Abstract

BackgroundCarney complex (CNC) is a very rare disease. Although thyroid lesions are included in the diagnostic criteria for CNC, they are an infrequent occurrence.Case presentationThe patient was a 69-year-old woman who had undergone the removal of a left atrial myxoma 10 years earlier, at the age of 59. At the time of the operation, thyroid ultrasonography (US) revealed multiple hypoechoic nodules. Thyroid scintigraphy revealed an increased uptake of 99mTc in these lesions, which was consistent with toxic multinodular goiter, and she was diagnosed with CNC. Genetic studies showed no mutation in the PRKAR1A (protein kinase A regulatory subunit 1-α) gene. From then on, she received annual brain magnetic resonance imaging (MRI), abdominal computed tomography (CT), and thyroid US examinations. Her follicular thyroid nodules gradually increased in number and size. Although aspiration cytology samples from the thyroid nodules diagnosed them as class III, thyroid cancer could not be ruled out. The patient underwent a partial thyroidectomy, and the pathological diagnosis was multiple follicular adenomas.ConclusionCareful and frequent evaluation of the thyroid gland should be required for CNC patients due to the potential for carcinoma to develop in the context of a variety of follicular thyroid lesions.

Highlights

  • Carney complex (CNC) is a very rare disease

  • CNC is a very rare disease, with about 750 cases worldwide and only 33 cases in Japan having been reported to date

  • Up to 60% of CNC patients had thyroid nodules; nonspecific cystic disease was present in 75%, follicular adenomas in 25%, and papillary or follicular carcinomas in up to 10% of the cases [5]

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Summary

Background

Carney complex (CNC) is a familial tumor syndrome first reported by J. The patient was in a good nutritional state and in no Hattori et al Surgical Case Reports (2018) 4:34. She had been operated on 10 years earlier to extirpate a left atrial myxoma (Fig. 1). Thyroid scintigraphy revealed an increased focal uptake of 99mTc in the same lesions in the left lobe, being consistent with toxic multinodular goiter. These corresponded to the criteria of CNC, and she was diagnosed with CNC. The surface was rugged and slightly hard It included multiple nodules with a maximum size of 4.0 cm. The patient was discharged on the 7th day after surgery following an uneventful postoperative course and has been well ever since

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