Bone marrow examination in patients with Ewing sarcoma/peripheral primitive neuroectodermal tumor without metastasis based on 18F-fluorodeoxyglucose positron emission tomography/computed tomography

Chiaki Inagaki,Kenji Tamura,Akihiko Shimomura,Chikako Shimizu,Hitomi Sumiyoshi Okuma,Akihiko Yoshida,Ayumu Arakawa,Makoto Kodaira,Kan Yonemori,Yasuhiro Fujiwara,Emi Noguchi,Chitose Ogawa,Mayu Yunokawa,Tatsunori Shimoi,Kazuki Sudo,Asuka Kawachi

doi:10.1007/s12032-019-1279-8

Abstract

Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/PNET) is an aggressive bone tumor. Bone marrow aspiration and biopsy (BMAB) has been recognized as the gold standard for assessing bone marrow status. While the latest guideline suggests the need to omit bone marrow aspiration in patients with no findings on 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET) based on one retrospective report, there is no study using 18F-FDG PET/computed tomography (CT). We retrospectively reviewed 26 consecutive, previously untreated, ES/PNET patients. We compare the results of bone marrow aspiration and biopsy (BMAB) and those of 18F-FDG PET/CT in ES/PNET patients. All of the 21 patients without metastases on 18F-FDG PET/CT had negative BMAB. The sensitivity of bone marrow involvement in bone metastases positive patients on 18F-FDG PET/CT was 75% (3/4), and the specificity was 100% (22/22). In addition to the metastatic findings on 18F-FDG PET/CT, tumor diameter, lactate dehydrogenase level at diagnosis, and the presence or absence of bone metastasis were factors related to bone marrow involvement. It may be a reasonable option to omit BMAB in ES/PNET patients with no distant metastasis based on 18F-FDG PET/CT findings.

Highlights

Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/PNET) is a member of the Ewing sarcoma family of tumors and originated from small undifferentiated neuroectodermal cells
All except one patient underwent combination imaging, which consisted of 18F-FDG PET/computed tomography (CT) (n = 26), contrasted CT (n = 25), and magnetic resonance imaging (MRI) of the primary site (n = 11), at the initial assessment
The result of this study indicated that having no metastatic site following 18F-FDG PET/CT is an accurate negative indicator of bone marrow involvement

Summary

Introduction

Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/PNET) is a member of the Ewing sarcoma family of tumors and originated from small undifferentiated neuroectodermal cells. ES/PNET is histologically characterized by small blue round cells and molecularly defined by the fusion of FET (FUS, Fused in Sarcoma; EWS, Ewing sarcoma breakpoint region 1, [ abbreviated EWSR1]; and TAF15, TATA box binding protein associated factor 68 kDa) gene family with ETS (E26 transformation-specific) family; with the most common fusion being EWSR-1-FLT1 (Fmsrelated tyrosine kinase 1) [1, 2]. ES/PNET mainly affects adolescents and young adults, and has an aggressive nature. Metastatic status at diagnosis is the most significant prognostic factor of ES/PNET [3]. Patients with localized disease can expect 70–80% of 5-year overall survival.

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