Long-term survival in refractory recurrent neuroblastoma.
BACKGROUND: Patients older than age 1 year with metastatic neuroblastoma have poor survival. A patient with a unique history of metastatic neuroblastoma by virtue of disseminated nodal disease with multiple relapses and a 14-year survival is presented. METHODS: A case history of a 24-year-old man who was diagnosed at age 9 years with Evans stage IV neuroblastoma with multiple recurrences is reviewed. A review of the literature was undertaken to uncover similar patients with advanced stage disease and an absence of hematogenous metastasis. RESULTS: Eleven patients older than age 1 year, in addition to the case presented, have been reported in the literature with stage IV disease without extralymphatic metastasis. These patients have a survival of 50% at 5 years as compared with 15% in historical controls. Long-term survival with recurrent disease has not previously been noted in this population. Other favorable prognostic factors including favorable histology, and a single N-myc oncogene copy were documented in the present case. CONCLUSION: A tendency toward improved survival is seen in patients with nonhematogeneous stage IV neuroblastoma. It is unknown whether long-term survival with residual disease, as observed in the present case, is seen in other patients with stage IV disease without extralymphatic metastasis. Prospective analysis of other patients with stage IV neuroblastoma without extralymphatic metastasis is required to confirm these observations.