Extrarenal testicular Wilms' tumor in a 3-year-old child.

We report an extremely rare case of extrarenal testicular Wilms' tumor in a 3-year-old boy with intrabdominal undescended left testis. The patient was admitted because of pain and vomiting, with evidence of a huge abdominal mass. At surgery a large tumor arising from the intrabdominal testis was found. Histology showed the classical triphasic Wilms' tumor elements: epithelial, mesenchymal and blastemal areas. Extrarenal Wilms' tumors account for only 3% of all Wilms' tumors and just -100 cases have been reported in literature. Testicular origin is anecdotic. We present histomorphological, histogenetic, clinical, diagnostic, prognostic and therapeutic features of this rare tumor.

[An unusual case of macroscopic hematuria in pediatric age]. / Un caso insolito di ematuria macroscopica in età pediatrica.

An unusual case of macroscopic hematuria in a 14 year old boy is presented. At the time of the first hospital admission, no urinary tract infection could be demonstrated, in spite of the associated symptoms of stranguria and dysuria. At ultrasound examination, only a mild thickening of the upper bladder wall was detected, and cystoscopy showed a huge oedema and inflammation of the mucosal layer. The biopsy of the bladder was characterized by a definite eosinophilic infiltration; due to this particular hystologic pattern, the diagnosis of eosinophilic cystitis was made. In the following months, the boy did not improve. Recurrent hematuria occurred, and a pseudo-polypoid mass in the inner bladder wall was detected at ultrasonography. A limited resection of the vesical dome was performed, to remove completely the mass. The hystologic examination showed Schistosoma Haematobium eggs in the bladder wall, with a typical granulomatous reaction. The post-operative course was uneventful, and the child was completely cured after Praziquantel treatment. The Authors underline the need to take into account Schistosomiasis in cases of hematuria, particularly when this symptom affects boys coming from countries where Bilharziasis is endemic.

Burst suppression and impairment of neocortical ontogenesis: electroclinical and neuropathologic findings in two infants with early myoclonic encephalopathy.

We report the electroclinical and neuropathologic correlations in 2 children aged 2.5 months affected by early myoclonic encephalopathy characterized by epileptic seizures, erratic myoclonus, and an EEG pattern of burst suppression. Despite different etiologies, the neuropathologic findings showed similar abnormalities in both cases, with no substantial impairment of the myelination processes. Islands of matrix tissue scattered in the periventricular region and neurons aligned marginally in the bulbar olives were detected. The presence of numerous large spiny neurons dispersed in the white matter along the axons of the cortical gyri was the most striking finding. The neurons have been interpreted as abnormally persisting interstitial cells in 2.5-month-old children. These early generated neurons, normally present during neocortical histogenesis, are programmed to die near the end of gestation or soon after birth. The interstitial cells are regarded as a waiting compartment of afferent fibers during cortical development. Their persistence in our patients represents an anatomic condition for cortical disconnection providing a pathophysiologic basis to burst-suppression phenomena.