Wermer's syndrome. A case report

Wermer's syndrome (or multiple endocrine adenomatosis type I) is a hereditary condition characterized by tumours or hyperplasia involving multiple endocrine organs. Most commonly the pituitary and parathyroid glands and the pancreas are involved. We record a further case, the patient presenting with acromegaly due to a pituitary adenoma, hypercalcaemia due to a parathyroid adenoma and peptic ulceration.     

Late recurrent parathyroid adenoma.

A case history of recurrent hyperparathyroidism 17 years following removal of an adenoma is presented. Reference is made to possible familial implications noted in this instance. The rarity of recurrent hyperparathyroidism is attested to by the fact that less than 20 authenticated cases have been reported to 1974. Arteriography may be greatly helpful in localizing the recurrent or missed tumor. Etiologic factors possibly associated with recurrence are discussed.     

Zollinger-Ellison syndrome associated with parathyroid adenomas and ectopic gastric tissue in the lower esophageal mucosa.

A rare case of the Zollinger-Ellison syndrome associated with hyperparathyroidism and ectopic gastric tissue in the lower esophageal mucosa is reported. Preoperatively the patient, a 53-year-old woman, had hyperchlorhydria and her fasting serum gastrin concentration was mildly elevated. There was a considerable increase in the gastric acid output and concentration of serum calcium after secretin infusion. At operation the patient had a gastric ulcer 10 cm in diameter, an islet cell tumour of the pancreas 14 cm in diameter, and ectopic gastric mucosa in the distal third of the esophagus. A gastrectomy was perfomed, the pancreatic tumour excised and part of the distal esophagus removed through a left thoracotomy. Four months after the operation the gastrin concentration had returned to low normal, but the serum calcium values remained high. One month later two parathyroid adenomas were removed which effectively cured the hypercalcemia.     

Multiple endocrine neoplasia: Part I. Wermer's syndrome.

Multiple endocrine neoplasia (MEN) type I is a genetically inherited disorder which predominantly involves the parathyroid and pituitary glands and pancreatic islets. Symptoms relevant to each endocrine gland may be present individually or in combination, since the tumours or hyperplasia can occur either synchronously or alone. Measurement of plasma hormone levels, endocrine function tests and radiological examination are necessary to make the diagnosis. Resection of the affected glands or treatment with specific pharmacotherapy is required for control of the disease. Long-term follow-up examination is vital to detect the appearance of lesions in other endocrine glands or the development of metastases. Screening of relatives and genetic counselling are important in the management of affected families.     

A large intrathoracic parathyroid adenoma.

A case is described in which an unusually large parathyroid adenoma was visible on the plain chest radiograph taken during the investigation of hypercalcaemia. This was diagnosed preoperatively and a scheme is suggested whereby such a disgnosis can now readily be made. The differential diagnosis is discussed ant the literature is reviewed.     

Zollinger-Ellison syndrome: special considerations.

Twelve patients with Zollinger-Ellison syndrome and one patient with WDHA syndrome are reviewed. Three of the Z-E patients exhibited MEA, two having hyperinsulinism and one hyperparathyroidism. Ages ranged from nine to 71 years. Diagnosis of Z-E syndrome was established from history, gastric acid secretion, radiologic studies, serum gastrin measurements and from actual tissue biopsy in 10 of the 12 patients. Total gastrectomy was performed in 8 of the 12 Z-E patients, with abolition of the ulcer diathesis in all. However, in none of our patients was there objective evidence of subsequent tumor regression. Three patients remain alive. Four died of tumor, one from post-total gastrectomy complications, one from post-subtotal gastrectomy in another hospital, two from ulcer hemorrhage, and one from electrolyte imbalance with autopsy diagnosis of Z-E tumor. A patient is recorded in detail who exhibited both hyperinsulinemia and hypergastrinemia from a malignant islet cell tumor, had the tumor "debulked" four times over a 14 year period and whose hepatic metastases were temporarily abolished by streptozotocin infusion. The question is raised regarding relationships between chronic organic hyperinsulinism and subsequent hypergastrinemia.