Rv. Benya et al., ZOLLINGER-ELLISON SYNDROME CAN BE THE INITIAL ENDOCRINE MANIFESTATIONIN PATIENTS WITH MULTIPLE ENDOCRINE NEOPLASIA-TYPE-I, The American journal of medicine, 97(5), 1994, pp. 436-444
PURPOSE: To determine whether patients with multiple endocrine neoplas
ia type I (MEN-I) can initially present with Zollinger-Ellison syndrom
e (ZES), and to learn whether ZES exhibits any distinguishing features
when it occurs as a first manifestation of MEN-I. PATIENTS AND METHOD
S: Sixty patients who had been referred to a clinical research center
with ZES were examined by cohort analysis. Twenty-eight had MEN-I and
32 did not. In patients with MEN-I, we analyzed the temporal relations
hips between the clinical and biochemical manifestations of ZES and th
e other endocrinopathies associated with the neoplasia. To determine w
hether patients who had ZES as a first manifestation of MEN-I (n = 8)
had any distinguishing clinical characteristics, we compared them to a
cohort of patients with established sporadic ZES (n = 32) matched for
age, sex, and time since the onset of symptoms consistent with ZES. R
ESULTS: Of the 28 patients with ZES and MEN-I, 11 initially presented
with ZES and hyperparathyroidism (HP) and 1 with evidence only for pit
uitary disease. Eight patients (29%) presented with features of ZES an
d developed clinical and biochemical evidence for HP later, while the
same number developed these 2 endocrinopathies in the opposite order.
In whichever order ZES and HP occurred, the time from the diagnosis of
the first to the diagnosis of the second was similar. It ranged from
9 to 177 months in patients who presented with ZES first, and from 12
to 264 months in patients who presented with HP first. At the time of
initial diagnosis, the patients who presented with ZES as a manifestat
ion of MEN-I had no distinguishing ZES-related symptoms, biochemical a
ssays, or tumor imaging results compared to the cohort of patients who
had the syndrome sporadically. CONCLUSION: Patients with MEN-I can in
itially present with a symptomatic pancreatic endocrine tumor syndrome
without any other disease manifestations. In patients with ZES and ME
N-I, up to one third may present with ZES without evidence of any othe
r endocrinopathy. Consequently, patients with presumed sporadic ZES sh
ould undergo continual biochemical screening for other endocrinopathie
s characteristic of MEN-I and, in the future, genetic studies for the
MEN-I gene.