The Children's Oncology Group (COG) AREN0321 study was designed to investigate the management of patients with high-risk renal tumors (anaplastic Wilms tumor, clear cell sarcoma of the kidney, rhabdoid tumor, and RCC). 6 To our knowledge to date, there have been no prospective cooperative clinical trials for pediatric RCC. The importance of such review has been reinforced by a recent report noting a higher prevalence of tRCC than previously was recognized with expert histologic reclassification. 1, 3- 5 Broad applicability of any insights reported to date has been limited by the small size of reports and the lack of expert centralized pathology review. 2- 4 A second distinguishing feature of pediatric RCC relates to a higher incidence of regional lymph node (LN) involvement, with an apparently more favorable prognosis when such LN disease is completely resected compared with adult RCC. For example, pediatric RCC is most commonly translocation-type RCC (tRCC), often harboring chromosomal translocations involving the TFE3 gene at Xp11.2, rather than the clear cell RCC typically diagnosed in adults. However, recently published work has clearly demonstrated that pediatric RCC is biologically and clinically distinct from most adult RCC cases.
AREN0321 QARC SERIES
1, 2 Compared with other pediatric renal tumors, our knowledge of RCC is limited, and treatment recommendations are based on small retrospective case series and reports 1- 5 or have been taken from guidelines for “adult” RCC. Renal cell carcinoma (RCC) is the second most common solid renal malignancy in pediatric and adolescent patients, accounting for 2% to 6% of primary renal tumors diagnosed in this population. A prospective study of patients with tRCC and RMC with M1 or recurrent disease is needed to optimize treatment. Conclusionsįavorable short-term outcomes can be achieved without adjuvant therapy in children and adolescents with completely resected RCC, independent of lymph node status. The predominant RCC subtypes associated with mortality were tRCC and RMC. Among patients presenting with metastases, 2 of 8 patients (2 of 5 patients with RMC) were alive (1 with disease) at the time of last follow-up, including 1 patient who was lost to follow-up (succinate dehydrogenase deficiency).
Lymph node status was N0 in 21 patients, N1 in 21 patients (tRCC in 15 patients, RMC in 3 patients, papillary RCC in 2 patients, and not otherwise specified and/or other in 1 patient), and Nx in 26 patients.
Histology was TFE-associated RCC (translocation-type RCC tRCC) in 40 patients, RCC not otherwise specified and/or other in 13 patients, papillary RCC in 9 patients, and renal medullary carcinoma (RMC) in 6 patients. Surgery included radical nephrectomy (53 patients ), partial nephrectomy (12 patients ), and unknown (3 patients ). Sixty patients underwent resection of all known sites of disease, including 2 patients with stage IV disease. Stage was classified according to the American Joint Committee on Cancer TNM stage seventh edition as stage I in 26 patients, stage II in 7 patients, stage III in 26 patients, and stage IV in 8 patients, and was not available in 1 patient. ResultsĪ total of 68 patients were enrolled (39 of whom were male median age of 13 years ). Methodsįrom 2006 to 2012, patients aged <30 years with centrally reviewed pathology of RCC were enrolled prospectively. Goals of the study included establishing epidemiological, treatment, and outcome data and confirming that patients with completely resected pediatric RCC, including lymph node–positive disease (N1), have a favorable prognosis without adjuvant therapy. To the authors' knowledge, AREN0321 is the first prospective clinical study of pediatric and adolescent renal cell carcinoma (RCC).
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