Ewing sarcoma is a rare tumor that occurs commonly in the long bones of children or adolescents that can also arise in soft tissues including the extremities, retroperitoneum, chest wall, and rarely in the liver as primary sites. We report a case of Ewing sarcoma arising primarily in the liver and, to our knowledge, this is the fourth reported case of Ewing sarcoma occurring in the liver. A 27-year-old Japanese woman was admitted with sudden onset right upper abdominal pain. Clinical examination revealed a multilocular cystic mass consisting of thickened, irregular septa and nodal walls in the right hepatic lobe. Ultrasound-guided aspiration biopsy of the liver mass showed clusters of small atypical round cells and the clinical preoperative diagnosis was mucinous cystadenoma of the liver. The patient underwent an extended right hepatectomy and histopathological findings revealed sheet-like proliferation of small- to medium-sized round cells. Tumor cells were positive for periodic acid-Schiff reaction and immunoreactive for glycoprotein C99 and gene NKX2.2, as well as the neuroendocrine markers, CD56 and synaptophysin. EWS-FLI-1 fusion transcript type 1 was detected by reverse transcriptase polymerase chain reaction. Pathological and molecular analysis confirmed the diagnosis of Ewing sarcoma arising primarily in the liver and the patient received adjuvant systemic chemotherapy with vincristine, doxorubicin, and cyclophosphamide, alternating with ifosfamide and etoposide. We found no evidence of recurrence 15 months after completing chemotherapy. We present an extremely rare case of Ewing sarcoma arising primarily in the liver. To our knowledge, this is the fourth reported case of Ewing sarcoma occurring in the liver, and the first case with a multilocular cystic liver mass. Imaging examinations of the other three reported cases showed solid tumors and a diffuse enlarged liver without mass lesion. Clinicians should consider the possibility of Ewing sarcoma in young patients with a multilocular cystic mass with thick and/or irregular cyst walls in the liver.

10070 Background: Cancer patients receiving chemotherapy experience physical and psychosocial distress that negatively influences quality of life (QOL). Self-help workbook is expected to support them to cope with the distress, to facilitate communication with medical staff, and to improve QOL. We conducted a randomized controlled trial to evaluate the effectiveness of self-help workbook intervention on QOL. Methods: Japanese patients with breast, colorectal, gastric, and lung cancer receiving chemotherapy on an outpatient basis were randomized to the intervention arm or the control arm. In the intervention arm, patients received workbooks originally made for this study, read advice on how to cope with problems related to disease or treatment, and filled out questionnaires on the workbooks periodically. In the control arm, patients were treated with standard care without the workbooks. EORTC QLQ-C30, cancer site-specific modules (EORTC QLQ-BR23, CR29, STO22, or LC13), and HADS were evaluated at baseline, after 12 weeks, and after 24 weeks. The primary outcome measure was global QOL (GQOL). Two-way repeated-measures analysis of variance was used to investigate the intervention effect. Results: From June 2014 to March 2015, 200 patients were enrolled to this study. Median age was 58 years (range 28-83), 81% of patients were female, 65% metastatic disease, 68% breast, 23% colorectal, 5.5% gastric, and 3.5% lung cancer. 90% of 100 patients in the intervention arm and 91% of 100 patients in the control arm completed questionnaires at all the time points. At baseline, after 12 weeks, and after 24 weeks, mean scores of GQOL were 68, 64, and 64 in the intervention arm and 63, 61, and 61 in the control arm, respectively. GQOL did not significantly improve in the intervention arm as compared with in the control arm. Among patients with non-metastatic disease, emotional functioning scores of EORTC QLQ-C30 tended to improve in the intervention arm. Conclusions: Although self-help workbook intervention was feasible in cancer patients receiving chemotherapy on an outpatient basis, the effect of the intervention on QOL was limited in this study. Clinical trial information: UMIN000012842.