Pseudocarcinoid Syndrome Associated With Hypogonadism and Response to Testosterone Therapy

To characterize a disorder of episodes of flushing and increased levels of 5-hydroxy-indoleacetic acid (5-HIAA) in men with secondary hypogonadism who respond to testosterone therapy. 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 treatment with testosterone. In addition, six male patients with hypogonadism (three with primary and three with secondary hypogonadism) without flushing were assessed. The three patients with flushing and secondary hypogonadism (serum total testosterone 5.45 ± 0.63 nmol/L, free testosterone 89.3 ± 7.0 pmol/L, follicle-stimulating hormone 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 (imol/24 h) but normal blood serotonin levels (9.66 + 1.58 (imoI/L). During a pentagastrin-calcium stimulation test, serum calcitonin and blood serotonin values were normal in patients with secondary hypogonadism and flushing. Detailed investigation showed no evidence of a carcinoid tumor. Urinary 5-HIAA levels became normal (16.6 ± 1.73 umol/24 h) after treatment with testosterone. When testosterone therapy was discontinued in two patients, flushing and increased urinary 5-HIAA levels recurred. Furthermore, flushing and the elevated urinary 5-HIAA values resolved when testosterone treatment was reinitiated. The six patients with hypogonadism without flushing had normal urinary 5-HIAA levels (14.9 ±3.31 umol/24 h). Male patients with flushing and increased urinary 5-HIAA levels should undergo assessment for hypogonadism after screening for carcinoid tumor. If hypogonadism is diagnosed, resolution of flushing and normalization of 5-HIAA may be achieved with testosterone treatment. We suggest that pseudocarcinoid syndrome associated with hypogonadism be the descriptive label used for this combination of clinical features.