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OBJECTIVE. The aim of this study was to review our institutional experience managing pheochromocytomas and paragangliomas in children. METHODS. A retrospective chart review of the Mayo Clinic database from 1975 to 2005 identified 30 patients < 18 years of age with histologically confirmed pheochromocytoma or paraganglioma. RESULTS. There were 12 patients with pheochromocytomas and 18 with paragangliomas. The most common presenting symptoms were hypertension (64%), palpitation (53%), headache (47%), and mass-related effects (30%). Nine patients (30%) had a genetic mutation or documented family history of pheochromocytoma or paraganglioma. Fourteen patients (47%) had malignant disease, whereas 16 (53%) had benign disease. Logistic analysis showed that statistically significant risk factors for malignancy were (1) paraganglioma, (2) apparently sporadic, as opposed to familial, pheochromocytoma or paraganglioma, and (3) tumor size of >6 cm. Surgical resection was performed for 28 patients (93%), with perioperative mortality and major morbidity rates of 0% and 10%, respectively. Resection achieved symptomatic relief for 25 patients (83%). All patients with benign disease appeared cured after resection. For patients with malignant disease, the 5- and 10-year disease-specific survival rates were 78% and 31%, respectively, and the mean survival time was 157 [+ or -] 32 months. CONCLUSIONS. The incidence of malignant pheochromocytoma/paraganglioma was high in children (47%), particularly those with apparently sporadic disease, paraganglioma, and tumor diameters of >6 cm. Patients with a known genetic mutation or familial pheochromocytoma/paraganglioma were more likely to achieve resection with negative microscopic margins and had improved disease-specific mortality rates. Surgical resection remains the treatment of choice for pheochromocytoma and paraganglioma. Key Words pheochromocytoma, paraganglioma, children, surgery Abbreviations VMA--vanillylmandelic acid HVA--homovanillic acid MIBG--meta-iodobenzylguanidine, PHEOCHROMOCYTOMA AND PARAGANGLIOMA are exceptionally rare neoplasms in children. Both tumors develop from neural crest cell lineage, stain positive for chromaffin, and produce catecholamines, including dopamine, norepinephrine, and epinephrine. Tumors [...]

Objective. Primary hyperparathyroidism (HPT) is unusual in children. We reviewed our experience with HPT to better characterize these children. Methods. The retrospective review of patients Results. Fifty-two patients were identified. Median age was 16.8 years (range: 4-18.9) with a female-to-male ratio of 3:2. Eighty-five percent had an elevated parathyroid hormone (PTH) level, and 15% had an inappropriately normal PTH level during hypercalcemia. Serum calcium was elevated in all patients except for 2 with multiple endocrine neoplasma (MEN)-IIA and 1 with familial non-MEN HPT, but both had elevated PTH levels. Alkaline phosphatase levels were significantly higher in children with documented bone involvement. At presentation 41 patients (79%) were symptomatic and end-organ damage (nephrocalcinosis, nephrolithiasis, acute pancreatitis, or bone involvement) occurred in 23 patients (44%). Thirty-four patients (65%) had a single adenoma; hyperplasia was identified in 16 patients (27%), and of these cases, 57% occurred in patients diagnosed with MEN-I. Short-term complications included transient hypocalcemia in 29 patients (56%) and transient vocal cord paralysis in 2 patients (4%). Long-term complications were significant for permanent hypocalcemia in 2 patients (4%) and no recurrent laryngeal nerve injuries. No parathyroid abnormalities were identified during exploration in 4 (8%) children. Long-term follow-up was achieved in 98% of patients for a mean and median of 13 years. Resolution of hypercalcemia was achieved in 94% of cases. Conclusion. The diagnosis of primary HPT in pediatric patients is frequently delayed, is commonly symptomatic, and has significant morbidity. For children in whom HPT is suspected, evaluation of serum calcium and PTH levels is diagnostic in 100% of children. Parathyroid resection is effective at restoring normal serum calcium, has few complications, and is the treatment of choice for children with primary hyperparathyroidism. Pediatrics 2005;115:974-980; children, MEN, hypercalcemia, parathyroid adenoma., ABBREVIATIONS. HPT, hyperparathyroidism; PTH, parathyroid hormone; MEN, multiple endocrine neoplasia. Hyperparathyroidism (HPT) predominantly affects adults (incidence of 1 in 1000) and is very rare in children (incidence of 2-5 in [...]

Background: Inguinal hernia repair remains one of the most common operations performed by pediatric surgeons. We described a new surgical approach for treating bilateral inguinal hernias in girls through a small single transverse supra-pubic incision. Materials and Methods: A new approach was performed on female children 12-years-old and younger with bilateral inguinal hernias between January 2005 and April 2012. Technique: A single transverse suprapubic incision (1-1.5 cm) was made. Using sharp and blunt dissection bilateral hernias were exposed and repaired using a standard high ligation. Results: Ninety-nine girls with a preoperative clinical diagnosis of bilateral inguinal hernia were included. Median age was 2 years (range: 1 month to 12 years). All patients underwent general anesthesia. Median operative time was 12 minutes (range 5-22). There were no intra-operative complications or misdiagnosis. Two patients had bilateral sliding hernias and the remainder had indirect hernias. Post-operatively two patients developed non-expanding small hematomas, both treated non-operatively without sequelae. There were zero hernia recurrence and median follow-up was 5 years (range: 1-8 years) on 99% of patients. Conclusion: We described a new, safe, simple, and rapid approach for bilateral inguinal hernia repair in female pediatric population. A single transverse suprapubic skin incision was adequate for exposing both inguinal regions with excellent postoperative results. [ABSTRACT FROM AUTHOR]