Prolactin secretion abnormalities in patients with the "syndrome of spottyskin pigmentation, myxomas, endocrine overactivity and schwannomas" (Carney complex)

Carney complex is a multiple neoplasia and lentiginosis syndrome. Acromegal y due to growth hormone (GH)-producing adenomas has been considered the onl y pituitary-related manifestation of the complex. In the present study, sev en patients with Carney complex, who belonged to three unrelated kindreds a nd had relatives with acromegaly, were investigated for the presence of GH and prolactin (PRL) secretion abnormalities (familial cases). In addition, four patients with the complex and no family history of the complex were st udied (sporadic cases). Seven of the patients were female and four were mal e; their mean age was 24.27 +/- 4.34 years, Sampling every 20 min for one h our in the morning and under fasting and unstimulated conditions was perfor med in all patients, for determination of serum PRL levels. Insulin-like gr owth factor-I (IGF-I) levels and magnetic resonance imaging (MRI) of the pi tuitary were also obtained. Patients who had elevated IGF-I levels underwen t investigation for acromegaly by oral glucose tolerance test and thyrotrop in-releasing hormone. Seven of the 11 patients (63.6%) had moderately high PRL levels (mean Z score value 2.12 +/- 0.66); three of these patients also had elevated IGF-I levels but a negative investigation for acromegaly, All patients had negative pituitary MRI, The patient with the highest PRL leve ls underwent 24-hour sampling every 20 min, which demonstrated a higher bas eline secretion of this hormone with preservation of its circadian pulsatil ity, Hyperprolactinemia was present in two siblings and a parent-child pair . We conclude that prolactin hypersecretion, albeit mild, appears to be fre quent in patients with Carney complex, is inherited in an autosomal dominan t manner in kindreds with the syndrome, and may be associated with elevated IGF-I levels in some, but not all, patients.