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.

Comparison of Clinico-Radiological Features between Congenital Cystic Neuroblastoma and Neonatal Adrenal Hemorrhagic Pseudocyst

OBJECTIVE: To evaluate the radiological and clinical findings of congenital cystic neuroblastomas as compared with those of the cystic presentation of neonatal adrenal hemorrhage. MATERIALS AND METHODS: We analyzed the US (n = 52), CT (n = 24), and MR (n = 4) images as well as the medical records of 28 patients harboring congenital cystic neuroblastomas (n = 16) and neonatal adrenal hemorrhagic pseudocysts (n = 14). The history of prenatal detection, location, size, presence of outer wall enhancement, internal septations, solid portion, calcification, turbidity, vascular flow on a Doppler examination, and evolution patterns were compared in two groups of cystic lesions, by Fischer's exact test. RESULTS: All (100%) neuroblastomas and three (21%) of the 14 hemorrhagic pseudocysts were detected prenatally. Both groups of cystic lesions occurred more frequently on the right side; 11 of 16 (69%) for neuroblastomas and 11 of 14 (79%) for hemorrhagic pseudocysts. The size, presence of solid portion, septum, enhancement, and turbidity did not differ significantly (p > 0.05) between the two groups of cystic lesions. However, tiny calcifications (n = 3) and vascular flow on color Doppler US (n = 3) were noted in only neuroblastomas. The cystic neuroblastomas became complex solid and cystic masses, and did not disappear for up to 90 days in the three following cases, whereas 11 of the 14 (79%) hemorrhagic pseudocysts disappeared completely and the three remaining (27%) evolved to calcifications only. CONCLUSION: Although the imaging findings of two groups of cystic lesions were similar, prenatal detection, the presence of calcification on initial images, vascularity on color Doppler US, and evolution to a more complex mass may all favor neuroblastomas.

Congenital Neuroblastoma with Multiple Metastases: A Case Report

Neuroblastomas are derived from the neural crest ectoderm, and are the most common solid abdominal masses of infancy. Congenital neuroblastoma, however, is rare. We report a rare case of congenital neuroblastoma with multiple metastases found at autopsy, performed at 2 days after birth. He was born by cesarian section and weighed 2,350 g. His respiration was weak and abdomen was distended. The patient died 2 days after birth. Postmortem examination revealed a relatively well demarcated ovoid mass, in the left adrenal, with necrosis and hemorrhage. Multiple small metastatic tumor nodules in the liver, lung, kidney, brain, rib, thyroid glands, and spleen, were noted. The histopathological pictures confirmed the diagnosis of neuroblastoma of the adrenal with multiple metastasis.

Carcinoma suprarrenal congénito / Congenital carcinoma of the adrenal gland

En una niña recién nacida con una masa abdominal derecha y discreta hipertrofia del clítoris, la ultrasonografía abdominal y la cintigrafía hepática sugirieron inicialmente un tumor hepático; sin embargo, las mediciones en el plasma de 17-hidroxiprogesterona (12,5 ng/ml), actividad de renina (> 25 ng/ml * h), DHEA-S (12.500 *g%) y cortisol (8,1 *g/dl), como los de una segunda ecografía abdominal, hicieron evidente la ubicación suprarrenal del tumor. La masa fue extirpada quirúrgicamente en su totalidad, resultando en el estudio anatomopatológico un carcinoma suprarrenal. La paciente recibió tratamiento pre y postoperatorio con hidrocortisona y se encuentra en excelentes condiciones. Existen muy pocos informes en la literatura mundial sobre carcinomas suprarrenales identicados en el período neonatal. Se revisan otras publicaciones, enfatizando los hallazgos clínicos, exámenes que apoyan el diagnóstico y el tratamiento más recomendado actualmente

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