A calcitonin and vasoactive intestinal peptide-producing pancreatic endocrine tumor associated with the WDHA syndrome.
BACKGROUND
Although pancreatic endocrine tumor can produce a variety of hormones, few pancreatic tumors produce a high systemic calcitonin concentration. Furthermore, calcitonin-producing pancreatic tumors rarely produce elevations of VIP in addition.


METHODS
We evaluated and treated a 50-yr-old woman with the WDHA syndrome. Abdominal computed tomography (CT) detected a tumor in the tail of the pancreas. Peripheral plasma calcitonin and VIP concentrations were markedly increased to 2000 pg/mL (normal, <74 pg/mL) and 7200 pg/mL (normal, <100 pg/mL), respectively. We diagnosed a calcitonin- and VIP-producing pancreatic endocrine tumor, which was removed by distal pancreatectomy including splenectomy.


RESULTS
Plasma calcitonin and VIP were determined in blood from the vein draining the tumor and splenic vein, sampled at operation. These secreted concentrations were extremely high: 4640 and 3610 pg/mL for calcitonin; 24700 and 13500 pg/mL for VIP. Calcitonin and VIP were also highly elevated in the resected tumor. Plasma calcitonin and VIP rapidly decreased after tumor resection. The patient has been well without recurrence for over 20 yr.


CONCLUSIONS
An unusual pancreatic tumor secreting vasoactive intestinal peptide (VIP) caused WDHA syndrome (watery diarrhea, hypokalemia, and achlorhydria/hypochlorhydria) and also hypercalcemia. The latter was only partially offset by a large excess of calcitonin also secreted by the tumor.
Ichimura
Tatsunosuke
T
Hokkaido University Graduate School of Medicine, Division of Cancer Medicine, Cancer Medicine, Surgical Oncology, Sapporo, Japan. tatsu@med.hokudai.ac.jp
Kondo
Satoshi
S
Okushiba
Shunichi
S
Morikawa
Toshiaki
T
Katoh
Hiroyuki
H
D002116:Calcitonin / Q000378:metabolism*; D005260:Female; D006801:Humans; D006934:Hypercalcemia / Q000209:etiology; D008875:Middle Aged; D010190:Pancreatic Neoplasms / Q000150:complications* / Q000473:pathology; D014660:Vasoactive Intestinal Peptide / Q000032:analysis; D003969:Vipoma / Q000209:etiology* / Q000473:pathology
D014660:Vasoactive Intestinal Peptide; D002116:Calcitonin