Melanie Richards

Pancreatoduodenal (PD) neoplasms represent the principal disease-specific lethality in multiple endocrine neoplasia type 1 (MEN1). Potential oncologic benefits of PD resection must be weighed against operative morbidities, compromised pancreatic function, and quality of life (QOL). Fifty MEN1 patients underwent PD resections during 1984-2004. Postoperative pancreatic function and QOL were assessed by EORTC QLQ-C30 and a disease-specific questionnaire (response rate, 78%). Twelve patients (24%) had asymptomatic disease detected by screening; 38 patients (76%) were symptomatic. All gross neoplasm was resected in 80% of patients. No patients died; 21 patients (42%) had complications. At 5 years postoperatively, 60% of patients were alive without disease, 24% of patients were with disease, 10% of patients died of PD neoplasms, 4% of patients died of other malignancies, and 2% of patients died of an unknown cause. Diabetes that requires insulin or oral hypoglycemics developed in 20% of p...

Patients with hyperparathyroidism have alterations in carbohydrate metabolism characterized by insulin resistance, hyperinsulinemia, and glucose intolerance. The clinical significance of these findings in the management of patients with diabetes mellitus (DM) after parathyroidectomy for hyperparathyroidism has been controversial. A retrospective review identified 87 patients with DM and hyperparathyroidism who underwent parathyroidectomy. The follow-up documentation of 70 patients who underwent diabetic management was then evaluated to assess the benefit of parathyroidectomy on glucose management. Thirteen patients had type 1 DM, and 74 patients had type 2 DM. Primary hyperparathyroidism was present in 93% of patients with type 2 DM; 64% of patients with type 1 DM had secondary hyperparathyroidism. At follow-up, glucose control was stable in 40% of patients, had improved in 37% of patients, and had deteriorated in 23% of patients (P = .003). Improved glucose control was not dependent on age, duration of DM, duration of hyperparathyroidism, length of follow-up, or calcium levels. The patients with decreased requirements had a significantly lower parathyroid hormone level (P = .05). Improved glucose control was most significant in patients whose condition was managed with oral hypoglycemics (P = .05) or insulin (P = .03). The clinical and laboratory investigations on the influence of hyperparathyroidism on DM support the benefit of parathyroidectomy in patients with DM. Patients with type 1 and type 2 DM show improvement in glucose control after parathyroidectomy. The presence of DM and hyperparathyroidism is an indication for parathyroidectomy because it results in either stabilization or improved glucose control in 77% of patients.

Cystic neuroblastoma is exceedingly rare; only 29 cases have been reported in the medical literature. The authors treated two children with cystic neuroblastoma. The first was a 2-week-old boy with an adrenal lesion; the second was a 15-month-old girl with solitary thoracic cystic neuroblastoma. Cystic neuroblastoma is located, almost exclusively, in the adrenal gland. In contrast to solid neuroblastoma, cystic neuroblastoma has a benign course. Cystic neuroblastoma is diagnosed earlier than solid neuroblastoma, and the former rarely presents with metastatic lesions. All previous reported cases of cystic neuroblastoma were diagnosed before 21 months of age. The presence of calcification was rare, and only 9.5% had documented elevation of the vanillylmandelic acid or homovanillic acid level. Surgical resection of all gross tumor was accomplished in 86% of children with cystic neuroblastoma, and in this group none of the patients had reported recurrent disease. Both present cases also had surgical resection of the entire gross tumor, and likewise have had no evidence of recurrent disease. This report brings the total number of reported pediatric cases of cystic neuroblastoma to 31; to the authors' knowledge, this is the first report of a child with solitary cystic neuroblastoma in an intrathoracic location.