Connatal localized neuroblastoma: The case to delay treatment

Abstract

BACKGROUND. Spontaneous regression is well documented for a subset of widespread neuroblastomas (Stage 4S) and for localized residual tumors after incomplete resection. Possible spontaneous regression of untreated localized neuroblastoma in infants is frequently discussed, but has very rarely been demonstrated clinically. METHODS. We report four patients with localized neuroblastoma detected early; all were tumors of the adrenal gland. One patient was detected antenatally by ultrasound, the other three tumors were detected incidentally by sonography at the ages of birth, 1 week, and 7 weeks, respectively. In three patients treatment was delayed in order to await a possible spontaneous regression, and in one patient treatment was delayed due to an uncertain diagnosis. RESULTS. Local tumor growth was observed in three patients, and the tumors were removed 7, 12, and 16 weeks, respectively, after the initial diagnosis of neuroblastoma. All three patients are free of disease. The fourth patient developed liver metastases 4 weeks after the first suspicion of neuroblastoma. Progressive disease ended in death at the age of 17 months. CONCLUSIONS. None of the four patients showed spontaneous tumor regression. Noninvasive examinations and invasive investigations (in two patients) were unable to predict the tumor's behavior. Based on present knowledge, a general 'wait and see' strategy cannot be recommended for early and incidentally detected neuroblastoma patients.