INTERNATIONAL NEUROBLASTOMA STAGING SYSTEM STAGE-1 NEUROBLASTOMA - A PROSPECTIVE-STUDY AND LITERATURE-REVIEW

Purpose: To gain insight into the management of nonmetastatic neurobla stoma by examining clinical and biologic features of International Neu roblastoma Staging System (INSS) stage I tumors. Methods: Patients wer e staged by both the INSS and the Evans staging system and were evalua ted for biologic prognostic factors. Patients with INSS stage 1 receiv ed no cytotoxic therapy, The literature was reviewed for clinical and biologic data about INSS stage 1. Results: We evaluated 10 consecutive patients (median age, 17.5 months) with INSS stage 1; all remain dise ase-free (median follow-vp duration, > 5 years), tumors were in the ab domen (n = 6), chest (n = 3), or pelvis (n = 1), Neuroblastoma involve d margins of resection in six tumors, Poor-prognostic biologic finding s included tumor-cell diploidy (n = 2) and unfavorable Shimada histopa thology (n = 2). Two patients were to receive chemotherapy for, respec tively, a tumor deemed unresectable and a tumor classified as Evans st age III; second opinions resulted in surgical management alone in each case. Published reports confirm that some INSS stage 1 patients (1) a re at risk for overtreatment, and (2) have poor-prognostic biologic fi ndings yet do well. Conclusion: Surgery alone suffices for INSS stage 1 neuroblastoma, even if biologic prognostic factors are unfavorable, microscopic disease remains after surgery, and tumor size is suggestiv e of ''advanced-stage'' status in other staging systems, Attempts to r esect regionally confined neuroblastomas should take precedence over i mmediate use of cytotoxic therapy; otherwise, some patients may receiv e chemotherapy or radiotherapy unnecessarily.