LAUSR

FIGURE 1. A, Abdominal CT scan showing multiple liver metastases. B, Abdominal magnetic resonance imaging scan showing no evidence of liver metastases. W e report an extremely rare case of a metastatic VIPoma with insulin cosecretion, which showed complete response to lanreotide, along with capecitabine and temozolomide (CAPTEM). To our knowledge, no other similar case has been reported. This case suggests that CAPTEM might be a valid option for well-differentiated functioning pancreatic neuroendocrine tumors (pNETs) with hormonal cosecretion. VIPomas are rare NETs, and the majority present with distant metastases at diagnosis (70%). Surgical resection is the standard treatment. Somatostatin analogs are the first-line medical treatment, but second-line remains uncertain. Everolimus and sunitinib have been approved for pNETs, whereas peptide receptor radionuclide therapy (PRRT) has been increasingly used for functioning pNETs. Several chemotherapy regimens have also been suggested by consensus guidelines from international societies, including CAPTEM, with proven efficacy in retrospective studies. VIPomas are rarely associated with hormonal cosecretion. In 2 retrospective analyses of 323 and 435 pNETs, 4% and 3.5% developed cosecretion during the course of the disease, respectively. In both studies, hormonal cosecretion was observed exclusively in patients with metastatic disease. These patients had tumors with higher proliferative index and worse prognosis than other patients with functioning pNET. Few studies have been published regarding treatment of pNETwith hormonal cosecretion. They report on several therapeutic regimens, including PRRT, chemotherapy, everolimus, and sunitinib, generally with short-lasting responses. We report the case of a 48-year-old woman, who was admitted to the emergency department with watery diarrhea and severe hypokalemia (1.9 mEq/L). Abdominal computed tomography (CT) scan showed a 4.5-cm suspicious nodule in the pancreas tail and no distant metastases. Further workup revealed high VIP (vasoactive intestinal peptide). A distal pancreatectomy,