Standard Treatment Options
Survival of patients with renal cell carcinoma (RCC) is affected by stage of disease at presentation and the completeness of resection at radical nephrectomy. Overall survival rates range from 64% to 87%. The 5-year survival for stage I is 90% or higher, for stages II and III it is 50% to 80%, and for stage IV it is 9%, which is similar to the stage-for-stage survival in RCC in adults. Retrospective analyses and the small number of patients involved place limitations...
Added text to state that recent reports suggest that positron emission tomography–computed tomography (PET-CT) may aid in staging esthesioneuroblastoma (cited Broski et al. as reference 30).
Added Redlich et al. as reference 67 and Fu et al. as reference 128.
Added Venkatramani et al. as reference 56 and Yalçin et al. as reference 68.
Revised text to state that the most common primary benign heart tumors in children are rhabdomyomas, myxomas, teratomas, and fibromas (cited Becker and Padalino et al. as references 81 and 82, respectively). Also revised text to state that other benign tumors include histiocytoid cardiomyopathy tumors, hemangiomas, and neurofibromas (i.e., tumors of the nerves that innervate the muscles).
Added text to state that hypomethylation of the KCNQ1OT1 gene has also been associated with the development of adrenocortical tumors in patients without the phenotypic features of Beckwith-Wiedemann syndrome (cited Wijnen et al. as reference 15).
Added Speer et al. as reference 65 and level of evidence 3iiDiii.
Added text to state that in contrast to juvenile granulosa cell tumors, a recent study suggested that Sertoli-Leydig tumors with abdominal spillage should be treated with cisplatin-based chemotherapy.
Other Rare Childhood Cancers
Added Burnichon et al. as reference 45.
Revised Table 7 to state that MAX germline mutations account for less than 2% of all paragangliomas and pheochromocytomas, and the mean age at presentation is 34 years.
This summary is written and maintained by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ NCI's Comprehensive Cancer Database pages.