Late recurrence of a tumor of Ewing's sarcoma family of tumors: report of a case.

Takamasa Yun,Teruaki Mizobuchi,Hidemi Suzuki,Takekazu Iwata,Yukio Nakatani,Yuichi Sakairi,Ichiro Yoshino,Takahiro Nakajima,Shigetoshi Yoshida,Kaoru Nagato

doi:10.1186/s40792-015-0037-1

Abstract

A 27-year-old female presented with a history of a right chest wall tumor at 3 years of age. At that time, the tumor was surgically resected and diagnosed as Ewing’s sarcoma (EWS), and postoperative chemoradiotherapy was administered. The patient remained disease-free for 25 years. At age 27, chest computed tomography revealed a mass adjacent to the anterolateral thoracic wall. After surgery, the diagnosis was primitive neuroectodermal tumor (PNET). She died of the disease 10 months later. PNET and EWS were integrated into a single item in the 2002 WHO classification; thus, they are considered clinically and pathologically identical. The morphologic, immunohistochemical, and molecular biological characteristics of both specimens showed that the second tumor was a local recurrence of Ewing’s sarcoma family of tumors (ESFT). Our case is the longest duration local recurrence reported. Long-term recurrences of ESFT and patients with recurrent ESFT have a poor prognosis; thus, long-term follow-up is necessary.

Highlights

Tumors of Ewing’s sarcoma family of tumors (ESFT) are malignant tumors that originate in the central and peripheral nervous systems and soft tissues in children and adolescents
In 1921, James Ewing reported a rare type of sarcoma in children, adolescents, and young adults that is a highly malignant tumor of the bone and soft tissue, composed of small round cells [3]
With recent improvements in molecular biology, a translocation (11; 22) (q24; q12) specific to both Ewing’s sarcoma (EWS) and primitive neuroectodermal tumor (PNET) has been identified, and the EWS-FLI-1 fused gene was identified at the cleavage site of this translocation

Summary

Background

Tumors of Ewing’s sarcoma family of tumors (ESFT) are malignant tumors that originate in the central and peripheral nervous systems and soft tissues in children and adolescents. As either radiation-induced sarcoma or a local recurrence of EWS was suspected, we performed extirpation of the tumor and chest wall (the left second to the fourth ribs), partial resection of the right lung, and reconstruction of the chest wall (Figure 2b). Four cycles of VDC (vincristine, doxorubicin, cyclophosphamide) chemotherapy and two cycles of IE (ifosfamide, etoposide) and high-dose L-PAM (melphalan) and radiotherapy of left thigh bone for a total dose of 30 Gy was administered after the second operation She had recurrences in the right chest wall and upper middle lobe and metastases in the skull, rib, vertebrae, ilium, and thigh bone. She died of carcinomatous lymphangiosis 10 months after the second surgery

Findings

Discussion

Conclusions