The clinical features of 20 patients from five families with
multiple endocrine neoplasia syndrome type I (MEN-I) were studied. Nineteen patients (95%) had
hyperparathyroidism. Five patients who had a diagnosis during surgery of
adenoma and who had fewer than 3.5 glands removed had recurrence of
hypercalcemia after surgery. Fourteen patients (70%) had
pancreatic islet cell tumors. All had one or more elevated serum
polypeptide hormones, and six had symptoms related to the
hormones produced. Multiple pancreatic
tumors were identified in the nine patients who underwent surgery. Three patients who died had a mean survival of 6.3 +/- 2.9 years. Eight patients had
pituitary tumors; seven had macroadenomas. Of the eight patients with
pituitary tumors, seven had high serum
prolactin and responded to
bromocriptine therapy, whereas the eighth patient had
acromegaly treated with
radiotherapy. It was concluded that
hypercalcemia due to
hyperparathyroidism in MEN-I syndrome patients should be managed by a resection of four glands and
transplantation of one half gland into the forearm because none of the patients has shown evidence of a recurrence, and serum
calcium levels have been normal. Pancreatic
tumors, which are usually multiple, may be asymptomatic. Patients with these
tumors usually have long survival rates, even with distant
metastasis. Total
pancreatectomy may be the method of choice, especially in patients with
gastrinoma caused by the diffuse nature of the disease. Long-term follow-up is needed, however, with more patients.
Pituitary tumors are primarily
prolactin-producing
tumors, and medical treatment is the method of choice.