OBJECTIVE: In the US, second non-ocular malignancies are the primary cause of death in retinoblastoma survivors with the germline RB1 mutation. Soft tissue sarcomas are one of the most likely malignancies to pose a risk to these patients, with leiomyosarcoma (LMS) being the most common subtype. As our cohort is followed for a longer period, we discover new second malignancy risks for these patients. METHODS: We estimated the risk for uterine leiomyosarcoma (ULMS) in a cohort of 1854 patients with retinoblastoma who were diagnosed at two US institutions from 1914 through 1996. The standardized incidence ratio and excess absolute risk were calculated by comparison with population data from the Connecticut Tumor Registry or from National Cancer Institute Surveillance, Epidemiology, and End Results (SEER) database. The cumulative risk at 50 years of age was also calculated. RESULTS: Seven of 525 female hereditary retinoblastoma patients developed ULMS. Five of these patients were used in the risk analysis, resulting in an excess risk of 3.87 per 10,000 women. Among hereditary patients who developed ULMS the excess risk increases dramatically with age: to 20/10,000 for female hereditary retinoblastoma patients aged between 30 and 39 years, and to 27/10,000 for patients aged 40+ years. CONCLUSION: There is a substantial excess risk of ULMS in female hereditary retinoblastoma patients. As more patients survive into their thirties, this number is likely to increase. These findings raise the question of early childbearing, screening and prophylactic measures in hereditary retinoblastoma patients: all issues that would benefit from confirmation from other retinoblastoma cohorts, to allow for better guided counsel of these patients.
OBJECTIVE: In the US, second non-ocular malignancies are the primary cause of death in retinoblastoma survivors with the germline RB1 mutation. Soft tissue sarcomas are one of the most likely malignancies to pose a risk to these patients, with leiomyosarcoma (LMS) being the most common subtype. As our cohort is followed for a longer period, we discover new second malignancy risks for these patients. METHODS: We estimated the risk for uterine leiomyosarcoma (ULMS) in a cohort of 1854 patients with retinoblastoma who were diagnosed at two US institutions from 1914 through 1996. The standardized incidence ratio and excess absolute risk were calculated by comparison with population data from the Connecticut Tumor Registry or from National Cancer Institute Surveillance, Epidemiology, and End Results (SEER) database. The cumulative risk at 50 years of age was also calculated. RESULTS: Seven of 525 female hereditary retinoblastomapatients developed ULMS. Five of these patients were used in the risk analysis, resulting in an excess risk of 3.87 per 10,000 women. Among hereditarypatients who developed ULMS the excess risk increases dramatically with age: to 20/10,000 for female hereditary retinoblastomapatients aged between 30 and 39 years, and to 27/10,000 for patients aged 40+ years. CONCLUSION: There is a substantial excess risk of ULMS in female hereditary retinoblastomapatients. As more patients survive into their thirties, this number is likely to increase. These findings raise the question of early childbearing, screening and prophylactic measures in hereditary retinoblastomapatients: all issues that would benefit from confirmation from other retinoblastoma cohorts, to allow for better guided counsel of these patients.
Authors: D Lawrence Wickerham; Bernard Fisher; Norman Wolmark; John Bryant; Joseph Costantino; Leslie Bernstein; Carolyn D Runowicz Journal: J Clin Oncol Date: 2002-06-01 Impact factor: 44.544
Authors: F Amant; M de la Rey; C M Dorfling; L van der Walt; G Dreyer; L Dreyer; I Vergote; B G Lindeque; E J Van Rensburg Journal: Gynecol Oncol Date: 2002-04 Impact factor: 5.482
Authors: Robert L Giuntoli; Daniel S Metzinger; Connie S DiMarco; Stephen S Cha; Jeff A Sloan; Gary L Keeney; Bobbie S Gostout Journal: Gynecol Oncol Date: 2003-06 Impact factor: 5.482
Authors: Danielle Novetsky Friedman; Joanne F Chou; Kevin C Oeffinger; Ruth A Kleinerman; Jennifer S Ford; Charles A Sklar; Yuelin Li; Mary S McCabe; Leslie L Robison; Brian P Marr; David H Abramson; Ira J Dunkel Journal: Cancer Date: 2016-01-11 Impact factor: 6.860
Authors: Emily S Tonorezos; Danielle Novetsky Friedman; Dana Barnea; Machteld I Bosscha; Guillermo Chantada; Charlotte J Dommering; Pim de Graaf; Ira J Dunkel; Armida W M Fabius; Jasmine H Francis; Mary-Louise C Greer; Ruth A Kleinerman; Wijnanda A Kors; Suzanne Laughlin; Annette C Moll; Lindsay M Morton; Petra Temming; Margaret A Tucker; Flora E van Leeuwen; Michael F Walsh; Kevin C Oeffinger; David H Abramson Journal: Ophthalmology Date: 2020-05-15 Impact factor: 12.079
Authors: Ruth A Kleinerman; Margaret A Tucker; Byron S Sigel; David H Abramson; Johanna M Seddon; Lindsay M Morton Journal: J Natl Cancer Inst Date: 2019-09-01 Impact factor: 13.506
Authors: Jennifer S Ford; Joanne F Chou; Charles A Sklar; Kevin C Oeffinger; Danielle Novetsky Friedman; Mary McCabe; Leslie L Robison; Ruth A Kleinerman; Yuelin Li; Brian P Marr; David H Abramson; Ira J Dunkel Journal: J Clin Oncol Date: 2015-09-28 Impact factor: 44.544
Authors: Inga-Marie Schaefer; Meijun Z Lundberg; Elizabeth G Demicco; Joanna Przybyl; Magdalena Matusiak; Frédéric Chibon; Davis R Ingram; Jason L Hornick; Wei-Lien Wang; Sebastian Bauer; Laurence H Baker; Alexander J Lazar; Matt van de Rijn; Adrian Mariño-Enríquez; Jonathan A Fletcher Journal: Cancer Date: 2021-03-31 Impact factor: 6.860
Authors: A MacCarthy; A M Bayne; P A Brownbill; K J Bunch; N L Diggens; G J Draper; M M Hawkins; H C Jenkinson; J E Kingston; C A Stiller; T J Vincent; M F G Murphy Journal: Br J Cancer Date: 2013-05-14 Impact factor: 7.640