Comparison of Outcomes between Prophylactic and Rescue Therapy of Surfactant in Premature Infants

PURPOSE: To compare early and later surfactant instillation in neonates with a birth weight of <1,250 g and/or less than 30 weeks' gestation, following the changes in the National Health Insurance policy of the Republic of Korea. METHODS: Preterm infants diagnosed with respiratory distress syndrome and instilled with an exogenous surfactant from April 2006 to August 2012 were included in this study. The subjects were divided into the two groups: the prophylactic group (n=19) included neonates who were instilled with surfactant within 30 minutes after birth in the delivery or operating room, and the rescue group (n=27) included neonates who were treated with surfactant from 30 minutes to 10 hours after birth for the treatment of respiratory distress syndrome. We compared the two groups in terms of short- and long-term outcomes. RESULTS: The groups showed no significant difference in gestational age and birth weight. The prophylactic group had a shorter duration of mechanical ventilation of synchronized intermittent mandatory ventilation but longer parenteral nutrition and mechanical ventilation, including continuous positive airway pressure without synchronized intermittent mandatory ventilation. There are significant differences in the occurrence of long-term common complications such as patent ductus arteriosus and parenteral nutrition-associated cholestasis. The ventilation index, oxygenation index, mean airway pressure, and arterial-to-alveolar oxygen pressure ratio were lower in the prophylactic group than in the rescue group. CONCLUSION: In comparison with late instillation, early surfactant instillation can reduce the period and requirement of mechanical ventilation. It also reduces the occurrence of patent ductus arteriosus and parenteral nutrition-associated cholestasis in newborns.

Efficacy of Erythromycin and Metoclopramide in Neonates with Feeding Intolerance

PURPOSE: Feeding intolerance is common in premature infants. It may extend the parenteral nutrition period and increase the risk of complications. We evaluated the efficacy of erythromycin and metoclopramide in neonates with feeding intolerance. METHODS: Between December 2006 to August 2011, 114 neonates with feeding intolerance were divided into two groups treated with either erythromycin or metoclopramide in the neonatal intensive care unit of Chung-ang University Hospital, a tertiary care center. We defined neonates with feeding intolerance as those who either could not be fully fed enterally (120 mL/kg/day) within 7 days or who skipped feeding more than twice per day because the gastric residual volume was >20% of each feed or more than 50% once. The time taken to achieve 50%, 75%, and 100% enteral feeding was estimated retrospectively. RESULTS: The erythromycin group achieved 50% feeding (P=0.047), 75% feeding (P=0.042), and 100% feeding (P=0.039) earlier than the metoclopramide group. The erythromycin group achieved 100% feeding earlier than the metoclopramide group among infants with birth weight > or =1,500 g (P=0.036) and those with gestational age > or =34 weeks (P=0.008). CONCLUSION: Compared with metoclopramide, erythromycin improves feeding in neonates with feeding intolerance, especially in infants with birth weight > or =1,500 g and in those with gestational age > or =34 weeks.

Congenital Chloride Diarrhea in Dizygotic Twins / 대한소아소화기영양학회지

Congenital chloride diarrhea (CLD) is a rare inherited autosomal recessive disorder. Mutations of the solute carrier family 26 member 3 gene cause profuse, chloride ion rich diarrhea, which results in hypochloremia, hyponatremia and metabolic alkalosis with dehydration. If a fetal ultrasound shows bowel dilatation suggestive of bowel obstruction, or if a neonate shows persistent diarrhea and metabolic alkalosis, CLD should be considered in the differential diagnosis. The severity of CLD varies, but early detection and early therapy can prevent complications including growth failure. We report a case of dizygotic twins affected by CLD who had been born to non-consanguineous parents. Both of them showed growth failure, but one of the twins experienced worse clinical course. He showed developmental delay, along with dehydration and severe electrolyte imbalance. He was diagnosed with CLD first at 6-month age, and then the other one was also diagnosed with CLD.

Congenital Epidermoid Cyst of the Oral Cavity: Prenatal Diagnosis by Sonography

Epidermoid cysts are benign developmental anomalies that are rarely observed in the oral cavity of neonate. If large in size, especially in the developing fetus or newborn infant, they can cause swallowing difficulty and occasionally respiratory difficulty. We report a case of epidermoid cyst in the oral cavity detected prenatal sonography. The sonographic finding was large cystic mass, measuring 30x25 mm. In this case, supplies and equipment for an emergency tracheostomy were made available prior to the delivery. However, the infant did not require intervention to secure the airway. The lesion was surgically excised, and histologic diagnosis was epidermoid cyst. After 6 months of follow up, the cyst had not recurred. This case illustrates the value of accurate prenatal diagnosis and planned perinatal management using a team approach.

Etiology of Pediatric Healthcare-associated Infections in a Single Center (2007-2011) / 병원감염관리

BACKGROUND: Healthcare-associated infections (HAIs) are among the most important threats to patient safety. When hospitalized children face these threats, there is morbidity, mortality, prolonged hospitalization, and increased healthcare costs. Research on local healthcare epidemiology is necessary to enhance collective knowledge and evidence formanaging this problem. METHODS: We performed a retrospective analysis of databases of patients who were diagnosed with HAIs at Chung-Ang University Hospital (CAUH) from 2007 through 2011. Cases were selected from the microbiology registry databases. The data on prevalence of HAIs in various wards and its annual trends were compared to previously reported nationwide data. Moreover, we analyzed the patterns of antibiotic susceptibility results for HAI pathogens. RESULTS: A total of 181 HAIs were identified in 122 patients. The HAI rate among pediatric patients at CAUH was 2.4/1,000 person-hospital days. Urinary tract infections (UTIs) (53 episodes, 29.3%) were the most common, followed by pneumonia (33 episodes, 18.2%). Staphylococcus aureus was found to be the most common gram-positive organism, whereas Escherichia coli was the most common gram-negative organism. Methicillin-resistant S. aureus (MRSA) comprised 84% of the S. aureus infections. Imipenem resistance was detected in 58.8% and 55.0% of Acinetobacter baumannii and Pseudomonas aeruginosa isolates, respectively. CONCLUSION: Between 2007 and 2011, UTIs were the most common type of HAIs, and MRSA was the most common pediatric HAI pathogen, both in the general ward and intensive care unit at the CAUH. Further research on the epidemiology and pathogenesis of HAIs is necessary and prevention measures should be implemented to prevent HAIs in children.

Early congenital syphilis presenting with skin eruption alone: a case report / 소아과

Congenital syphilis is one of the most well-known congenital infections, yet it remains a worldwide public health problem. Congenital syphilis can involve any organ system and present with various symptoms. However, early diagnosis of congenital syphilis is difficult because more than half of the affected infants are asymptomatic, and the signs in symptomatic infants may be subtle and nonspecific. Here, we report a case of congenital syphilis with only a skin rash, which led to a delay in diagnosis. This case indicates that congenital syphilis should be considered throughout early childhood.

The First Case of X-linked Alpha-thalassemia/Mental Retardation (ATR-X) Syndrome in Korea

Mutation of the ATRX gene leads to X-linked alpha-thalassemia/mental retardation (ATR-X) syndrome and several other X-linked mental retardation syndromes. We report the first case of ATR-X syndrome documented here in Korea. A 32-month-old boy came in with irritability and fever. He showed dysmorphic features, mental retardation and epilepsy, so ATR-X syndrome was considered. Hemoglobin H inclusions in red blood cells supported the diagnosis and genetic studies confirmed it. Mutation analysis for our patient showed a point mutation of thymine to cytosine on the 9th exon in the ATRX gene, indicating that Trp(C), the 220th amino acid, was replaced by Ser(R). Furthermore, we investigated the same mutation in family members, and his mother and two sisters were found to be carriers.

Influence of histologic chorioamnionitis and funisitis on the level of peripheral blood C-reactive protein at birth in preterm infants / 소아과

PURPOSE: The objective of this study is to determine the change of C-reactive protein (CRP) levels in the peripheral blood of preterm infants at birth according to the stage of intrauterine inflammation. METHODS: A total of 187 infants (<32 weeks of gestation) were divided into a "no histologic chorioamnionitis" [HCAM (-), n=85] group and a "histologic chorioamnionitis" [HCAM (+), n=102] group according to placental pathologic findings. Furthermore, the HCAM (+) group was subdivided into a "funisitis" [F (+), n=49] group and a "no funisitis" [F (-), n=53] group and also into a "funisitis/amnionitis" [FA (+), n=58] group and an "isolated chorio-deciduitis" [FA (-), n=44] group. High-sensitivity CRP levels in the peripheral blood at birth were measured. RESULTS: Peripheral blood CRP levels were significantly higher in the HCAM (+), F (+), F (-), and FA (+) groups than in the HCAM (-) group, but were not significantly different between the FA (-) and HCAM (-) groups. In addition, peripheral blood CRP levels were significantly higher in the F (+) and FA (+) groups than in the F (-) and FA (-) groups, respectively. For identification of amnionitis or funisitis, a cut-off value of 0.02 mg/dL was chosen. Clinical chorioamnionitis, proven early onset sepsis, histologic chorioamnionitis, and funisitis had higher incidences in infants with peripheral blood CRP levels higher than 0.02 mg/dL. CONCLUSION: The present study shows that peripheral blood CRP levels at birth in preterm infants born before 32 weeks' gestation is significantly increased in amnionitis or funisitis and might reflect the progress of histologic chorioamnionitis.

Clinical significance of matrix metalloproteinase 9 and tissue inhibitor of metalloproteinase 1 and 2 in Kawasaki disease / 소아과

PURPOSE: Kawasaki disease (KD) is a systemic vasculitis, a leading cause of pediatric acquired heart disease. Histopathological findings of coronary artery lesion (CAL) in KD indicate destruction of the coronary artery wall with diffuse vasculitis. Matrix metalloproteinases (MMPs) and their endogenous tissue inhibitors (TIMPs) might play central roles in this process. Special attention to MMP-9 has recently been emerging. This study was performed to investigate the clinical significance of MMP-9 and its inhibitors, TIMP-1 and TIMP-2, in KD. METHODS: We compared 47 KD patients with 14 febrile controls. Serum MMP-9 and TIMP-1, TIMP-2 were measured by ELISA and compared according to clinical stages and coronary involvement. RESULTS: In acute stage, MMP-9 and TIMP-1 were significantly higher, whereas TIMP-2 was lower, in KD than those in febrile controls (p<0.05). The elevated MMP-9 levels in acute phase significantly decreased during the subacute and convalescent phases (p<0.05). During acute phase, the MMP-9, TIMP-1, and MMP-9/TIMP-2 levels in the CAL group were lower than those in the non-CAL group, but they increased significantly in the subacute phase (p<0.05). MMP-9 has a positive correlation with TIMP-1 in the acute and subacute phases, and negative correlation with TIMP-2 in the subacute and convalescent phases (p<0.05). CONCLUSION: These results suggest that MMP-9, TIMP-1, and the imbalance in MMP-9 and TIMP-2 might play important roles on the pathophysiology of KD and especially on the development of CAL. However, further larger studies are needed.

Clinical Analysis of the Pediatric Patients with Neurological Disorders Seen in the Emergency Department / 대한소아신경학회지

PURPOSE: Pediatric emergencies with seizure and decreased mental status or loss of consciouseness may cause severe complications without early proper management. Distribution of neurological disorders may differ according to various demographics, it would be valuable to evaluate recent data in Korea. METHODS: Patients who had neurological problems and below 18 years old were defined as case. 1,058(5.6%) cases who visited the emergency department of Chung-Ang University Hospital from January 2005 to December 2008 were analyzed. The patients were also divided into four age groups: age 1 year or younger, preschool children(2-5 years), middle childhood group(6-10 years), and adolescent group(11-18 years). RESULTS: The male to female ratio was 1.5:1. Most children were between 2 to 5 years old(mean age=3 years). Febrile convulsion is the most common neurological disorders among infant and preschooler, meningitis is the most common among middle childhood, epilepsy and headache is the most common among adolescent. Main initial presentations were seizure followed by fever, headache and vomiting. Febrile convulsion was the most common neurological disorders followed by epilepsy and meningitis. Final dispositions of patients were admission, discharge, against medical device, and transfer to other hospital. Admission rate of patients with neurological disorders was higher than those of total patients. CONCLUSION: Neurological disorders are increasing in pediatric emergencies and have highest admission rate, mostly because of the parent's anxiety and severity of disease itself. Therefore, not only detect and treat neurological disorder in early stage, but also reassuring parent is important.

Epidemiologic and clinical features in children with acute lower respiratory tract infection caused by human metapneumovirus in 2006-2007 / 소아과

PURPOSE: The causes of acute lower respiratory tract infection (ALRTI) are mostly attributable to viral infection, including respiratory syncytial virus (RSV), parainfluenza virus (PIV), influenza virus A/ B (IFV A/ B), or adenovirus (ADV). Several Korean studies reported human metapneumovirus (hMPV) as a common pathogen of ALRTI. However, studies on seasonal distribution and clinical differences relative to other viruses are insufficient, prompting us to perform this study. METHODS: From November 2006 to October 2007, we tested nasopharyngeal aspiration specimens in children hospitalized with ALRTI with the multiplex reverse transcriptase-polymerase chain reaction to identify 6 kinds of common pathogen (hMPV, RSV, PIV, IFV A/ B, and ADV). We analyzed positive rates and clinical features by retrospective chart review. RESULTS: We detected 38 (8.4%) hMPV-positive cases out of 193 (41.8%) virus-positive specimens among 462 patients. HMPV infection prevailed from March to June with incidence peaking in April. HMPV-positive patients were aged 1-5 years (76.3%), and the ratio of boys to girls was 1.2:1. The median age was 27 months. HMPV primarily caused pneumonia (76.3%) (P=0.018). Average hospitalization of HMPV-associated ALRTI patients was 5.8 days. In addition, they showed parahilar peribronchial infiltration (100%) on chest X-ray, normal white blood cell count (73.7%), and negative C-reactive protein (86.8%) (P>0.05). All hMPV-positive patients recovered without complication. CONCLUSION: HMPV is a common pathogen of ALRTI in Korean children, especially in 1-5 year olds, from March to May. Immunocompetent children diagnosed with hMPV-associated ALRTI may have a good prognosis.

A case of epignathus tumor causing obstruction of upper airway immediately after birth / 대한주산의학회잡지

Teratoma originate from one or more germ cell layer and commonly arise from sacrococcygeal area in neonate. Teratoma arising from the oropharyngeal cavity is called "epignathus tumor" and is extremely rare in neonate. Clinical manifestation of epignathus tumor vary from asymptomatic to severe respiratory distress symptom. It is reported that most of the tumor are benign in nature. Large teratoma can be diagnosed by prenatal ultrasonography, but most cases were diagnosed with computed tomography or magnetic resonance image after birth. Prognosis is determined by the need for neonatal resuscitation for respiratory distress at the time of birth and the extent of tumor, involving large vessles, skull base or communication with the brain. We experienced a case of epignathus tumor in a neonate with severe respiratory distress immediately after birth, so that reported with review of the literature.

A case of herpes zoster in a 4-month-old infant / 소아과

Herpes zoster in infancy is very rare but can be developed following intrauterine or postnatal exposure to varicella zoster virus. We report a case of herpes zoster in a 4-month-old male infant. He had no prior history of primary varicella or varicella vaccination. His mother had no history of varicella infection and no contact history with varicella during pregnancy. He had a history of exposure to his father with herpes zoster 3 months ago, and to his cousin with convalescent chickenpox 2 months ago. Multinucleated, giant cells were shown on a Tzanck smear. He was treated with acyclovir and first generation cephalosporin for herpes zoster with Staphylococcal skin infection, with complete resolution without sequelae.

A case of premature born with bowel perforation and gangrene due to intrauterine midgut volvulus caused by meconium ileus / 대한주산의학회잡지

Midgut volvulus is commonly complicated with malrotation, and develops mainly in infants before 1 year old, especially in neonate. Intrauterine midgut volvulus is an extremely rare disease therefore is difficult to diagnose. Furthermore unless the fetus has malrotation, symptoms and results of tests suspicious of fetal midgut volvulus are nonspecific. There are some reports that meconium ileus could be a cause of intrauterine midgut volvulus from foreign countries, however has never been reported in Korea. So we report a case of prematurity born with bowel perforation and gangrene due to intrauterine midgut volvulus caused by meconium ileus.

A Case of Typical Kawasaki Disease with Vulvar Erythema and Edema in 21 Days Old Neonate

Kawasaki diseases occur frequently in children beyond the age of 4 years, but classic Kawasaki disease in newborns are rare and have not been reported in Korea yet. We report a case of classic Kawasaki disease in neonate who is 21 days old girl and has no response to gamma-globulin although early diagnosis and treatment. Her clinical features met typical Kawasaki disease, but unusually she had a non suppurative labia major erythema and severe edema instead of cervical lymphadenitis. She had persistent fever and progressive carditis with coronary dilatation in spite of 3 doses of gamma globulin. Her fever was subsided after 3 doses of intravenous methyl prednisolone. In the subacute stage her other symptoms subsided and appeared typical desquamation of fingers and toe. Finally she had mild mitral regurgitation and diffuse coronary dilatation when she was discharged. Since one year follow up, with continuous aspirin medication, now she is very healthy, and her coronary dilatation was normalized and not any more valvular regurgitation.

A Case of Teratocarcinoma with Central Diabetes Insipidus / 소아과

Central diabetes insipidus is a rare disorder that can result as a consequence of diverse etiologies, including malformations, autoimmune, infiltrative(e.g. neoplastic or histiocytosis) or traumatic processes, as well as mutations in the gene encoding arginine vasopressin. Idiopathic central diabetes insipidus is a diagnosis of exclusion, one that has been made less frequently through the decades. Idiopathic central diabetes insipidus in children and adolescent requires a frequent follow-up regimen using serial brain MRI and CSF examinations especially if an isolated pituitary stalk thickening or loss of a hyperintense signal in the posterior lobe is observed. Also, so-called "idiopathic" central diabetes insipidus warrants close follow-up to determine the etiology, especially if anterior pituitary hormone deficiencies are detected. We report a case of idiopathic central diabetes insipidus with growth hormone deficiency and loss of a hyperintense signal in the posterior lobe of pituitary in the brain MRI. We followed up with serial contrast enhanced brain MRI and CSF evaluation for the early detection of an evolving occult hypothalamic-stalk lesion and finally detected a newly developed teratocarcinoma in the suprasellar region.

A Case of Maple Syrup Urine Disease detected by Tandem Mass Spectrometry in Newborn Screening Test

Maple syrup urine disease (MSUD) is an autosomal recessive disorder. Impaired activity of the branched-chain 2-oxo acid dehydrogenase complex causes accumulation of branched-chain L-amino and 2-oxo acid that can exert neurotoxic effects. MSUD presents heterogeneous clinical and molecular phenotypes. Severity of the disease, ranging from classical to mild variant types, is commonly classified on the basis of indirect parameters, e.g. onset, leucine tolerance and/or residual enzyme activity in cell. Since early 1990's, tandem mass spectrometry has been applied to newborn screening, because it is amenable to population-wide testing for a large number of disorders of fatty acid, organic acid, and amino acid metabolism. And so, we report a case of MSUD in 15 days old boy detected by newborn screening using tandem mass spectrometry.

Effect of High Temperature on Electrical Seizure-induced Neuronal Damage in Explant Culture of Rat Hippocampus / 대한소아신경학회지

PURPOSE: Epileptic patients have a increasing tendency to develop seizure attack in high temperature. This finding suggests that high temperature may have an effect on neuronal hyperexcitability and injury of epileptic brain. Therefore, the influence of high temperature on normal and epileptic brain was studied in organotypic explant cultures of rat. METHODS: Fourteen days-in-vitro cultures from 8 day-old rat pups were perfused with standard aCSF bubbled with 95%/5% O2/CO2 in a microchamber. Stimulus train(0.3 sec, 60 Hz) was applied to Schaffer collaterals in CA3 and extracellular field potential was recorded in the CA1 pyramidal layer. At 36degrees C initially, AD was evoked. In high temperature(HT) group, the cultures were subjected to 39degrees C for a period of 8 min before the second stimulus train was applied. They were then restored to 36degrees C for 10 min. In normal temperature group, temperature was maintained at 36degrees C for the second stimulus train. The cultures were returned to the incubator and observed serially for neuronal damage. Intensity of propidium iodide fluorescence indicative of neuronal injury was quantitated by digital image analysis. The cultures on the same insert that were not stimulated served as the unstimulated groups. RESULTS: There was not a statistically significant difference in neuronal damage between the unstimulated high-temperature(HT) and normal-temperature(NT) group. In CA1 sector, % damage(mean+/-SEM) was 0.42+/-0.20 vs 0.27+/-0.05 at 24 hrs(HT vs NT group, n=16 each, P>0.05, Student t-test); 1.81+/-0.79 vs 1.43+/-0.27 at 48 hrs(P>0.05); 3.50+/-1.32 vs 3.35+/-0.56 at 72 hrs(P>0.05). In CA3 sector, % damage was 0.34+/-0.10 vs 0.20+/-0.03 at 24 hrs(P>0.05); 0.99+/-0.20 vs 0.83+/-0.23 at 48 hrs(P>0.05); 2.00+/-0.38% vs 2.26+/-0.35% at 72 hrs(P>0.05). Neuronal damage on AD induced cultures during febrile setting(n=16) was significantly higher than in nonfebrile setting(n=16). In CA1 sector, % damage was 6.63+/-2.56 vs 0.92+/-0.45 at 24 hrs(febrile setting vs nonfebrile setting, P= 0.036); 26.37+/-7.44 vs 4.99+/-2.23 at 48 hrs(P=0.010); 38.59+/-9.63 vs 6.48+/-2.30 at 72 hrs (P=0.003). In CA3 sector, % damage was 1.23+/-0.48 vs 3.91+/-2.37 at 24 hrs(P=0.277); 13.09+/-5.75 vs 5.93+/-3.27 at 48 hrs(P=0.288); 27.86+/-8.68 vs 7.54+/-3.74 at 72 hrs(P=0.04). CONCLUSION: At high temperature, seizures in epileptic brain may be more injurious than seizures in normal temperature.

Detection of Virulence Genes in Uropathogenic Escherichia coli by Multiplex Polymerase Chain Reaction(PCR) : Comparison with Clinical Characteristics / 대한신장학회잡지

BACKGROUND: We identified virulence genes in uropathogenic E. coli isolates and studied the association between virulence gene and clinical characteristics in order to predict the severity and recurrency. METHODS: 39 Escherichia coli strains from patients with urinary tract infection were clinically and genotypically characterized. The strains were examined genotypically by using the multiplex polymerase chain reaction for presence of 5 urovirulence genes : pyelonephritis-associated pili(pap), S. fimbriae(sfa), afimbrial adhesin(afa), cytotoxic necrotizing factor (cnf), and alpha-hemolysin(hly). The patient's clinical characteristics were determined retrospectively. RESULTS: 17 pap(+), 4 sfa(+), 7 afa(+), 6 cnf(+), and 8 hly(+) strains were identified. And there were 10 genotypes. Among them, genotype pap(+)sfa(-)afa(-)cnf(-) hly(-) was most dominant(36%). But no urovirulence gene was detected in 12 strains(31%). When the data was analyzed, it was apparent that an association among various urovirulence genes exists. sfa gene was frequently associated with cnf gene(p < 0.001). And afa gene was associated cnf and hly gene(p= 0.026, <0.001). An association between cnf gene and hly gene was observed(p=0.002). Positive rates of virulence genes were not different between male and female. In infancy, pap(-)sfa(-)afa(+)cnf(+)hly(+) genotype was dominant. In 2-15 years old age group, pap(-) sfa(-)afa(-)cnf(-)hly(-) genotype was dominant. And in 16- 40 years old age group, pap(+)sfa(-)afa(-)cnf(-)hly(-) was dominant. So, some virulence genotype might be associated with specific age group. Presence of virulence gene or specific genotype was not different among diseases(acute pyelonephritis, cystitis, asymptomatic bacteriuria). So, virulence genes were not associated with severity of urinary tract infection. Virulence genes were not associated with susceptibility of recurrent infection. In neurogenic bladder patients, there were significantly more sfa(+) strains (p=0.019). And all isolates of neurogenic bladder patients were genotype pap(+)sfa(+)afa(-)cnf(+)hly(-)(p < 0.001). CONCLUSION: In this study, We found which genotype is most dominant in uropathogenic Escherichia coli, and that virulence genes do not suggest severity or recurrency of urinary tract infection. In neurogenic bladder patients, some virulence genes were more prevalent.

A Case of Primitive Neuroectodermal Tumor of the Adrenal Gland

Primitive neuroectodermal tumors(PNETs) are a group of small round cell tumors that may arise in the central or peripheral nervous system. In the extracranial location, these neoplasms may occur anywhere and at any age group, but most likely in the bone and soft tissue of children and young adults. The incidence of adrenal gland involvement is very rare. This tumor is diagnosed by histopathological, immunohistochemical, and molecular pathological examinations. PNETs are highly aggressive tumors, locally recurrent and rapidly metastasizing to lung, liver, lymph node, and brain. The outcome is poor with a 5 year survival rate below 40% in spite of aggressive combined treatment including surgery, chemotherapy, and radiation therapy. We experienced a case of PNET of adrenal gland, mimicking ruptured Wilms tumor on the US and CT scan.