Carney complex with primary pigmented nodular adrenocortical disease and bilateral papillary thyroid carcinoma occurring 11 years apart - A case report

A 41-year-old man came to our outpatient department in October 1990 with a Cushingoid appearance. From October to November 1990, this patient's endocrinologic hormone studies showed adrenal-dependent Cushing syndrome. An adrenal scintigram with I-131-6 beta-iodomethyl-19-norcholesterol showed bilateral adrenal uptake. Abdominal computed tomography (CT) revealed no adrenal abnormality. In June 1991, abdominal magnetic resonance imaging showed a tiny nodule at the lateral limb of the right adrenal gland. Primary pigmented nodular adrenocortical disease (PPNAD) was confirmed pathologically after a right adrenalectomy in October 1991. The abnormally high plasma and urinary cortisol levels persisted subsequent to the right adrenalectomy. Repeat abdominal CT revealed a left adrenal nodule 2.0 cm in diameter in the left renal hilum. Left adrenalectomy was performed in December 1991. The microscopic picture of the left adrenal gland was the same as that of the right adrenal. Cortisol hypersecretion was cured by the second operation. A nodule was palpated in the left lobe of the thyroid gland in February 2002. Ultrasonography of the thyroid gland revealed multiple bilateral hypoechoic nodules. Bilateral papillary thyroid carcinoma (PTC) was diagnosed pathologically showing a bilateral thyroidectomy in March 2002. Metastatic PTC to the lymph nodes of the neck was established after surgical dissection in June 2002. This is a case of Carney complex with PPNAD followed by bilateral, PTC, which occurred 11 years later. Regular long-term follow up is suggested by this case for the early detection of neoplasia in subjects with Carney complex with PPNAD.