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Plasma Chromogranin A in Patients with Multiple Endocrine Neoplasia Type 1

Departments of Medical Sciences (D.G., B.E., K.Ö., B.S.) and Clinical Chemistry (M.S., G.L.), University Hospital, S-751 85 Uppsala, Sweden; and the Departments of Medicine (R.S.), Huddinge University Hospital and St. Göran Hospital, S-11281 Stockholm, Sweden

Address all correspondence and requests for reprints to: Britt Skogseid, M.D., Department of Endocrine Oncology, Division of Internal Medicine, University Hospital, S-751 85 Uppsala, Sweden. E-mail: britt.skogseid{at}medicin.uu.se

Plasma chromogranin A (CgA) has been claimed to be a sensitive marker for neuroendocrine tumors, but its role in the early diagnosis of multiple endocrine neoplasia type 1 (MEN 1) pancreatic endocrine tumors has not been evaluated. We measured CgA in 36 patients with MEN 1, of whom 9 lacked pancreatic involvement, 20 had biochemical evidence of pancreatic endocrine tumors, and 7 displayed radiologically detectable pancreatic tumors. CgA was also analyzed in 25 patients with sporadic pancreatic endocrine tumors, 39 subjects with inflammatory bowel disease, 7 patients harboring nonendocrine pancreatic disease, and 19 healthy controls. Four of 9 of the MEN 1 patients without pancreatic involvement had elevated CgA. Furthermore, 60% with biochemically unequivocal tumors and all with a radiologically visible tumor showed elevations. All 25 patients with sporadic pancreatic endocrine tumor had increased CgA, as had 28% of patients with inflammatory bowel disease and 57% with nonendocrine pancreatic disease. Mean day to day CgA variation was 29% (range, 0–113%) in the neuroendocrine tumor patients and 21.0% (range, 0.0–47%, within reference range) among healthy controls. In summary, nonendocrine diseases may cause elevation of CgA, and its spontaneous variation can be considerable. Plasma chromogranin A is the most sensitive of the basal markers for neuroendocrine tumors, but cannot replace other established measures when screening for early pancreatic involvement in MEN 1.

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