Secondary bone/soft tissue sarcoma in childhood cancer survivors: A nationwide hospital-based case-series study in Japan

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, Tatsuo Kuroda

Research output: Contribution to journalArticlepeer-review

9 Citations (Scopus)


Background: Secondary 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). Methods: We 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. Results: The 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. Conclusion: The prognoses of CCSs with secondary sarcoma may depend on the primary cancer or prior relapse of primary cancer.

Original languageEnglish
Pages (from-to)806-814
Number of pages9
JournalJapanese journal of clinical oncology
Issue number9
Publication statusPublished - 2018 Sept 1


  • Bone/soft tissue sarcoma
  • Childhood cancer
  • Late effects
  • Secondary cancer
  • Secondary sarcoma

ASJC Scopus subject areas

  • Oncology
  • Radiology Nuclear Medicine and imaging
  • Cancer Research


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