The biological behavior of primary cerebral neuroblastoma: a reappraisal of the clinical course in a series of 70 cases.

The outcome in 70 patients with primary cerebral neuroblastoma was evaluated in light of their age incidence, histological subtypes, and modes of treatment. In more than 80% of the patients, primary cerebral neuroblastoma occurred in the first decade, most often before the age of 2 years (26%). Over half of the tumors were histologically of the classic variant, the remainder showing varying degrees of mesenchymal stromal response (desmoplastic or transitional variants). Patients with a tumor of the desmoplastic or transitional variant without ganglionic differentiation had a lower mean age at the time of diagnosis than those harboring a tumor of the classic variant. Age could not be correlated with the presence of ganglionic differentiation. The overall 3-year survival rate was 60%. At least 30% of patients have a 5-year postoperative survival. Eight patients survived 7 years after diagnosis, and 3 for more than 10 years. The period of risk for tumor recurrence or death was a function of the initial age at diagnosis (Collins's law). Most deaths or fatal recurrences occurred within the first 3 years after operation. No correlation could be made between survival times and histological variant of the tumor or the presence of ganglionic differentiation. Our data do not indicate that, overall, postoperative irradiation with or without chemotherapy improved length of survival, but an occasional favorable clinical response to chemotherapy did occur. Because of the high incidence of local recurrence and the relative frequency of cerebrospinal metastasis, prophylactic whole brain and spinal irradiation is probably justified.