11/52. achlorhydria, parietal cell hyperplasia, and multiple gastric carcinoids: a new disorder. We describe a 54-year-old woman who had multiple gastric carcinoid tumors arising in the setting of marked hypergastrinemia associated with a lack of acid production by hypertrophic parietal cells. The serum gastrin level was 1,400 pg/mL, and investigation revealed no evidence for either of the recognized causes for hypergastrinemia-associated carcinoids, autoimmune gastritis, and Zollinger-Ellision syndrome. Partial gastrectomy was performed. Pathologic examination showed multiple intramucosal and invasive carcinoid tumors of the body and fundus in a background of marked ECL cell hyperplasia. There were no gastric or duodenal ulcerations. One perigastric lymph node was metastatically involved. The oxyntic mucosa showed marked hyperplasia and hypertrophy of the parietal cells. Some of these cells were vacuolated, and many displayed protrusions of apical cytoplasm into dilated oxyntic glands filled with inspissated eosinophilic material. Similar findings have occurred in 1 other patient, strongly indicating that the clinicopathologic alterations in the 2 cases are not random but, on the contrary, represent a very rare disorder of gastric carcinoids associated with an intrinsic acid secretion abnormality of the parietal cells. ( info) |
12/52. Hypokalemic rhabdomyolysis due to WDHA syndrome caused by VIP-producing composite pheochromocytoma: a case in neurofibromatosis type 1. A 47-year-old woman with neurofibromatosis type 1 suffered from general muscle weakness and watery diarrhea. Laboratory findings showed elevated muscular enzymes, severe hypokalemia and excessive production of catecholamines and vasoactive intestinal polypeptide (VIP). A computed tomography scan showed a 10 cm left adrenal mass, in which [(131)I]-metaiodobenzylguanidine scintigraphy showed high uptake. After she underwent surgical removal of the tumor, all the symptoms and signs subsided. A histological study revealed that the mass consisted of pheochromocytoma and ganglioneuroma, respectively producing catecholamines and VIP. In immunohistochemical staining of neurofibromin, pheochromocytoma and ganglion cells showed positive staining, whereas the staining was negative for nerve bundles and schwann cells. We concluded that the patient had hypokalemic rhabdomyolysis due to watery diarrhea, hypokalemia and achlorhydria (WDHA) syndrome, which was induced by a VIP-producing composite pheochromocytoma. Composite pheochromocytoma is a neuroendocrine tumor that is composed of pheochromocytoma and ganglioneuroma, both derived from the neural crest. Deficiency of neurofibromin in schwann cells might have played an important role in the development and the growth of the composite pheochromocytoma in this patient. ( info) |
| Spontaneous remission of gastric acid hypersecretion in the zollinger-ellison syndrome occurs rarely. This study shows the development of gastric secretory mucosal atrophy resulting in achlorhydria and loss of pepsin secretion in a 63-year-old woman with the zollinger-ellison syndrome. Reduced secretion began soon after starting treatment with omeprazole, and achlorhydria became complete 6 months later. The patient remains well with normal endoscopy results and is achlorhydric 4 years after the start of treatment and 34 months after stopping omeprazole. She was not colonized with helicobacter pylori until 36 months after developing achlorhydria. serum gastrin has increased from 1000 to between 5000 and 12,500 ng/L (pg/mL), was not suppressible by gastric acidification, and was not associated with G-cell hyperplasia. She also has a normal schilling test and normal immunoglobulins, and lacks antibodies to parietal cells or H , K( )-ATPase. Moderate enterochromaffinlike cell hyperplasia is apparent for the first time on the latest biopsy sample. ( info) |
14/52. Radiologic and pathologic characteristics of the WDHA syndrome. A case of a non-beta islet cell tumor of the pancreas that produced the WDHA (watery diarrhea, hypokalemia, and achlorhydria) syndrome is presented. An enlarged body-tail region of the pancreas is demonstrated on transaxial views; multiple fluid-filled loops of small and large bowel are also noted. The angiography of the tumor is similar to other non-beta islet cell lesions consisting of a large hypervascular mass with hypertrophied feeding vessels and a persistent, dense capillary stain. The demonstration of elevated levels of vasoactive intestinal polypeptide in both tumor and plasma and the ultrastructural description of endocrine granules may help to explain the pathophysiology in this case. ( info) |
15/52. Recurrent obstipation as a complication of partial pancreatectomy for non-beta cell adenoma of the pancreas. Verner and Morrison, in 1958, reported non-insulin-secreting tumours of the pancreas that were associated with a syndrome of refractory diarrhea, achlorhydria and hypokalemia. Surgical resection of such tumours results in rebound acid hypersecretion and cessation of the watery diarrhea. The authors report the case of an 84-year-old man who had three of the four major criteria for diagnosis of the Verner Morrison syndrome. hypokalemia was absent, but this was possibly due to the large doses of potassium chloride that he was taking in conjunction with diuretics. After resection of the tumour severe obstipation with resultant bowel obstruction developed in addition to rebound hypersecretion and relief of watery diarrhea. Treatment, consisting of bulk laxatives in appropriate amounts, alleviated the obstipation. ( info) |
16/52. Multiple gastric carcinoid tumours associated with atrophic gastritis. A case of multiple gastric carcinoid tumours is described in an elderly man with atrophic gastritis and hypergastrinaemia. This case provides further evidence of an association between hypochlorrhydric states and some gastric endocrine tumours. ( info) |
17/52. Benign gastric ulcers and pernicious anemia. The formation of a benign gastric ulcer in an achlorhydric milieu is a rare phenomenon. Since 1971, only 12 cases have been reported. Early cases were treated surgically for fear of gastric cancer, but since that time such ulcers have been successfully treated medically. The authors describe the case of a patient who had no concomitant ulcerogenic factors and whose ulcer occurred in the gastric cardia at the esophagogastric junction. We believe this is the first case report of a patient with pernicious anemia meeting these conditions. ( info) |
| This report describes the clinicopathologic features of a 55-yr-old man found to have a bleeding, postbulbar duodenal ulcer and fasting hypergastrinemia. Gastric analysis revealed pentagastrin-fast achlorhydria. Healing of the ulcer was documented 8 wk after vagotomy, antrectomy, gastrojejunostomy, and a course of sucralfate therapy. The etiology of the postbulbar ulcer was uncertain. This is the first documented case of a duodenal ulcer with pentagastrin-fast achlorhydria. ( info) |
19/52. Multifocal gastric carcinoid tumours, achlorhydria, and hypergastrinaemia. Multiple polypoidal carcinoid tumours of the stomach were found in 5 patients with achlorhydria (4 of whom had pernicious anaemia) as a result of autoimmune atrophic gastritis. The tumours were small (nearly all less than 1 cm diameter) and appeared to grow very slowly, if at all; no significant enlargement or complications were seen during periods of observation of up to 6 years. No extragastric hormonal syndromes were identified. They differed from the carcinoid tumours usually found in the intestinal tract by being composed of argyrophil (not argentaffin) cells of the enterochromaffin-like (ECL) type. fasting plasma levels of gastrin, which is believed to be trophic to ECL cells, were very high in all patients. Thus, chronic hyperplasia of gastric ECL cells (as a result of hypergastrinaemia) may have been responsible for development of the tumours. Long-term, uninterrupted achlorhydria produced by potent inhibitors of gastric acid secretion might therefore predispose to carcinoid tumours of the stomach. ( info) |
| An 8-week-old infant presented with vomiting and failure to thrive due to small bowel obstruction caused by a diffusely enlarged pancreas. Surgical bypass of the obstruction was followed by secretory diarrhea, hypokalemia, and dehydration. plasma vasoactive intestinal peptide (VIP) (823pg/ml), pancreatic polypeptide (4,500 pg/ml), and neurotensin (680 pg/ml) concentrations were markedly elevated. No neoplastic process was identified. Therapy with the long-acting somatostatin analogue SMS 201-995 was followed by decline in VIP concentrations (900 to 200-300 pg/ml), decrease in stool frequency, and normalization of serum electrolytes. During 12 months of somatostatin analogue therapy, length and weight progressed along the 3rd percentile on the Tanner growth chart. ( info) |