Features and outcome of neuroblastoma detected before birth

Background/Purpose:The growing use of routine ultrasonography during pregna ncy is leading to an increasing number of prenatally diagnosed neuroblastom as. Optimal strategy has not yet been defined for these patients, because k nowledge on this particular neuroblastoma (NB) population is still limited. However, definite guidelines are needed to avoid inadequate treatment. The authors analyzed the cases of antenatally detected NE (ADNB) reported in t he Italian Neuroblastoma Registry during the past 6 years to elucidate the features of this subset of NE. Methods: The Italian Neuroblastoma Registry was reviewed for the period Jan uary 1993 to December 1998 to collect clinical, radiographic, surgical, and histopathological data on ADNB cases. NE stage was evaluated according to INSS criteria. All patients had undergone imaging (computed tomography or m agnetic resonance imaging) of the primary tumor and bone marrow biopsy befo re surgical resection. Results: Seventeen patients were identified. Primary tumour site was adrena l glands in 16 cases and retroperitoneal ganglia in 1. Stage distribution w as stage 1, 13 cases; stage II-A, 1 case; stage II-B, 1 case; stage IV-S, 2 cases. All cases underwent primary tumour resection. Mean age at surgery w as 4 weeks. Resection of primary tumor was radical in 16 cases, partial in 1. All tumors were characterised by favourable histology according to Shima da classification. N-myc gene amplification was studied in 14 patients. N-m yc amplification was detected only in a newborn with stage It-A NE, who die d of massive bleeding 2 days after tumor resection. DNA index and Ip deleti on were studied in 11 and 8 patients, respectively. Both diploidy and delet ion of Ip were observed in a newborn who subsequently died of disease progr ession despite surgery, chemotherapy, and radiation therapy. Fourteen of 17 patients currently a re alive a nd free of disease, and one with IV-S NB a nd short follow-up is alive with disease. Conclusions: Our data give evidence that in most cases infants with ADNB re present a subset of patients with excellent outcome. Aggressive treatment m ay not always be necessary. Infants with ADNB with unfavorable features sho uld undergo early surgical excision, whereas patients with favourable featu res could be observed awaiting spontaneous regression of the mass, reservin g delayed surgery for tumors that increase in size or do not regress. Copyr ight (C) 2000 by W.B. Saunders Company.