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Pediatric adrenocortical tumors are rare and heterogeneous endocrine malignancies. Objectives To report clinical, biochemical, and histological features, staging, and therapeutic interventions in a cohort of 28 patients treated at a single tertiary center. Methods A retrospective review of medical records of children with PACT (diagnosed before Results Mean age at diagnosis was 4.6 years (range, 0.3–17.3 years) and median follow-up was 4.17 years (range, 0–12 years). Female to male ratio was 2.5:1. Signs and symptoms that prompted medical intervention were hormonal overproduction (57%), abdominal complaints (36%), and hypertensive encephalopathy (7%). In patients with clinically virilizing tumors (n=16) mean height standard deviation score (SDS) and bone age advance were significantly higher while body mass index (BMI) SDS was significantly lower than in those with clinical Cushing’s (n=10) (p Conclusions Height SDS and BMI SDS seem to mirror hormonal secretion in pediatric adrenocortical tumors. Higher DHEAS levels were found in patients with more advanced disease. Further large-scale studies are needed to validate a possible role for DHEAS as a biochemical marker of tumor stage and to draw robust conclusions on the use of adjuvant chemotherapy.

Collaborative clinical trials have enlarged the knowledge base about pediatric tumors; however, this is not the case for rare tumors (RT).To describe the prevalence, clinical characteristics, and course of RT in pediatric patients diagnosed at Hospital Garrahan.Descriptive, retrospective study of patients aged 0-18 years diagnosed with a RT and admitted between January 2007 and December 2017.Out of 1657 patients diagnosed with solid tumors, 164 (9.9 %) were RT; 71.95 % (118) of patients were younger than 14 years and 81.7 % (130) were males. In order of frequency, RT were thyroid carcinoma (60), adrenal carcinoma (14), lung tumors (14), melanoma (13), salivary gland cancer (11), gastrointestinal tumors (8), non-germ cell gonadal tumors (7), pancreatic tumors (7), renal carcinomas (6), nasopharyngeal carcinomas (5), pheochromocytoma and paraganglioma (5), and thymic carcinoma in 1 patient. Treatment depended on tumor type and stage. The median follow-up was 34.9 months (range: 1-128.5 months); 133 patients (78.7 %) are alive and only 10 patients (6 %) were lost-to-follow-up.The prevalence of RT was 9.9 %. Twenty-seven percent occurred in adolescents. The most frequent tumors included thyroid carcinoma, adrenal carcinoma, and melanoma. Treatment and course varied based on tumor histology. Predisposing molecular alterations were found in 5.3 % of patients; 3.5 % had a history of cancer.Introducción. Los ensayos clínicos cooperativos han aumentado el conocimiento sobre los tumores pediátricos; sin embargo, no es el caso de los tumores raros (TR). Objetivo. Describir prevalencia, características clínicas y evolución de los TR en la edad pediátrica diagnosticados en el Hospital Garrahan. Material y métodos. Estudio descriptivo retrospectivo de pacientes entre 0 y 18 años con diagnóstico de TR ingresados entre enero de 2007 y diciembre de 2017. Resultados. De 1 657 pacientes con diagnóstico de tumores sólidos, 164 (9,9 %) correspondieron a TR, 71,95 % (118) eran menores de 14 años y 81,7 % (130) eran varones. En orden de frecuencia, los TR fueron: carcinoma de tiroides (60), carcinoma suprarrenal (14), tumores pulmonares (14), melanoma (13), carcinoma de glándulas salivales (11), tumores gastrointestinales (8), tumores gonadales no germinales (7), tumores pancreáticos (7), carcinomas renales (6), carcinomas nasofaríngeos (5), feocromocitoma y paraganglioma (5) y carcinoma de timo en 1 paciente. El tratamiento recibido dependió del tipo de tumor y del estadio. Con una mediana de seguimiento de 34,9 meses (rango: 1-128,5 meses), 133 pacientes (78,7 %) están vivos y solo 10 pacientes (6 %) se perdieron durante el seguimiento. Conclusión. La prevalencia de TR fue del 9,9 %. El 27 % se presentaron en adolescentes. Los tumores más frecuentemente diagnosticados fueron carcinoma de tiroides, carcinoma suprarrenal y melanoma. El tratamiento y la evolución varió según el tipo histológico. Se hallaron alteraciones moleculares predisponentes en el 5,3 % de los pacientes, el 3,5 % tenían antecedente de patología oncológica.

Background: Previous studies have shown that children and adolescents with COVID-19 generally have mild disease. Children and adolescents with cancer, however, can have severe disease when infected with respiratory viruses. In this study, we aimed to understand the clinical course and outcomes of SARS-CoV-2 infection in children and adolescents with cancer. Methods: We did a cohort study with data from 131 institutions in 45 countries. We created the Global Registry of COVID-19 in Childhood Cancer to capture de-identified data pertaining to laboratory-confirmed SARS-CoV-2 infections in children and adolescents (

Background Cooperative clinical trials has increased the knowledge on pediatric tumors, however, this is not the case for rare tumors (RT). Objective To describe the incidence, clinical characteristics and outcome of RT in the pediatric age diagnosed at Garrahan Hospital. Material and methods Retrospective descriptive study of patients (pts) between 0 and 18 years admitted between January 2 007 and December 2 017, with diagnosis of RT. Results Of 1 657 pts with diagnosis of solid tumors, 164pts (9.9%) corresponded to RT, 71.95% (118pts) were under 14 years old and 81.7% (130pts) were male. In order offrequency RT were: thyroid carcinoma (ca) 60pts, adrenal ca 14pts, lung tumors 14pts, melanoma 13pts, salivary glands ca 11pts, gastrointestinal tumors 8pts, non-gonadal germinal tumors 7pts, pancreatic tumors 7pts, renal ca 6pts, nasopharyngeal ca 5pts, pheochromocytoma/paraganglioma 5pts, timo 1pte. The treatment received depended on the type of tumor and stage. With a median follow-up of 34.9 months (range: 1- 128.5 months), 133pts (78.7%) are alive and only 10pts (6%) were lost to follow-up. Conclusion Knowing these initial data will allow us to propose new registration strategies and to develop multidisciplinary proposals for diagnosis, treatment and follow-up.

Botulism is an important public health problem in Argentina. It is a potentially fatal disease, and its diagnosis may be difficult. There are rare presentation forms of the disease, such as acute abdomen. We present a 4-month baby with a 3-day constipation condition, associated with weakness and abnormal eating attitude in the last 12 hours. The baby presented preserved muscle tone, with no changes in sucking or deglutition according to the mother's observations. Altered sensorium and acute abdomen were found; the patient was entered into the operating room with presumptive diagnosis of intussusception, which was confirmed by pneumatic desinvagination. During hospitalization, the patient did not make good progress and presented weak cry, progressive hypotonia and respiratory failure requiring intensive care. Clostridium botulinum was isolated from the stool sample and botulinum toxin type A was isolated from serum. The patient was treated with equine botulinum toxin. Twenty five days after admission, he was totally recovered.

Cardiac tamponade is an uncommon disorder in pediatric patients. We report a case of cardiac tamponade secondary to accumulation of cerebrospinal fluid due to ventriculo-atrial derivation (VAD) disfunction. An 11-month-old girl was assisted because of respiratory distress and tachycardia, without fever. She had a ventriculoatrial derivation, because of hydro-cephalus since two month earlier. The echocardiogram showed a pericardial effusion and the distal VAD located inside the right cardiac ventricle. The distal VAD was retired and replaced between superior cava vein and right atrium. The patient recovered ad integrum without pericardial effusion. In cases of patients with VAD presenting a clinical disorder, it should be ruled out VAD dysfunction or inappropriate placement.

Botulism is an important public health problem in Argentina. It is a potentially fatal disease, and its diagnosis may be difficult. There are rare presentation forms of the disease, such as acute abdomen. We present a 4-month baby with a 3-day constipation condition, associated with weakness and abnormal eating attitude in the last 12 hours. The baby presented preserved muscle tone, with no changes in sucking or deglutition according to the mother's observations. Altered sensorium and acute abdomen were found; the patient was entered into the operating room with presumptive diagnosis of intussusception, which was confirmed by pneumatic desinvagination. During hospitalization, the patient did not make good progress and presented weak cry, progressive hypotonia and respiratory failure requiring intensive care. Clostridium botulinum was isolated from the stool sample and botulinum toxin type A was isolated from serum. The patient was treated with equine botulinum toxin. Twenty five days after admission, he was totally recovered.

Community-acquired Staphylococcus aureus (CA-SA) infections are becoming more frequent. Most cases present an infection of skin and soft tissue, and the most invasive forms observed are osteoarticular and pleuropulmonary infections. Meningitis is a rare manifestation of Staphylococcus aureus infections. We describe an unusual case of CA-MRSA infection. An infant of eight months presented with signs of irritability and 4 days duration fever, with alternating sensory and abdomen pain. Acute abdomen surgery was discarded and hospitalization was decided with diagnosis of sepsis due to probable enteral focus; antibiotics were indicated. Blood cultures and cerebrospinal fluid culture were positive for MRSA. Sepsis with meningitis by MRSA was diagnosed. On the 7th day of hospitalization the infant presented neurological signs and symptoms. On the resolution computed tomography and the magnetic resonance, images compatible with myelitis were observed. The patient complied with the 21 day endovenous treatment, and showed positive results, being discharged from hospital a month after the appearance of the symptoms.

Cardiac tamponade is an uncommon disorder in pediatric patients. We report a case of cardiac tamponade secondary to accumulation of cerebrospinal fluid due to ventriculo-atrial derivation (VAD) disfunction. An 11-month-old girl was assisted because of respiratory distress and tachycardia, without fever. She had a ventriculoatrial derivation, because of hydro-cephalus since two month earlier. The echocardiogram showed a pericardial effusion and the distal VAD located inside the right cardiac ventricle. The distal VAD was retired and replaced between superior cava vein and right atrium. The patient recovered ad integrum without pericardial effusion. In cases of patients with VAD presenting a clinical disorder, it should be ruled out VAD dysfunction or inappropriate placement.

Acute otitis media is the most common bacterial infection in children. Despite efficient antibiotic therapy, there are still reports of both intratemporal and intracranial complications with potential risk of high morbidity and mortality. Mastoiditis, apical petrositis and labyrinthitis are caused by the extension of purulent middle ear infection into nearby structures. Giuseppe Gradenigo first described the clinical triad of acute otitis media, unilateral pain in the region innervated by the 1 masculine and 2 masculine branches of the trigeminal nerve and ipsilateral abducens nerve paralysis. This is a serious but rare complication of middle ear infection that should be suspected in every patient with unilateral headache and abducens nerve palsy. We report a case of Gradenigo's syndrome in a 6-year-old boy.