The patient is a 47y WF with a history of scleroderma and Reynaud’s disease, who suffers with episodes of explosive watery diarrhea, hypotension and severe diaphoresis. These episodes occur one to two times a month and last approximately 8 hours.
Occasionally, she experiences “smaller” episodes consisting only of diaphoresis. Before these attacks started approximately two years ago, the patient also had small bowel hypomotility problems attributed to her scleroderma, and has been on chronic TPN. She has failed several trials at jejunal tube feeds, and currently has a g-tube for drainage of her stomach. In addition, she has experienced multiple episodes of line sepsis with gram negative enteric organisms and has had several Hickman catheters and port-a-caths infected and removed. Finally, she has had GI bleeding with angiodysplasia found on colonoscopy. EGD has been unremarkable.
Her other work-up to date consists of the following “positive” findings: an elevated serum pancreatic polypeptide level (not a very specific test for neuroendocrine tumors as a large percentage of patients with chronic secretory diarrhea may have an elevated level without any tumor) and CT and MRI of the abdomen that shows an “enlargement ” of the head of the pancreas but not a specific tumor mass. In addition, the pancreatic duct appears dilated. CT-guided biopsy of this mass revealed normal pancreatic tissue with normal appearing islet cells. Serum gastrin and serotonin levels were normal and urine 5-HIAA was normal. She does have documented steatorrhea indicating a blockage of pancreatic lipase or an inhibition of pancreatic lipase secretion by the pancreatic polypeptide. The suspected diagnosis is a neuroendocrine tumor.
I do not think it is a gastrinoma or insulinoma or carcinoid. Perhaps it is a somatostatinoma or neurotensinoma or less likely a pancreatic polypeptide tumor. She is currently scheduled for exploratory laparotomy with possible Whipple procedure next week. I am attemptting to arrange intraoperative ultrasound to try and localize a tumor mass and obtain a tissue diagnosis before proceeding with any extensive surgical procedures in this already debilitated patient. However, if a neuroendocrine tumor is present, I would like to try and make this woman’s life a little more functional. Are there any other preoperative or intraoperative tests you would recommend? If a tumor is found is enucleation adequate therapy or is Whipple or total pancreatectomy required? If we do not find a tumor at laparotomy, where else do we “look” while we are “in there” ?