[Acute shock secondary to congenital neuroblastoma: Neonatal case report]. / Shock agudo secundario a un neuroblastoma congénito: Caso clmico neonatal.

Congenital neuroblastoma is the most frequent malignant solid tumor in the neonatal period. The clinical presentation is usually either by prenatal diagnosis or by palpation of an abdominal mass. Staging allows classifying it according to risk groups with a different prognosis and treatment. In the neonatal period, it is characterized by a high rate of spontaneous regression and good prognosis (disease-free survival at 5 years greater than 90 %). We present a clinical case of congenital neuroblastoma whose presentation, shock and hypertension, was only described on a previous occasion. Antihypertensive treatment along with systemic chemotherapy produced clinical control and patient improvement.

El neuroblastoma congénito es el tumor sólido maligno más frecuente en el período neonatal. La forma de presentación suele ser por diagnóstico prenatal o por una masa abdominal. Su estadificación permite clasificarlo en grupos de riesgo con pronóstico y tratamiento diferentes. En el período neonatal, se caracteriza por la alta tasa de regresión espontánea y el buen pronóstico (supervivencia libre de enfermedad a los 5 años superior al 90 %). Se presenta un caso clínico de neuroblastoma congénito cuya forma de presentación, shock e hipertensión, solo estaba descrita en otra ocasión antes. El tratamiento antihipertensivo, junto con la quimioterapia sistémica, produjo el control clínico y la mejoría del paciente.

Tumor maligno congénito. Síndrome de Pepper / Congenital malignant tumor: Pepper's Syndrome

Pepper's syndrome refers to a neuroblastoma originated in the adrenal glands that usually metastasizes to the liver with abdominal development and respiratory involvement because of thoracic compression. The metastasic tumors are usually infrequent with an unfavorable prognosis. The cases reported in the world literature are very few. The congenital form of neuroblastoma is uncommon. The aim of this report was to describe a typical clinical case of a new born who died because of a metastasic malignant tumor, comptible with a Pepper's syndrome

Causa infrecuente de hipertensión arterial en lactantes: neuroblastoma congénito quístico suprarrenal: caso clínico / A rare cause of hypertension in childhood: congenital cystic adrenal neuroblastoma: clinical case

The frequency of hypertension (HBP) in children has increased significantly over the past decade. The younger the patient the greater the likelihood of having secondary HBP. Thus, the main causes of hypertension in new-borns are of renovascular or parenchymatous origin. objective: To present the case of an infant with hypertension caused by a congenital cystic neuroblastoma (NB). Case History: Newborn with prenatal diagnosis of adrenal cyst, who evolved with significant hypertension unresponsive to medical therapy. Neuroblastoma was suspected on the basis of magnetic resonance imaging findings and resection of the lesion was able to resolve the hypertension and to confirm the diagnosis by anatomo-pathological study. Conclusion: Most cases of neonatal hypertension are of renal origin, with the 2 largest categories being renovascular and renal parenchymal diseases. NB is the most common neonatal malignancy. It usually presents as an abdominal mass of antenatal diagnosis, being the hypertension an unusual form of presentation.

La frecuencia de hipertensión arterial (HTA) en niños ha aumentado significativamente en la última década. A menor edad del paciente mayor es la probabilidad de que la HTA sea secundaria. Así, las principales causas de HTA en recién nacidos son de origen renovascular o parenquimatoso. objetivo: Presentar el caso de un lactante hipertenso por neuroblastoma (NB) congénito quístico. Caso Clínico: Recién nacido con diagnóstico prenatal de quiste suprarrenal, quien evolucionó con HTA por sobre el percentil 99 para edad, sexo y talla, sin respuesta a terapia farmacológica. La resonancia magnética permitió realizar el diagnóstico presuntivo de neuroblastoma congénito y la resección de la lesión permitió resolver la HTA y confirmar el diagnóstico. Conclusión: La HTA en recién nacidos generalmente se debe a causas secundarias. El NB es el tumor maligno neonatal más frecuente que se puede presentar como una masa abdominal de diagnóstico antenatal, siendo la HTA una forma infrecuente de presentación.

Neuroblastoma congênito como achado incidental em nécropsias rotineiras de fetos e recém-nascidos - Ocorrência e aspectos anatomopatológicos

Objetivos: Avaliação da frequência e dos aspectos anatomopatólogicos do neuroblastoma congênito observados em necrópsias de rotinas de fetos e recém-nascidos. Métodos: Foram pesquisadas 1750 necrópsias de rotineiras de fetos e recém-nascidos do Serviço de Anatomia Patológica da Casa Maternal e da Infância "Leonor Mendes de Barros", da então Legião Brasileira de Assistência, São Paulo. Foram realizados em um período de 20 anos, de 1953 a 1973, com exame histólogico dos principais órgãos. Resultados: Identificamos nove casos de neuroblastoma congênitos, com frequência aproximada de 1/94 autópsias: três casos evidentes à macroscopia: um volumoso, primário na região pélvica, com metástase no fígado, pâncreas, linfonodos peripancreáticos e supra-renais; o outro no mediano superior , paravertebral, com 4,5 cm sem disseminação e um terceiro em supra renal, com 2,5 cm de diâmetro, pseudocístico devido a intensa necrose e hemorragia, considerado em regressão. Em relação aos outros seis casos, todos limitados às supra-renais um deles, caso Nº 2, foi percebido após o corte da glândula já fixada em formol, com 1 cm de diâmetro; os outros casos (3,4,5,6 e 9) foram achados histológicos de rotina

[Costello syndrome associated to a neuroblastoma. Presentation of a case]. / Síndrome de Costello asociado a neuroblastoma. Presentación de un caso.

We present the case of a newborn with Costello syndrome who died due to heart arrhythmia. In the autopsy, a neuroblastoma was found. The male patient was born at term. During the first hours of life, he developed severe respiratory failure requiring mechanical ventilation. Phenotypic features included cranial and facial dysmorphia, short thorax, tachycardia, heart murmur, abdominal distention, hepatomegaly, short extremities, widespread petechias, diminished muscular tone, ungueal hypoplasia in toes, bilateral cryptorchidia, and generalized redundant skin. In the evolution he presented several sepsis episodes, difficulty for feeding, supraventricular arrhythmia, two heart arrests, and opisthotonos, and died at 65 days of life due to heart arrhythmia. The autopsy revealed hydrocephaly, a neuroblastoma, and a heart without anatomic alterations. Costello syndrome was diagnosed. Costello syndrome is not frequent; in this patient, the diagnosis was suspected in life and was confirmed postmortem, the topic is reviewed, the important aspect in this case is the association with a neuroblastoma.

Congenital neuroblastoma in a boy born to a woman with bipolar disorder treated with carbamazepine during pregnancy.

1. A metastatic neuroblastoma was detected immediately after birth in a boy born to a 26 year old woman with bipolar disorder, who received carbamazepine (400 mg/day) all through her pregnancy. The primary tumor was probably located in the adrenal gland of the right side, and multiple metastatic lesions were detected in the skin. 2. In this report the authors review the literature about the side effects teratogenic and carcinogenic effects of carbamazepine, the epidemiology and evolution of the neuroblastoma, and the current scientific opinion about the pharmacological treatment of the pregnant with mood disorders. 3. A causal relationship between the use of carbamazepine and the neuroblastoma development in the present case can not be established; however, as the carcinogenic and teratogenic effects of the drug have been basically assessed in epileptic women, our aim is to alert the medical community in order to conduct further research in psychiatric patients.

Hidrops fetalis y neuroblastoma congénito: presentación de 1 caso / Hydrops fetalis and congenital neuroblastoma: report of a case

Se presenta un caso de hydrops fetalis asociado a un neuroblastoma de la suprarrenal derecha con metástasis hepática. Se revisa la literatura de los casos informados durante las 3 últimas décadas y las hipótesis que tratan de explicar este tipo de hydrops de causa no inmunológica