Neurosurgical implications of Carney complex

Object. The authors present their neurosurgical experience with Carney comp lex. Carney complex, characterized by spotty skin pigmentation, cardiac myx omas, primary pigmented nodular adrenocortical disease, pituitary tumors, a nd nerve sheath tumors (NSTs), is a recently described. rare, autosomal-dom inant familial syndrome that is relatively unknown to neurosurgeons. Neuros urgery is required to treat pituitary adenomas and a rare NST, the psammoma tous melanotic schwannoma (PMS), in patients with Carney complex. Gushing's syndrome, a common component of the complex, is caused by primary pigmente d nodular adrenocortical disease and is not secondary to an adrenocorticotr opic hormone-secreting pituitary adenoma. Methods. The authors reviewed 14 cases of Carney complex, five from the lit erature and nine from their own experience. Of the 14 pituitary adenomas re cognized in association with Carney complex, 12 developed growth hormone (G H) hypersecretion (producing gigantism in two patients and acromegaly in 10 ), and results of immunohistochemical studies in one of the other two were positive for GH. The association of PMSs with Carney complex was establishe d in 1990. Of the reported tumors. 28% were associated with spinal nerve sh eaths. The spinal tumors occurred in adults (mean age 32 years, range 18-49 years) who presented with pain and radiculopathy. These NSTs may be malign ant (10%) and, as with the cardiac myxomas, are associated with significant rates of morbidity and mortality. Conclusions. Because of the surgical comorbidity associated with cardiac my xoma and/or Gushing's syndrome, recognition of Carney complex has important implications for perisurgical patient management and family screening. Stu dy of the genetics of Carney complex and of the biological abnormalities as sociated with the tumors may provide insight into the general pathobiologic al abnormalities associated with the tumors may provide insight into the ge neral pathobiological features of pituitary adenomas and NSTs.