Unusual functioning endocrine tumors

Opinion statement

Endocrine surgeons should maintain a high index of suspicion when patients are diagnosed with clinical signs or symptoms of parathyroid carcinoma. Although rare, the best chance for cure of these patients is at the time of the initial operation. Surgical resection of recurrent disease can provide effective palliation and can sometimes be assisted using gamma-probe directed dissection of sestamibi-labeled tumor tissue. Treatment of hyper-parathyroidism in the setting of multiple endocrine neoplasia type 1 (MEN-1), particularly in the reoperative setting, can be aided by using the rapid intraoperative parathyroid hormone assay to judge the adequacy of parathyroid debulking. In addition, in selected cases, the gamma probe can assist in identifying the location of ectopic or autografted sestamibi-labeled parathyroid tissue. Patients with incidental adrenal masses rarely require fine needle aspiration to exclude metastatic cancer. Fine needle aspiration, if performed, should never precede hormone evaluation to exclude pheochromocytoma. Patients who are diagnosed with incidental adrenal masses in the setting of a prior or concurrent cancer diagnosis are equally likely to have a primary adrenal mass as they would be to have metastatic cancer in the adrenal gland. Pheochromocytomas occasionally develop in patients with MEN-1. In suspicious cases, molecular identification of an MEN-1 mutation can be used to confirm the diagnosis. Preoperative hormone evaluation of a patient with an adrenal incidentaloma should include evaluation for subclinical Cushing’s syndrome through an overnight 1-mg dexamethasone suppression test. Identification of this condition allows for safe peri- and postoperative steroid hormone replacement, with very slow withdrawal of exogenous steroids to allow the opposite adrenal gland to recover and avoid postoperative Addisonian crisis. Paragangliomas are more commonly multifocal and malignant compared to pheochromocytomas. Evaluation of patients with paragangliomas should include radiographic staging for multifocality and metastatic disease, and postoperative hormone and radiographic follow-up evaluation should be performed. Consideration should be given to genetic testing for von Hippel-Lindau and succinate dehydrogenase mutations. Surgical treatment of rare functioning pancreatic and duodenal endocrine tumors, such as metastatic sporadic insulinoma and MEN-1-associated gastrinoma, can provide effective palliation. Surgical treatment should be integrated into a comprehensive treatment scheme that recognizes the natural history of the disease and incorporates appropriate adjunctive therapies and follow-up strategies.