Abstract
BackgroundVIPomas are rare neuroendocrine tumors poorly described in the literature. Aggressive resection of patients with advanced VIPoma neuroendocrine tumors has rarely been reported.Case presentationA 46 year old women presented with abdominal pain and diarrhea. A three-dimensional (3-D) pancreas protocol computed tomography scan revealed an 18 × 12 cm pancreatic VIPoma abutting the liver, stomach, spleen, left adrenal, colon that also invaded the distal duodenum – proximal jejunum at the ligament of Treitz in association with sinistral portal hypertension. Following preoperative proximal splenic artery embolization, the patient with underwent successful en bloc resection of the locally advanced VIPoma in conjunction with a diaphragmatic resection, total gastrectomy, splenectomy, left adrenalectomy, as well as small and large bowel resection. The estimated blood loss was 500 ml. All margins were negative (R0 resection). The patient is alive and disease-free.ConclusionThis case illustrates the role of aggressive resection of pancreatic neuroendocrine tumors and highlights several key technical points that allowed for successful resection.
Highlights
VIPomas are rare neuroendocrine tumors poorly described in the literature
This case illustrates the role of aggressive resection of pancreatic neuroendocrine tumors and highlights several key technical points that allowed for successful resection
We provide a brief review of the role of aggressive resection of pancreatic neuroendocrine tumors and highlight several key technical points that allowed for successful resection
Summary
VIPomas are rare neuroendocrine tumors with an annual incidence of about 1 per 10,000,000 individuals.[1]. Assessment of the relationship between the tumor and adjacent vascular structures, such as the portal and superior mesenteric vein (SMV) as well as the celiac and superior mesenteric artery (SMA), is critical in determining preoperative resectability. A repeat threedimensional (3-D) pancreas protocol CT scan revealed an 18 × 12 cm mass abutting the liver, stomach, spleen, left adrenal, colon and invading the distal duodenum – proximal jejunum at the ligament of Treitz. Given the size of the mass and the associated extensive varices, the patient underwent preoperative proximal splenic artery embolization (Figure 3). Twenty-four hours following this, the patient was taken to surgery where she was found to have a very large mass arising from the body and tail of the pancreas that invaded the left diaphragm, stomach, left adrenal, fourth portion of the duodenum – first portion of the jejunum, transverse colon, and spleen. She received no adjuvant therapy and is currently alive and disease-free at 6 months of follow-up
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