Wermer Syndrome

Neoplastic disease, characterized by tumors or hyperplasia of the parathyroid and pituitary glands and the islands of Langerhans, with increased incidence of adrenocortical and thyroid disease. Association with diffuse neuroendocrine tumors in the thymus, bronchi, and duodenopancreas.

Multiple Endocrine Neoplasia type I, MEN I.

Genetic disorder first reported by Paul Wermer, American internist in 1954, and in 1955 by Robert Milton Zollinger and Edwin Homer Ellison, American surgeons.

Both sexes equally affected. It is rare in childhood. Prevalence in general population ranges between 1:20,000 and 1:40,000.

Autosomal dominant. More than 80% of cases caused by inactivating mutations (including nonsense mutations, deletions and insertions) of the MEN1 gene, MENIN; localized to chromosome 11q13.

Precise role of MENIN is unclear. It is likely to be a tumor-suppressor gene. Mutations lead to hyperplasia of the endocrine organs: pancreatic islet cell adenoma (40% of cases, most commonly gastrinomas and insulinomas), parathyroid adenoma (95% of cases), pituitary adenoma (30% of cases), adrenocortical adenoma, prolactinoma, glucagonoma, insulinoma, and vasointestinal peptide tumor. Lesions of nonendocrine organs may also be present: bronchial carcinoma and carcinoids, thymomas and thymic carcinoid, duodenal carcinoid, malignant schwannoma, ovarian tumors, and lipomas.

Clinical features and the results of biochemical tests according to the presentation (hypoglycemia, hypercalcemia, hyperphosphatemia, anemia, relevant endocrine abnormalities). Also, the addition of radiological information (delineation and localization of peptic ulcers, pituitary tumor, renal stones, bronchial tumors, etc.) is essential.

Symptoms and signs associated with the glands involved and the function of adenomas; gastrinoma (intractable peptic ulcer, high incidence of bleeding, perforation and obstruction, ectopic ulcers in esophagus and small intestines, diarrhea, steatorrhea, weight loss); pituitary adenoma (headache, visual field defects; acromegaly; hyperthyroidism; amenorrhea); parathyroid adenoma (hypercalcemia and renal stones); adrenal (Cushing syndrome); insulinoma (hypoglycemia); glucagonoma (hyperglycemia, stomatitis, skin rash); islet cell tumors (can produce glucagon, vasoactive inhibitory polypeptide, prostaglandins, adrenocorticotropic hormone, parathyroid hormone, antidiuretic hormone, serotonin); multiple lipomas; bronchial tumors (cough, hemoptysis, breathlessness).

Acid-base balance and electrolyte disturbances must be corrected preoperatively to avoid arrhythmia, hemodynamic instability, and potentiation of action of neuromuscular blockers.

Emergency surgery may be indicated for complications of peptic ulcer disease. Presence of hypovolemia necessitates perioperative fluid resuscitation, rapid sequence induction, and pharmacological prophylaxis against gastric aspiration (e.g., sodium citrate). Invasive monitoring may be indicated: close perioperative glucose monitoring for hypoglycemia, anesthetic problems associated with acromegaly, Cushing syndrome, and hyperthyroidism. Depending on size and site of bronchial tumor, expect airway compression and ventilatory difficulties; superior vena cava obstruction may impede venous return.

Renal dysfunction may affect clearance of some drugs.