Chemotherapy Section, IRCCS-Rizzoli Orthopaedic Institute, Bologna, Italy.
Department of Surgical Pathology, IRCCS-Rizzoli Orthopaedic Institute, Bologna, Italy.
Pediatr Blood Cancer. 2019 Jun;66(6):e27653. doi: 10.1002/pbc.27653. Epub 2019 Feb 5.
Ewing sarcoma (ES) is the second most common bone tumor in adolescents and children. Staging workup for ES includes imaging and bone marrow biopsy (BMB). The effective role of BMB is now under discussion.
A monoinstitutional retrospective analysis reviewed clinical charts, imaging, and histology of patients with diagnosis of ES treated at the Rizzoli Institute between 1998 and 2017.
The cohort included 504 cases of ES of bone; 137 (27%) had metastases at diagnosis, while the remaining 367 had localized disease. Twelve patients had a positive BMB (2.4%). Eleven had distant metastases detected at initial workup staging with imaging assessment: six patients presented with bone metastases, five with both bone and lung metastases. Only one patient with ES of the foot (second metatarsus) was found to have bone marrow involvement with negative imaging evaluation (0.3%).
On the basis of our data, we suggest reconsidering the effective role of BMB in initial staging workup for patients with ES with no signs of metastases by modern imaging techniques. In metastatic disease, the assessment of the bone marrow status may remain useful to identify a group of patients at very high risk who could benefit from different treatment strategies.
尤因肉瘤(ES)是青少年和儿童中第二常见的骨肿瘤。ES 的分期检查包括影像学检查和骨髓活检(BMB)。BMB 的有效作用目前仍存在争议。
一项单机构回顾性分析回顾了 1998 年至 2017 年在里佐利研究所接受 ES 诊断和治疗的患者的临床病历、影像学和组织学资料。
该队列包括 504 例骨 ES 患者;137 例(27%)诊断时已有转移,其余 367 例为局限性疾病。12 例患者的 BMB 阳性(2.4%)。11 例患者在初始分期评估的影像学检查中发现远处转移:6 例患者有骨转移,5 例患者既有骨转移又有肺转移。只有 1 例足部(第二跖骨)ES 患者的骨髓受累,而影像学评估为阴性(0.3%)。
根据我们的数据,我们建议重新考虑 BMB 在无现代影像学检查转移迹象的 ES 患者初始分期中的作用。在转移性疾病中,骨髓状态的评估可能仍然有助于确定一组极高风险的患者,这些患者可能受益于不同的治疗策略。