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Completely resectable tumors have a favorable prognosis if they are small, whereas disease recurs in 50% of patients with large, localized tumors. It still remains to be defined which patients benefit from more aggressive therapy in addition to complete tumor resection. Briefly, no additional therapy was recommended for patients with completely resected stage I, II, and III (T3, N0, M0) tumors (AJCC 7th staging system). Four cycles of chemotherapy (alternating NN-1: vincristine, ifosfamide, doxorubicin, and NN-2: carboplatin and etoposide) with mitotane over a nine-month period were advised for patients with stage III (T1-2, N1, and M0) tumors, while patients with stage IV tumors should have received eight cycles of chemotherapy with mitotane over an 18-month period. Patients aged ≥ 4 years more frequently presented with advanced tumor stages, including distant metastases, compared to younger patients. Noteworthy is that complete tumor resection was more frequently achieved in younger patients, while tumor spillage occurred with increasing frequency in older patients. In the ARAR0332 study, the overall survival of stage II patients with completely resected large tumors was only 78.8%. In patients with R1/2 resection and/or spillage, as well as in stage III and IV patients, chemotherapy plus mitotane is recommended. In the ARAR0332 protocol, with the combination of chemotherapy plus mitotane, the 5-year overall survival in stage III patients was excellent (94.7%) but poor (7.1%) in stage IV patients receiving the same regimen. The outcome in patients with advanced and metastatic ACTs was poor and was best predicted by the COG stage and five-item microscopic score. @@
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