title: Second malignant neoplasms in children with retinoblastoma: the St. Jude Children's Research Hospital experience.
abstract: A retrospective review of 172 children with primary diagnosis of retinoblastoma (RB) was completed at St. Jude Children's Research Hospital to evaluate the incidence of second malignant neoplasm (SMN) development. Sixty-five patients presented with bilateral RB and 107 with unilateral RB. During follow-up, which ranged from 6 to 340 months (median = 170 months), 6 children (3.5%) developed SMN. All patients who developed SMN presented with bilateral disease (n = 5) or asynchronous bilateral disease (n = 1); two patients had a family history of RB. All had received irradiation. Four patients developed osteogenic sarcoma within this irradiated volume, one developed a basal cell carcinoma in the temporal region (within the irradiation field), and one was diagnosed with a lower extremity Ewing's sarcoma. Time to development of SMN ranged from 125 to 194 months post-irradiation. Initial irradiation total dose ranged between 32 and 45.76 Gy. Three patients were treated with anterior field irradiation and three received lens-sparing techniques (anterior/lateral n = 2, lateral n = 1). At last follow-up, 4/6 patients had died of SMN. The crude incidence is 3.5% with an estimation of risk using the density method of 24% at 20 years for SMN development. The specifics of the treatment associated with these second malignancies and the possible reasons for the reported incidence of SMN will be discussed.
authors.affiliation: Department of Radiation Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA.
authors.forename: J
authors.initials: J
authors.lastname: Fontanesi
authors.forename: D M
authors.initials: DM
authors.lastname: Parham
authors.forename: C
authors.initials: C
authors.lastname: Pratt
authors.forename: D
authors.initials: D
authors.lastname: Meyer
mesh_terms: D002648:Child; D002675:Child, Preschool; D005134:Eye Neoplasms / Q000150:complications / Q000473:pathology / Q000532:radiotherapy*; D005500:Follow-Up Studies; D006776:Hospitals, Pediatric; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D009381:Neoplasms, Radiation-Induced / Q000453:epidemiology* / Q000209:etiology; D016609:Neoplasms, Second Primary / Q000453:epidemiology* / Q000209:etiology; D012175:Retinoblastoma / Q000150:complications / Q000473:pathology / Q000532:radiotherapy*; D012189:Retrospective Studies; D012307:Risk Factors; D013714:Tennessee / Q000453:epidemiology