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Titolo:
MORTALITY FROM 2ND TUMORS AMONG LONG-TERM SURVIVORS OF RETINOBLASTOMA
Autore:
ENG C; LI FP; ABRAMSON DH; ELLSWORTH RM; WONG FL; GOLDMAN MB; SEDDON J; TARBELL N; BOICE JD;
Indirizzi:
NCI,RADIAT EPIDEMIOL BRANCH,EPN 408,6130 EXECUT BLVD ROCKVILLE MD 20852 NCI,RADIAT EPIDEMIOL BRANCH,EPN 408,6130 EXECUT BLVD ROCKVILLE MD 20852 HARVARD UNIV,SCH MED,DANA FARBER CANC INST,DIV MED ONCOL BOSTON MA 02115 HARVARD UNIV,SCH MED,DANA FARBER CANC INST,DIV CANC EPIDEMIOL & CONTROL BOSTON MA 02115 BOSTON CHILDRENS HOSP,JOINT CTR RADIAT THERAPY BOSTON MA 00000 HARVARD UNIV,SCH MED BOSTON MA 02115 MASSACHUSETTS EYE & EAR INFIRM BOSTON MA 02114 CORNELL UNIV,MED CTR,NEW YORK HOSP,CTR OPHTHALM ONCOL NEW YORK NY 10021 HARVARD UNIV,SCH PUBL HLTH CAMBRIDGE MA 02138
Titolo Testata:
Journal of the National Cancer Institute
fascicolo: 14, volume: 85, anno: 1993,
pagine: 1121 - 1128
Fonte:
ISI
Lingua:
ENG
Soggetto:
NON-OCULAR CANCER; BILATERAL RETINOBLASTOMA; HUMAN SARCOMAS; OSTEO-SARCOMA; SUSCEPTIBILITY GENE; MALIGNANT NEOPLASMS; NONOCULAR TUMORS; BONE SARCOMAS; EXPRESSION; DELETION;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
54
Recensione:
Indirizzi per estratti:
Citazione:
C. Eng et al., "MORTALITY FROM 2ND TUMORS AMONG LONG-TERM SURVIVORS OF RETINOBLASTOMA", Journal of the National Cancer Institute, 85(14), 1993, pp. 1121-1128

Abstract

Background: Children diagnosed with retinoblastoma, a rare cancer of the eye, tend to develop and die of second primary cancers in childhood and adolescence, but few investigations have followed patients into adulthood. Retinoblastoma is frequently caused by inherited mutations of the RB1 tumor suppressor gene. Most patients with germline (hereditary) mutations have bilateral disease. Purpose: We sought to quantify the mortality from second malignancies among long-term survivors of retinoblastoma and to identify factors that predispose to these deaths. Methods: A retrospective cohort study examined mortality among 1603 patients enrolled at 1 year after diagnosis of retinoblastoma during theperiod 1914-1984. Data on demography, family history, and retinoblastoma treatment were collected by medical chart review and questionnaireinterview. Number of deaths, by cause, was compared with the corresponding expected figure based on U.S. mortality data for the general population for 1925-1990. Results: Follow-up was complete for 1458 patients (91%) for a median of 17 years after retinoblastoma diagnosis. A total of 305 deaths occurred, 167 of them from retinoblastoma. There were 96 deaths from second primary tumors (relative risk [RR] = 30), 21 from other known causes (RR = 1.0), and 21 from ill-defined or unknown causes. Statistically significant excess mortality was found for second primary cancers of bone, connective tissue, and malignant melanoma and benign and malignant neoplasms of brain and meninges. Among 919 children with bilateral retinoblastoma, 90 deaths from second primary tumors occurred (RR = 60). Deaths from second tumors were more frequent among females (RR = 39) than males (RR = 22) (P = .007). The cumulativeprobability of death from second primary neoplasms was 26% at 40 years after bilateral retinoblastoma diagnosis, and additional cancer deaths occurred thereafter. Radiotherapy for retinoblastoma further increased the risk of mortality from second neoplasms. An excess of mortality from a second cancer, not seen in prior studies, was found among the684 children with unilateral disease (RR = 3.1; 95% confidence interval = 1.0-7.3). Conclusions: These findings implicate germinal mutations in the retinoblastoma gene in second cancer mortality. Radiotherapy treatment for retinoblastoma appears to further enhance the inborn susceptibility to development of a second cancer. Implications: Patients with retinoblastoma, particularly bilateral retinoblastoma, should have careful follow-up, and interventions should be developed to reduce mortality from a second cancer.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 21/09/20 alle ore 14:06:08