OBJECTIVE: The aim of the present study was to examine the clinical an
d pathological features of pituitary disease in patients with multiple
endocrine neoplasia Type I (MEN I) and to assess the prognosis. METHO
DS: Fifty-two patients with pituitary disease and MEN I were studied r
etrospectively. Medical records were reviewed, and all of the patients
known to be alive were sent a questionnaire to ascertain current dise
ase status. RESULTS: In 12 patients, pituitary disease was the initial
manifestation of MEN I. The most common lesion was prolactinoma, foll
owed, in frequency, by acromegaly and nonsecretory adenoma. Thirty-fou
r of the patients had surgical treatment at the Mayo Clinic, Rochester
, MN, as primary treatment, 3 had radiotherapy, and 12 received no spe
cific therapy. Twelve patients had adjunctive radiotherapy postoperati
vely. Of the 34 patients receiving surgical treatment, 33 had adenoma
and 1 had adenoma and pituitary hyperplasia. Immunocytochemical examin
ation demonstrated that many tumors showed reactivity for more than on
e pituitary hormone. On survival analysis, no excess pituitary-related
mortality was found, either in the surgically treated group or in the
group as a whole. CONCLUSION: On the basis of this study, we conclude
that pituitary disease is frequently the initial manifestation of MEN
I; that adenomas, particularly prolactinomas, are the rule and hyperp
lasia is rare; that a significant proportion of tumors are plurihormon
al; and that excess pituitary-related mortality is not a factor in pat
ients with MEN I.