Secondary skull base malignancies in survivors of retinoblastoma: The Memorial Sloan Kettering Cancer Center experience Journal Article
| Authors: | Liu, J. C.; Givi, B.; Wolden, S.; Kleinerman, R. A.; Dunkel, I. J.; Lee, N.; Shah, J. P.; Abramson, D. H.; Kraus, D. H. |
| Article Title: | Secondary skull base malignancies in survivors of retinoblastoma: The Memorial Sloan Kettering Cancer Center experience |
| Abstract: | We report the pathology and outcome of secondary skull base tumors in patients previously treated with external beam radiation for retinoblastoma (Rb). Rb patients are at increased risk of second head and neck primary malignancies due to early radiation exposure during treatment and loss of RB1 protein in genetic carriers. An institutional database was reviewed for patients with retinoblastoma who had previously received radiation therapy and subsequently developed skull base tumors. Seventeen patients met the selection criteria. The median age of Rb diagnosis was 12 months. Thirteen cases underwent enucleation in addition to radiation therapy as part of initial Rb treatment. A median of 19 years elapsed between the diagnosis of Rb and diagnosis of skull base malignancy. The most common tumors were osteogenic sarcoma (39%) and leiomyosarcoma (22%). Eleven (71%) patients received postoperative chemotherapy, and 7 (41%) received postoperative radiotherapy. Three (24%) patients underwent salvage surgery for recurrent disease. Five-year survival was 68%, and 10-year survival was 51% by Kaplan-Meier analysis. Secondary malignancy in Rb patients is a well-defined event. The use of surgery with appropriate adjuvant therapy was associated with a 51% 10-year survival in this study population. Copyright © 2011 by Thieme Medical Publishers, Inc. |
| Keywords: | osteosarcoma; cancer survival; child; clinical article; preschool child; overall survival; cancer recurrence; salvage therapy; cancer risk; cancer adjuvant therapy; postoperative care; neoplasms; adenocarcinoma; radiotherapy; retinoblastoma; heterozygote; data base; high risk patient; radiation exposure; cancer survivor; survival time; fibrosarcoma; infant; enucleation; external beam radiotherapy; skull base neoplasms; leiomyosarcoma; spindle cell carcinoma; skull base tumor; maxilla resection; second primary; retinoblastoma binding protein 1 |
| Journal Title: | Skull Base |
| Volume: | 21 |
| Issue: | 2 |
| ISSN: | 1531-5010 |
| Publisher: | Thieme Publishing |
| Date Published: | 2011-03-01 |
| Start Page: | 103 |
| End Page: | 108 |
| Language: | English |
| DOI: | 10.1055/s-0031-1275256 |
| PROVIDER: | scopus |
| PMCID: | PMC3312595 |
| PUBMED: | 22451810 |
| DOI/URL: | |
| Notes: | --- - "Export Date: 23 June 2011" - "CODEN: SBKAB" - "Source: Scopus" |
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