Soft-tissue Ewing sarcoma in a low-incidence population: comparison to skeletal Ewing sarcoma for clinical characteristics and treatment outcome.
OBJECTIVE
Due to the low incidence, treatments for Korean soft tissue Ewing sarcoma patients have been heterogeneous, and reported data are limited. In this study, we retrospectively analyzed soft tissue Ewing cases treated at our institution.


METHODS
We analyzed the clinicopathologic characteristics and treatment outcome of soft tissue Ewing sarcoma patients and compared with those of skeletal cases.


RESULTS
Twenty-seven soft tissue Ewing sarcoma cases were evaluated. Patients with soft tissue Ewing sarcoma were older than patients with skeletal tumors (P = 0.03), and tended to have metastasis at diagnosis (P = 0.12). However, sex ratios, pathologies, tumor volumes, and histologic response to preoperative chemotherapy were not different in the two groups. The 5-year overall survival (49.0%) and event-free survival (45.6%) of soft tissue Ewing sarcoma patients were similar to those of skeletal tumor patients (51.8% and 46.0%, respectively). Presence of metastasis at diagnosis and poor histologic response to preoperative chemotherapy were associated with an adverse outcome for both groups. Similar to skeletal tumors, central tumor location, pathology and tumor volume tended to be related to the survival of soft tissue Ewing sarcoma. However, age and the use of a modality other than surgery to achieve local control did not influence the survival of soft tissue Ewing sarcoma patients.


CONCLUSIONS
Our data could provide a basis to design a collaborative or multinational study targeting Ewing sarcoma family tumors.
Department of Pediatrics, Korea Cancer Center Hospital, Seoul, Republic of Korea.
Jun Ah
JA
Lee
Dong Ho
DH
Kim
Jung Sub
JS
Lim
Jae-Soo
JS
Koh
Mi Sook
MS
Kim
Chang-Bae
CB
Kong
Won Seok
WS
Song
Wan Hyeong
WH
Cho
Soo-Yong
SY
Lee
Dae-Geun
DG
Jeon
D000293:Adolescent; D000328:Adult; D001859:Bone Neoplasms / Q000453:epidemiology / Q000473:pathology* / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D009141:Musculoskeletal System / Q000473:pathology; D009367:Neoplasm Staging; D012189:Retrospective Studies; D012509:Sarcoma / Q000453:epidemiology / Q000473:pathology* / Q000628:therapy*; D012512:Sarcoma, Ewing / Q000453:epidemiology / Q000473:pathology* / Q000628:therapy*; D015996:Survival Rate; D016896:Treatment Outcome; D055815:Young Adult