Neuroendocrine tumors (NETs) of unknown primary: is early surgical exploration and aggressive debulking justifiable?

BACKGROUND: Neuroendocrine tumors (NETs) are rare tumors that often present with vague symptoms. Identification and localization of the primary NET can be challenging and the true incidence remains unclear. These patients have been thought to have a poor prognosis compared to those patients with a known primary. Therefore, traditionally the treatments for patients with unknown primaries have been passive and directed towards symptom control and/or cytoreduction of metastatic disease. We hypothesized that NET of unknown primary are predominantly low-grade and easily located surgically and therefore are amendable to surgical debulking and cytoreduction, which will likely increase survival in these patients. METHODS: The charts for all 342 surgical patients, seen in our clinic at Ochsner-Kenner between 1/2009 and 9/2012 were retrospectively reviewed to determine which patients had a pre-operative diagnosis of a "NET with unknown primary". Twenty-two patients (6.4%) were identified. For these patients, the rate of successful surgical exploration in which a primary site was identified was recorded. Survival for these "unknown primary" patients were compared to a large similar group of NET patients from a recent study collected from this same Ochsner clinic group. RESULTS: Twenty-two (22/342, 6.4%) NET patients with a pre-operative diagnosis of an unknown primary were explored and cytoreduced. The primary tumor site was identified in all 22 patients (100%). The primary sites identified for these patients were 19 small intestines (86.4%) and 3 pancreatic (13.6%). All 22 patients had low-grade tumors and all were still alive as of 9/2012, not allowing for a survival curve to be generated. CONCLUSIONS: Unknown primary NETs are not associated with a poor prognosis as previously reported. Timely surgical exploration and debulking always results in the identification of the primary and a maximum cytoreduction. Early surgical exploration with aggressive debulking is indicated for the treatment of these patients, as for the known counterpart.

Evaluation and management of the pediatric thyroid nodule.

Thyroid nodules are commonly diagnosed in adults. Although rare in children, the risk for thyroid cancer is much higher in the pediatric population compared with adults. Presenting as either a solitary nodule or a multinodular goiter, thyroid nodular disease in children requires a thorough workup that includes a detailed clinical examination comprised of prior history of thyroid disease in the patient or in their family, history of radiation exposure, careful palpation of the thyroid and lymph nodes, blood tests, ultrasonography, and cytological assessment. Thyroid surgery is the gold-standard treatment for pediatric thyroid nodules; nonetheless, the extent of surgery remains controversial. Because surgery is not without risk, the decision matrix necessitates focus on the benefits of surgery for the child contingent upon all the preoperative exams. New diagnostic technology such as molecular testing with fine needle aspiration biopsy may help distinguish between benign and malignant lesions while potentially decreasing surgery for benign disease. The objective of this review is to summarize new concepts in clinical disease management of nodular thyroid disease in the pediatric population, including patient history, medical examination, and diagnosis workup.

Prophylactic thyroidectomy: who needs it, when, and why.

The most common hereditary thyroid cancer is medullary thyroid cancer, which can be associated with MEN 2A, MEN 2B, or FMTC. In these patients, prophylactic thyroidectomy is recommended; timing of surgery is dependent on the specific RET mutation. Prophylactic thyroidectomy should include total thyroidectomy and accompanying central compartment neck dissection should be done for patients at high risk for micro-metastatic disease only. Surgery should be performed at tertiary care institutions by high-volume thyroid surgeons.

Use of a sestamibi-only approach to routine minimally invasive parathyroidectomy.

We performed this study to compare a sestamibi-only radio-guided approach (MIBI) versus using intraoperative parathyroid hormone monitoring (IOPTH) in the performance of minimally invasive parathyroidectomy (MIP) in patients with a clearly positive preoperative sestamibi scan from January 2000 to June 2010. Five of 81 patients in the MIBI group required additional surgery, three at the time of MIP when the intraoperative findings were in conflict with the preoperative sestamibi scan and two required a second operation as a result of an undiscovered second adenoma. In the IOPTH group, five patients had an unnecessary bilateral neck exploration as a result of an inadequate drop in PTH levels, whereas six had their disease cured because the PTH levels predicted additional pathology. One patient in the IOPTH group remains hypercalcemic and represents the only surgical failure in this study. The MIBI group had a shortened operating room time and less cost (P < 0.001). No deaths or complications, including recurrent laryngeal nerve injuries, occurred in this study. Although both strategies are effective in managing hyperparathyroidism, a MIBI-only approach is less expensive and has shorter operative times with an occasional need for reoperation, whereas the IOPTH group results in more extensive surgery that will occasionally be unnecessary.