Transplantation and Debulking Procedures for Neuroendocrine Tumors.

An array of treatment modalities is currently available in the management of patients with neuroendocrine tumors (NETs). Complete resection of the primary tumor and all metastatic lesions represents the only approach possessing intent to cure. However, due to patients' disease frequently being at an advanced stage on initial diagnosis only a minority of individuals are candidates for radical procedures. Cytoreductive surgery (debulking) may potentially confer improvements in quality of life and prolong overall survival. In light of the recent introduction of effective nonsurgical treatment options, the indication for cytoreductive surgery needs to be carefully assessed. The presence of nonresectable liver metastases is not a contraindication for resection of the primary tumor including locoregional disease in small bowel NETs. Resection of primary pancreatic NETs in the setting of unresectable metastatic liver disease may have a beneficial effect on the prognosis of selected patients with a tumor requiring less aggressive local surgery. Liver transplantation presents a generally accepted approach in meticulously selected patients with unresectable liver metastases. Stringent and validated selection criteria are not available. While overall survival is satisfactory, high recurrence rates hinder outcomes and call for the development and implementation of neoadjuvant and adjuvant concepts. Advances in intestinal transplantation over time may justify consideration of an individual with advanced neuroendocrine tumor disease unresponsive to standard medical or surgical treatment.

Modified liver-free multivisceral transplantation for a metastatic small bowel neuroendocrine tumor: a case report.

Neuroendocrine tumors originating from the small bowel frequently metastasize to the lymph nodes and/or liver. Although surgical extirpation of the primary tumor and locoregional metastases epitomizes the management of patients with such tumors, this is not always possible with conventional surgical techniques. Nonresectable, slow-growing tumors involving the mesenteric root represent a generally accepted indication for deceased donor intestinal and multivisceral transplantation. Furthermore, vascularized sentinel forearm flaps offer opportunities for monitoring graft rejection and tailoring immunosuppression regimens. Here, we report the first documented case of modified liver-free multivisceral transplantation preceded by neoadjuvant 177-lutetium peptide receptor radionuclide therapy in a patient with a small bowel neuroendocrine tumor and extensive lymph node metastases in the mesenterium. At a follow-up of 21 months the patient is biochemically and radiologically disease-free.