Surgical Management of Nonmultiple Endocrine Neoplasia Endocrinopathies: State-of-the-Art Review

The development of genetic testing has given patients with familial endocrine diseases the opportunity to be identified earlier in life. The importance of this technological advancement cannot be underestimated, as some of these heritable diseases have significant potential for malignancy. This article focuses on the identification and surgical management of familial endocrinopathies of the thyroid, parathyroid, adrenal glands, and pancreas. Familial endocrinopathies discussed include hereditary nonmedullary carcinoma of the thyroid, Cowden disease, familial adenomatous polyposis, Carney complex, Werner syndrome, familial medullary thyroid carcinoma, Pendred syndrome, hereditary hyperparathyroidism jaw-tumor syndrome, familial isolated hyperparathyroidism, Beckwith- Wiedemann syndrome, Li-Fraumeni syndrome, neurofibromatosis I, von Hippel-Lindau disease, and tuberous sclerosis.