• Yasushi Ishida, Miho Maeda, Souichi Adachi, Takeshi Rikiishi, Maho Sato, Hiroshi Kawaguchi, Atsushi Manabe, Mika Tokuyama, Hiroki Hori, Jun Okamura, Atsushi Ogawa, Hiroaki Goto, Ryoji Kobayashi, Shinji Yoshinaga, Junichiro Fujimoto, and Tatsuo Kuroda.
• Pediatric Medical Center, Ehime Prefectural Central Hospital, Matsuyama.
• Jpn. J. Clin. Oncol. 2018 Sep 1; 48 (9): 806-814.

BackgroundSecondary cancer is the most life-threatening late effect of childhood cancer. We investigated the clinical features of secondary bone/soft tissue sarcoma among childhood cancer survivors (CCSs).MethodsWe conducted a retrospective case-series study of 10 069 CCSs newly diagnosed with cancer between 1980 and 2009 across 15 Japanese hospitals. Twenty-one cases of pathologically diagnosed secondary bone/soft tissue sarcoma were selected, and the respective clinical courses were determined using additional questionnaires.ResultsThe primary cancers included retinoblastoma (n = 7), acute lymphoblastic leukemia (n = 5), lymphoma (n = 5), osteosarcoma (n = 1), rhabdomyosarcoma (n = 1), brain tumor (n = 1) and Langerhans cell histiocytosis (n = 1). The median age at the primary cancer diagnosis was 2.9 years, and the male-to-female ratio was 16:5. The histological classifications of the secondary sarcoma included osteosarcoma (n = 10), malignant peripheral nerve sheath tumor (n = 4), rhabdomyosarcoma (n = 3), Ewing's sarcoma (n = 3) and primitive neuroectodermal tumor (n = 1). The median latency period to the secondary sarcoma was 10.2 years. Significant risk factors for secondary sarcoma in the multivariate Cox regression model included a history of retinoblastoma as the primary cancer (hazard ratio [HR], 20.9; 95% confidence interval [CI], 5.70-76.5) and autologous stem cell transplantation (SCT) (HR, 2.56; 95% CI, 1.08-6.03). Seventeen CCSs with secondary sarcoma underwent radiation, and nine, hematopoietic SCT. Twelve CCSs with secondary sarcoma achieved disease-free survival, while CCSs with hematological cancer or relapsed primary cancer who developed secondary sarcoma had the worst prognoses.ConclusionThe prognoses of CCSs with secondary sarcoma may depend on the primary cancer or prior relapse of primary cancer.

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