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 <18 years of age) followed between 1987–2018 at Hospital de Pediatría Garrahan, Buenos Aires, Argentina. 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<0.05). Serum dehydroepiandrosterone sulfate (DHEAS) levels were significantly higher in stage IV than in stage I (p=0.03). Total adrenalectomy was performed in 26 patients. Eight patients (stage III-IV) received adjuvant chemotherapy. Five-year overall and disease-free survival were 100% for ST I-II, and 51% (95% CI 21–82) and 33% (95% CI 1.2–65) for ST III-IV, respectively (p=0.002). No statistical difference was found when comparing 2-year parameters with and without adjuvant chemotherapy. 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.
Arch Argent Pediatr 2015;113(5):e286-e289 / e286Presentación de casos clínicos RESUMEN El botulismo es un importante problema de salud pública en Argentina. Es una enfermedad potencialmente letal y de difícil diagnóstico. Existen casos de presentación infrecuente de dicha enfermedad, como el abdomen agudo. Exponemos el caso de un niño de 4 meses, que consultó por constipación de 3 días de evolución, asociada a decaimiento y regular actitud alimentaria de 12 horas de evolución. Presentaba tono muscular conservado, sin alteraciones en la succión ni deglución, según la referencia materna. Se constató sensorio alternante y abdomen agudo, por lo que ingresó a quirófano con sospecha de invaginación intestinal, la cual fue confirmada mediante desinvaginación neumática. Durante la internación, el paciente evolucionó desfavorablemente y presentó llanto débil, hipotonía progresiva e insuficiencia respiratoria, por lo que requirió cuidados intensivos. Se aisló Clostridium botulinum en la muestra de materia fecal y toxina botulínica tipo A en el suero. Recibió toxina antibotulínica equina como tratamiento, con recuperación total a los 25 días de haber ingresado. Palabras clave: botulismo, hipotonía muscular, Clostridium botulinum, invaginación.
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.
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