Skip metastases in appendicular Ewing sarcoma: relationship to distant metastases at diagnosis, chemotherapy response and overall survival.

Abstract

To identify in appendicular Ewing sarcoma (ES), if skip metastases (SM) are associated with distant metastases at presentation, response to neoadjuvant chemotherapy and overall outcome. Patients with appendicular ES from 2007 to 2021 who had whole-bone MRI to identify SM were included in the study. Patient demographics included age/gender, bone involved, the presence of SM, longitudinal tumour length, presence of extra-osseous disease and its axial depth if present from whole-bone MRI and lung metastases and distant bone metastases from staging studies. Response to neoadjuvant chemotherapy from resection specimens and overall survival were noted. Comparison of these factors between patients with and without SM was undertaken. Ninety-five patients (66 males; 29 females: mean age 15.8years; range 5-48years) were included. On whole-bone MRI, 80 (84.2%) patients had no SM and 15 (15.8%) patients had one or more SM. Of patients without a SM, lung metastases were present in 16 (21%), distant bone metastases in 7 (11%), while 51 (75%) had a good response to chemotherapy compared with 7 (50%), 3 (27%), and 10 (77%), respectively, in patients with a SM. SM were significantly associated with lung metastases (p = 0.02), but not with distant skeletal metastases (p = 0.13), chemotherapy response (p = 0.88), tumour length (p = 0.47), presence of (p = 0.15) or axial depth of extra-osseous disease (p = 0.4). SM were associated with a significantly poorer survival (p = 0.007) and three times greater risk of death during follow-up. In appendicular ES, the identification of a SM is associated with the presence of lung metastases at presentation and poorer survival.