PRENATALLY DIAGNOSED NEUROBLASTOMA

BACKGROUND. Prenatally diagnosed neuroblastomas have been reported in increasing numbers over the past several years, and there are now a fe w reviews based on up to 21 cases. The purpose of this article is to r eview the clinical and biologic features of prenatally diagnosed neuro blastoma based on a review of 55 cases. METHODS. A review was conducte d of 3 cases seen at the study institution and 52 other cases reported thus far in the literature. RESULTS. Prenatal diagnosis was made usua lly after 32 weeks of gestation. Approximately 93% of the tumors were adrenal in origin, and 44% of these were cystic. Thirty-seven patients (67%) had Stage I disease, 12 (22%) had Stage IV-S disease, and only 3 (5%) had Stage IV disease. The DNA index was favorable (>1) in 14 of 16 patients studied. None of these 16 patients studied had amplificat ion of the N-myc oncogene. Catecholamines were elevated in only 33% of the patients. The liver was the most common site of dissemination, wh ich was observed in 25% of patients; bone involvement was not observed in any patient. Ultrasonography failed to detect existing hepatic met astasis in three patients. Primary surgical resec tion was performed i n 47 patients (85%). Chemotherapy was given to five patients and radio therapy to three. Of the 50 patients for whom follow-up information wa s available, 45 (90%) were alive at a range of 2-120 months from diagn osis. CONCLUSIONS. Prenatally diagnosed neuroblastomas are predominant ly adrenal in origin and frequently cystic. The liver is the most comm on site of dissemination and bone involvement is notably absent. The v ast majority of these infants have a favorable stage of disease (I, II , and IV-S) and favorable biologic features, and consequently have an excellent prognosis. Although surgery alone is curative for most patie nts, a period of observation may avoid surgery in some individuals who may achieve spontaneous regression. (C) 1997 American Cancer Society.