Neuroblastoma in adults and adolescents: an indolent course with poor survival.

BACKGROUND: Neuroblastoma rarely occurs in adults, and less than 10% of cases occur in patients older than 10 years. It has been suggested that the behavior of this disease may be different in older patients than in young children. The purpose of this study was to investigate the presentation, biologic features, and outcome of adolescent and adult patients with neuroblastoma to define differences from childhood neuroblastoma.
METHODS: Medical record and pathology reviews were conducted for 16 patients age 13 years or older (13-33 years) at diagnosis who presented with neuroblastoma at the University of California-San Francisco (UCSF) during the period 1968-1995 (patients with intracerebral tumors, esthesioneuroblastoma, or ganglioneuroma were excluded). Six of these patients received their original diagnosis at UCSF, and the others were referred after diagnosis. The survival for the same period for all neuroblastoma patients ages 13-18 years (n = 38) registered in the Children's Cancer Group (CCG) was compared with the survival for those ages 1-13 years (n = 1912). Three of the UCSF patients were enrolled in CCG studies.
RESULTS: Biologic characteristics observed in adolescents and adults differed from those observed in younger patients. In the UCSF population, only 6 of 15 tested patients age 13 or older had elevated urinary catecholamines, and 0 of 6 tested patients had MYCN amplification. There were two patients with Stage I disease, three with Stage II, two with Stage III, and nine with Stage IV. Primary sites were adrenal, pelvic, and retroperitoneal in four cases each; mediastinal in two cases; and paraspinal in two cases. Metastases in nine patients at diagnosis were observed in bone in five; in bone marrow in four; in lymph nodes in three; in the liver in two; and in the pleura, breast, and dura in one patient each. 131I-metaiodobenzylguanidine uptake was observed in 9 of 11 patients. Initial treatment included surgery for 13 of 16 patients, chemotherapy for 10 of 16, radiation therapy for 7 of 16, and autologous bone marrow transplantation for 1 of 16. Relapses occurred in 15 of 16 patients and death in 13 of 16, with overall survival 30% 5 years after diagnosis. Only 1 patient currently remains free of clinical disease 24 months after diagnosis. Several of these patients had long courses from diagnosis to death, with multiple recurrences and/or chronic, persistent disease. In the CCG data base, 76% of patients ages 13-18 years had metastatic disease at diagnosis. In this group, only 1 of 32 had MYCN amplification. The actuarial survival of all CCG patients ages 13-18 years was 7% at 5 years and 4% at 10 years, whereas that for patients ages 1-13 years was 30% at 5 years and 23% at 10 years.
CONCLUSIONS: Neuroblastoma in adolescents and adults has different biologic characteristics and a longer course than in children; nevertheless, ultimately the outcome is poor regardless of stage. A much more aggressive or innovative therapeutic approach is needed for these patients.