Insular thyroid carcinoma in a patient with Cowden syndrome

Cowden syndrome is an autosomal dominant disorder characterized by benign and malignant hamartomatous lesions that can develop from all three germ cell derivatives. This disorder predisposes patients to develop malignant tumors of the breast, endometrium, and thyroid. We present a patient with clinically relevant manifestations of Cowden syndrome, with genetic verification, impacting by way of airway compromise due to hamartomas, urinary tract abnormalities, and insular thyroid cancer. This case illustrates the value of recognizing Cowden syndrome at an earlier stage when the patient could have received appropriate management to decrease the morbidity of untreated hamartomatous growths, and an elective thyroidectomy would have been a viable option to manage his malignancy. Through this case report, we provide further insight into management of this disorder.