± 14, CI 95%), and 9% (± 16, CI 95%), 24% (± 17, CI 95%), and 40% (± 20, CI 95%), respectively. Tumor size, IRS group, tumor invasiveness, nodal status, and best resection correlated with a favorable prognosis in patients with LD while best resection was the only significant factor in patients with RD. Conclusions Complete tumor resection correlates with long‐term survival in patients with ES.

Epithelioid Sarcoma—From Genetics to Clinical Practice

“…Only a few data on clinical features and optimal treatment of pediatric patients are described in the literature. ES is most common in older children (7–14 years) with a slight male predominance, but newborn cases have also been reported [ 9 , 10 , 109 , 110 , 111 ]. A typical location for pediatric ES are extremities, especially arms and hands.…”

“…A typical location for pediatric ES are extremities, especially arms and hands. There are some characteristics, such as location in superficial distal sites, indolent growth rate, and the tendency to locoregional relapses [ 9 , 10 , 110 , 111 ].…”

“…However, multimodal therapy, including chemotherapy and irradiation, is recommended for most children. Only adequately excised small tumors can be safely treated with surgery alone [ 9 , 109 , 110 ].…”

“…Chemotherapy includes vincristine and dactinomycin in combination with an alkylating agent and anthracycline, etoposide, or carboplatin depending on the patient’s risk group. Infants <12 months should receive reduced doses of chemotherapy [ 110 ].…”

“…In the whole evaluated group of 67 patients, they reported EFS and OS of only 29% and 48%, respectively (35% and 58% for patients with localized disease). Patients with metastatic disease had a 5-year EFS rate of 7% and an OS rate of 9% [ 110 ]. There is very little data on relapses in younger patients, and optimal therapy is still undefined.…”