Adrenal suppression secondary to prolonged inhaled corticosteroid use is usually limited to biochemical abnormalities, with no obvious clinical effects. Acute adrenal crisis is much rarer event but has been reported with increasing frequency. We report a case of a 7-year-old asthmatic child who presented with an acute history of lethargy after a respiratory infection. He was maintained on 220 μg/day of fluticasone propionate for several years. Initial evaluation revealed severe adrenal suppression, with undetectable cortisol levels and minimal response after stimulation with ACTH. After fluticasone was discontinued, a gradual recovery of the adrenal axis was seen. This case shows that acute adrenal crisis may be a consequence even at the usual prescribed doses, stressing the importance of using the lowest dose of inhaled steroids needed to control symptoms and having an increased awareness of this complication.
Pheochromocytomas are rare neuroendocrine tumors that secrete catecholamines and usually arise from the adrenal medulla. Catecholamine-producing tumors that arise from extra-adrenal chromaffin tissue are referred to as paragangliomas, or extra-adrenal pheochromocytomas. Contrary to the traditional "Rule of Tens," as many as 25% of pheochromocytomas occur in hereditary tumor syndromes, such as multiple endocrine neoplasia-2, von Hippel-Lindau disease, neurofibromatosis-1, or hereditary or familial paraganglioma syndrome. Surgical resection remains the only curative therapy for pheochromocytomas, and advances in minimally invasive techniques have shown laparoscopic adrenalectomy to be safe and effective. Paragangliomas are extremely rare tumors, especially in children, and the role of laparoscopy in their treatment is evolving. This case report and the accompanying video demonstrate that the laparoscopic approach to retroperitoneal paraganglioma resection provides excellent exposure of the tumor and surrounding structures. Given proper patient preparation and perioperative monitoring, laparoscopic exploration and resection of paragangliomas can be safely and successfully accomplished in children. In addition, genetic analysis should be considered for all patients presenting with these tumors.
Paragangliomas are extradadrenal neuroendocrine tumors, recently associated with gene mutations in the succinate dehydrogenase complex (SDH). These mutations are thought to be responsible for the familial paraganglioma syndrome. Average age of tumor diagnosis for SDH mutation carriers is about 30 years of age, but patients younger than 10 years have been reported. We present the case of a 13 year-old boy with abdominal paraganglioma, whose mother also had a history of thoracic paraganglioma diagnosed at 14 years of age. Both were found to carry a mutation in exon 4 of the SDHB gene, heterozygous for c.418G>T, p.Val140Phe sequence. Compared to the other SDH subtypes, SDHB associated tumors have been found to be much more aggressive. This has led to current recommendations that tumor screening of asymptomatic SDHB carriers should start as early as 10 years of age. An even earlier initiation seems warranted for the identified carriers in this family.
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