Watery diarrhea, hypokalemia and achlorhydria (WDHA) syndrome caused by vasoactive intestinal polypeptide (VIP) -producing tumor only rarely occurs in patients with nonpancreatic disease. A 49-year-old woman was referred for evaluation of a right adrenal tumor incidentally diagnosed by abdominal ultrasound during the investigation of chronic watery diarrhea. Laboratory findings showed hypokalemia and excessive production of VIP and catecholamines. After surgical resection of the tumor, diarrhea subsided and both electrolytes and affected hormone levels normalized. Immunohistochemical examination confirmed a diagnosis of pheochromocytoma, which contained VIP-positive ganglion-like cells. We herein present the clinical and histogenetic implications of this rare clinical entity, with literature review.
Mucinous cystic neoplasms (MCNs) of the pancreas occur mostly in females and are potentially sex hormone-sensitive. However, a MCN occurring during pregnancy is quite rare. A 30-year-old woman in the tenth week of pregnancy was referred to us because of a rapid increase in left hypochondrial distending pain. On ultrasound, the patient had a large intra-abdominal cystic lesion. She was thereafter diagnosed with missed abortion and a computed tomography scan showed that the lesion was a cystic tumor 18 cm in diameter originating from the pancreatic tail. The patient subsequently underwent tumor resection with distal pancreatectomy, sparing the spleen. Histopathological analysis of the specimen revealed a pancreatic MCN with moderate dysplasia. Immunohistochemically, the tumor was positive for both estrogen and progesterone receptors. To our knowledge, this is the first reported case of pancreatic MCN with moderate dysplasia in association with pregnancy. Our case strongly indicates that pancreatic MCN is female-hormone dependent.
Although several studies have reported that splenectomy increases susceptibility to bacterial infections, other reports have indicated that splenectomy does not induce such susceptibility. To clarify this discrepancy, we studied the effects of splenectomy in the models of lipopolysaccharide (LPS) or Escherichia coli challenge and intestinal obstruction, focusing on cytokine production and bacterial clearance in the liver. Using C57BL/6 mice at 4 weeks after splenectomy or a sham operation, either LPS or E. coli was injected or an intestinal obstruction was made to examine the mortality, serum cytokine levels, cytokine production of the liver mononuclear cells (MNCs), and bacterial clearance in the liver. As a result, no differences were observed in survival rates after LPS or E. coli challenge between the mice with and without splenectomy. However, in a model of intestinal obstruction, splenectomized mice survived significantly longer than the sham-operated mice. Liver MNCs from splenectomized mice produced a significantly larger amount of interferon-gamma compared with those from sham-operated mice. Furthermore, bacterial counts in the liver at 2 h after E. coli injection and at 24 h after intestinal obstruction were significantly decreased in splenectomized mice compared with sham-operated mice. In conclusion, splenectomy does not impair host defense against bacteria infection provided that recovery is sufficient to allow compensatory processes in the liver to occur.
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