Kmm. Shakir et al., PSEUDOCARCINOID SYNDROME-ASSOCIATED WITH HYPOGONADISM AND RESPONSE TOTESTOSTERONE THERAPY, Mayo Clinic proceedings, 71(12), 1996, pp. 1145-1149
Objective: To characterize a disorder of episodes of flushing and incr
eased levels of 5-hydroxyindoleacetic acid (5-HIAA) in men with second
ary hypogonadism who respond to testosterone therapy, Material and Met
hods: We present detailed case reports of three male patients who had
flushing, secondary hypogonadism, and increased urinary 5-HIAA levels
and describe their clinical and laboratory features before and after t
reatment with testosterone, In addition, six male patients with hypogo
nadism (three with primary and three with secondary hypogonadism) with
out flushing were assessed, Results: The three patients with flushing
and secondary hypogonadism (serum total testosterone 5.45 +/- 0.63 nmo
l/L, free testosterone 89.3 +/- 7.0 pmol/L, follicle-stimulating hormo
ne 3.85 +/- 0.58 IU/L, and luteinizing hormone 4.41 +/- 0.98 IU/L) had
increased urinary 5-HIAA levels (98.5 +/- 12.2 mu mol/24 h) but norma
l blood serotonin levels (9.66 +/- 1.58 mu mol/L). During a pentagastr
in-calcium stimulation test, serum calcitonin and blood serotonin valu
es were normal in patients with secondary hypogonadism and flushing, D
etailed investigation showed no evidence of a carcinoid tumor, Urinary
5-HIAA levels became normal (16.6 +/- 1.73 mu mol/24 h) after treatme
nt with testosterone, When testosterone therapy was discontinued in tw
o patients, flushing and increased urinary 5-HIAA levels recurred, Fur
thermore, flushing and the elevated urinary 5-HIAA values resolved whe
n testosterone treatment was reinitiated, The six patients with hypogo
nadism without flushing had normal urinary 5-HIAA levels (14.9 +/- 3.3
1 mu mol/24 h), Conclusion: Male patients with flushing and increased
urinary 5-HIAA levels should undergo assessment for hypogonadism after
screening for carcinoid tumor, If hypogonadism is diagnosed, resoluti
on of flushing and normalization of 5-HIAA may be achieved with testos
terone treatment, We suggest that pseudocarcinoid syndrome associated
with hypogonadism be the descriptive label used for this combination o
f clinical features.