Long-term follow-up of the "wait and see" approach to localized perinatal adrenal neuroblastoma.

BACKGROUND: Evidence-based guidelines for the management of localized perinatal adrenal neuroblastoma are not yet available. We describe our preliminary experience managing this tumor with a "wait and see" policy. METHODS: A single-center prospective study (February 2002 to December 2009) was conducted with 12 consecutive patients in whom an adrenal mass was detected antenatally or within the first 3 months of life. Diagnostic workup included the following investigations: measurement of urine catecholamine metabolites, imaging studies (ultrasonography, magnetic resonance imaging, or computed tomography), metaiodobenzylguanidine scintigraphy, and/or core needle biopsy. RESULTS: The male/female ratio was 1.4:1.0. Median tumor size at presentation was 29 mm (range 10-50 mm). Eight lesions were detected antenatally. Ten lesions were diagnosed as localized neuroblastoma. Of these ten lesions, four were excised because of parental preference (n = 2), tumor enlargement (n = 1) or tumor persistence (n = 1). The remaining six patients underwent watchful clinical observation, which showed progressive tumor shrinkage and complete regression within 10-39 months (median 12.5 months). The final two lesions were small predominantly cystic lesions without a clear-cut diagnosis. They were managed noninvasively. At an overall median follow-up of 109 months (range 30-122 months), all patients are alive and disease-free, although one patient progressed to stage 4 disease despite early excision of the primary tumor. CONCLUSIONS: Spontaneous regression of localized perinatal adrenal neuroblastoma occurs often, and a "wait and see" strategy seems justified in these small infants. Patients with enlarging or stable lesions that have persisted for several months may benefit from surgery, although prompt excision may not prevent tumor progression.

Prenatal diagnosis of duodenal obstruction selects cases with a higher risk of maternal-foetal complications and demands in utero transfer to a tertiary centre.

OBJECTIVES: It was the aim of this study to determine if prenatal diagnosis of congenital duodenal obstruction (CDO) selects high-risk pregnancies and demands special perinatal attention. METHODS: Medical records of 62 neonates with intrinsic CDO, admitted since 1981 in 2 institutions, were reviewed and divided into 2 groups: 39 cases, detected in utero by a prenatal ultrasonogram (group A), and 23 diagnosed at birth (group B). Prevalence of complete CDO, mean gestational age, mean birth weight, hydramnios, as well as maternal-foetal complications requiring emergency Caesarean section and associated with premature delivery were compared in the 2 groups. RESULTS: A complete CDO was found in 77% of patients in group A versus 48% of patients in group B (p < 0.02). Differences in mean gestational age and mean birth weight in the 2 groups were non-significant. Prenatally diagnosed patients presented a higher prevalence of hydramnios, maternal-foetal complications and premature delivery. CONCLUSIONS: Prenatal diagnosis selects patients with complete CDO and hydramnios. These pregnancies present a high incidence of maternal-foetal complications, which may require an emergency Caesarean section, and are frequently associated with premature delivery. These aspects must be considered in prenatal counselling. In utero transfer to a tertiary centre for delivery and appropriate perinatal care should be recommended.

Nephron-sparing surgery in children with primary renal tumor: indications and results.

Nephron-sparing surgery (NSS) is the treatment of choice for children with bilateral Wilms' tumor (WT), or with WT on a single kidney, or with WT and a disease of the contralateral kidney, or with benign kidney tumor. NSS is a reasonable alternative to nephrectomy in children at risk of metachronous WT, including children with genetic syndromes, children younger than 1 year of age, and children with hyperplastic nephroblastomatosis. The use of NSS in selected children with "low-risk" or stage I "intermediate-risk" WT and a normal contralateral kidney is still controversial. Available data suggest that, in children with WT, NSS does not impair the outcome and has a renal function advantage over nephrectomy.

Adenomyomatosis of the gallbladder in childhood: report of a case and review of the literature.

Adenomyomatosis of the gallbladder is considered an acquired pathologic condition that affects adults, with only 2 cases previously described in childhood. We report on a 5-year-old boy with incidental detection of this lesion by ultrasonography. Pathologic and clinical assessments are discussed in addition to treatment decision-making, including current insights into the relation between adenomyomatosis and malignancy of the gallbladder. The liberal use of ultrasonography in children with abdominal pain may result in detection of an increasing number of asymptomatic patients. Caregivers should be aware of this condition and its therapeutic implications in the pediatric population.