<unk>  can present with a sign or symptom related to a tumor or, in the case of<unk> b, with characteristic musculoskeletal and/or lip and/or gastrointestinal findings. Medullary thyroid carcinoma (MTC) represents the most frequent initial diagnosis. Occasionally pheochromocytoma or primary hyperparathyroidism may be the initial diagnosis. Pheochromocytoma occurs in 33-50% of<unk>  cases. In<unk> A, primary hyperparathyroidism occurs in 10–50% of cases and is usually diagnosed after the third decade of life. Rarely, it may present in childhood or be the sole clinical manifestation of this syndrome.MEN2A associates medullary thyroid carcinoma with pheochromocytoma in about 20–50% of cases and with primary hyperparathyroidism in 5–20% of cases.<unk> B associates medullary thyroid carcinoma with pheochromocytoma in 50% of cases, with marfanoid habitus and with mucosal and digestive neurofibromatosis. In familial isolated medullary thyroid carcinoma the other components of the disease are absent. In a review of 85 patients 70 had<unk> A and 15 had<unk> B. The initial manifestation of<unk>  was medullary thyroid carcinoma in 60% of patients, medullary thyroid carcinoma synchronous with pheochromocytoma in 34% and pheochromocytoma alone in 6%. 72% had bilateral pheochromocytomas.
Multiple endocrine neoplasia type 2
Multiple endocrine neoplasia type 1
Autoimmune polyendocrine syndrome type 1
Autoimmune polyendocrine syndrome type 2
Autoimmune polyendocrine syndrome type 3
McCune–Albright syndrome
Lightwood–Albright syndrome
Late onset congenital adrenal hyperplasia