Neonatal neuroblastoma (NBL) is the most common malignant tumor in neonates, but there have been few studies about it. The purpose of this study was to investigate the clinical features of NBL and to compare prenatal and postnatal diagnosed groups.
Nineteen patients who were diagnosed with NBL prenatally or within 28 days after birth from February 1986 to February 2013 in Seoul National University Hospital were enrolled in the study. The patients were categorized according to the International Neuroblastoma Staging System (INSS) and Children's Oncology Group (COG). Retrospective medical-record reviews were performed on these patients. The operative date, complication, pathological stage, and overall survival of the prenatally diagnosed group and the postpartum diagnosed group were compared.
Tumor was detected via prenatal ultrasonography in 8 patients (42.1%), and 11 patients (57.9%) were diagnosed within 28 days after birth. Based on INSS, the patients were divided into the stage I (n=8), stage II (n=1), stage III (n=3), stage IV (n=4), and stage IVs (n=3) groups, respectively. Based on COG, on the other hand, the patients were divided into the low-risk (n=8), intermediate-risk (n=8), and high-risk (n=3) groups. The postoperative complication rate was 29%. One patient died from complications from chemotherapy. The other 18 patients' mean follow-up period was 77.7 months. The differences between the postoperative complication rate, proportion of early-stage tumor, and overall survival of the prenatal and postnatal groups were not statistically significant (p=0.446, p=0.607, p=0.414).
NBL showed favorable outcomes but relatively higher postoperative complications. There seem to be no significant statistical differences in the postoperative complications, proportion of early-stage tumor, and overall survival between the prenatally diagnosed group and the postpartum diagnosed group.
With the widespread use of the obstetrical ultrasound, identification of a fetal suprarenal mass becomes more common. Most of these masses prove to be congenital neuroblastomas (CNB) postnatally. However, the diagnosis is often confused with other benign lesions and the post-natal management remains controversial. The medical records of 13 patients that underwent primary surgical excision for an antenatally detected adrenal CNB, between January 1995 and April 2009, were reviewed retrospectively. The clinical, radiological, surgical, and pathological data on the suprarenal mass were collected. Staging evaluation was performed after histological confirmation of the CNB. Most of the CNBs were stage I (N=11), with 1 stage IV and 1 stage IV-S. Four patients (3 stage I and 1 stage IV-S) had N-myc gene amplification. The stage I patients were cured by surgery alone, and stage IV patients underwent 9 cycles of adjuvant chemotherapy and currently have no evidence of disease after 39 months of follow-up. The patient with stage IV-S is currently receiving chemotherapy. There were no post-operative complications. For early diagnosis and treatment, surgical excision should be considered as the primary therapy for an adrenal CNB detected before birth. The surgery can be safely performed during the neonatal period and provides a cure in most cases. Surgical diagnosis and treatment of CNB is recommended in neonatal period.
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As prenatal ultrasonography becomes popular, the number of prenatal diagnosis of congenital surgical diseases is also increasing. To evaluate the impact of antenatal ultrasonography on outcome the mortality rate in neonatal surgical emergencies was studied. The authors retrospectively reviewed 281 patients (congenital diaphragmatic hernia: 44, tracheoesophageal fistula: 78, intestinal atresia: 98, omphalocele: 28 and gastroschisis: 33 who had been managed at Seoul National University Childrens Hospital, from January 1991 to December 2000. The patients were divided into two groups; group A (1991 to 1995; 139 patients) and group B (1996 to 2000; 142 patients). These two groups were subdivided into prenatally diagnosed subgroup and postnatally diagnosed subgroup. We analyzed the changes of prenatal diagnosis rate, total mortality rate, and mortality rate of subgroups. Prenatal diagnosis rate was increased significantly in group B (Group A: 24.5 % and Group B: 45.1 %). Total mortality rate of group A was 21.6 %, and that of group B was 10.6 %, showing a significant decrease in group B. However, in both group A and B, when compared antenatally diagnosed subgroup with postnatally diagnosed subgroup, the mortality rate was lower in postnatally diagnosed subgroups but statistically not significant. The authors conclude that although prenatal diagnosis rate has been increased, prenatal diagnosis itself has not resulted in significant improvement in outcome.
Improvement in prenatal ultrasonography is leading to diagnose choledochal cyst before birth and before onset of classical symptom more frequently. But, there is a controversy about optimal timing for cyst excision of prenatally diagnosed asymptomatic choledochal cyst. To identify the most appropriate timing for surgery in prenatally diagnosed choledochal cysts, we analyzed 6 patients who had operation for choledochal cysts within 30days after birth at the division of Pediatric Surgery, Samsung Medical Center and Inha University School of Medicine, from June 1995 to June 2002. Males were four and females 2, the mean age at operation was 11.2 days, and the median age 8.0 days. The range of gestational ages of the antenatal diagnosis of bile duct dilatation was 24 weeks to 32 weeks, mean was 38.3 weeks, and mean birth weight was 3,298.3 g. After birth, abdominal ultrasonography, hepatobiliary scintigraphy, and magnetic resonance cholangiopancratography (MRCP) were performed. Mean age at operation was 11.2 days. All patients had the cyst excision and Roux- en-Y hepaticojejunostomy. Immediate postoperative complication was not found. During the median follow-up period of 41 months, one patient was admitted due to cholangitis, and the other due to variceal bleeding. Early operative treatment of asymptomatic newborn is safe and effective to prevent developing complications later in life.
Prenatally diagnosed neuroblastomas have been reported in increasing numbers over the past several years. The vast majority are in favorable stages of the disease (stage I, II, IV-S). The authors experienced one case of stage IV-S neuroblastoma of the adrenal gland with liver metastasis, which regressed spontaneously after removal by adrenalectomy. This patient was noticed to have an abdominal mass at prenatal ultrasonography performed at 36weeks of gestation. This tumor was a neuroblastoma of the left adrenal gland with multiple liver metastases. Left adrenalectomy and liver biopsy were performed at 3 months of age. Thirty-eight months after surgery, an MRI demonstrated that the hepatic metastatic lesions had completely regressed without chemotherapy or radiation.
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