© The Author 2007. Published by Oxford University Press.
ARTICLE |
Risk of Soft Tissue Sarcomas by Individual Subtype in Survivors of Hereditary Retinoblastoma
Affiliations of authors: Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Department of Health and Human Services, Rockville, MD (RAK, MAT, JFF); Ophthalmic Oncology Service, Memorial Sloan-Kettering Cancer Center, New York, NY (DHA); Epidemiology Service, Massachusetts Eye and Ear Infirmary, Boston, MA (JMS); International Epidemiology Institute, Rockville, MD (RET).
Correspondence to: Ruth A. Kleinerman, MPH, Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Department of Health and Human Services, EPS 7044, 6120 Executive Blvd., Rockville, MD 20852 (e-mail: kleinerr{at}mail.nih.gov).
BACKGROUND: Survivors of hereditary retinoblastoma have an increased risk for second malignancies, especially soft tissue sarcomas. However, the risks of individual histologic subtypes of soft tissue sarcomas have not been evaluated.
METHODS: We estimated the risk for six subtypes of soft tissue sarcomas (fibrosarcoma, liposarcoma, histiocytoma, leiomyosarcoma, rhabdomyosarcoma, and others) in a cohort of 963 one-year survivors of hereditary retinoblastoma among patients diagnosed at two US institutions from 1914 through 1984. We calculated standardized incidence ratios (SIRs) for specific subtypes of soft tissue sarcomas by comparison with population data from the Connecticut Tumor Registry or from National Cancer Institute Surveillance, Epidemiology, and End Results database. We also calculated the cumulative risk for all soft tissue sarcomas combined.
RESULTS: We observed 69 soft tissue sarcomas in 68 patients with hereditary retinoblastoma. Risks were elevated for soft tissue sarcomas overall (SIR = 184, 95% confidence interval [CI] = 143 to 233) and for individual subtypes. Leiomyosarcoma was the most frequent subtype (SIR = 390, 95% CI = 247 to 585), with 78% of leiomyosarcomas diagnosed 30 or more years after the retinoblastoma diagnosis (SIR = 435, 95% CI = 258 to 687). Among patients treated with radiotherapy for retinoblastoma, we found statistically significantly increased risks of soft tissue sarcomas in the field of radiation. Irradiated patients also had increased risks of soft tissue sarcomas, especially leiomyosarcomas, outside the field of radiation, and risks of soft tissue sarcomas were increased in nonirradiated patients as well, indicating a genetic predisposition to soft tissue sarcomas independent of radiation. The cumulative risk for any soft tissue sarcoma 50 years after radiotherapy for retinoblastoma was 13.1% (95% CI = 9.7% to 17.0%).
CONCLUSION: Long-term follow-up of a cohort of survivors of hereditary retinoblastoma revealed a statistically significant excess of leiomyosarcoma and other soft tissue sarcomas that persists decades after the retinoblastoma diagnosis. Retinoblastoma survivors should undergo regular medical surveillance for sarcomas in their adult years.
| CONTEXT AND CAVEATS Prior knowledge People who have survived hereditary retinoblastoma have a higher risk of soft tissue sarcomas overall than the general population, but risks of individual soft tissue sarcoma subtypes have not been estimated. Study design Risk of sarcomas in retinoblastoma survivors at two institutions was compared with general population rates. Contribution In this large, long-term study, risks of six different soft tissue sarcoma subtypes were evaluated. Survivors had nearly 400 times the risk of leiomyosarcomas than the general population. Risks of some subtypes remained high for several decades. Almost all of the soft tissue sarcomas were diagnosed in patients who received radiation, although some tumors developed outside the field of radiation. Implications Patients with hereditary retinoblastoma may have a genetic predisposition to soft tissue sarcomas. Long-term surveillance for sarcomas may be warranted in survivors of hereditary retinoblastoma. Limitations Conclusions for some subtypes were based on small numbers of cases. Patients came from two institutions, limiting generalizability.
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Editorial about this Article
- Retinoblastoma Survivors: Sarcomas and Surveillance
- Anna T. Meadows
J Natl Cancer Inst 2007 99: 3-5.[Extract] [Full Text] [PDF]
Related Article in JNCI
- Press Release: Study Examines Risk of Soft Tissue Sarcomas in Hereditary Retinoblastoma Survivors
- Andrea Widener
J Natl Cancer Inst 2007 99: 1.[Extract] [Full Text]
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