Syndromic and Familial Acromegaly

Acromegaly can be a manifestation of a syndromic or familial disease. The McCune-Albright syndrome reunites the triad polyostotic osteodysplasia, café-au-lait skin spots, and endocrinopathies, particularly peripheral precocious puberty. Manifestations of Carney complex are skin-pigmented peri-orificial spots, cardiac myxoma, and primary pigmented nodular adrenal disease. Acromegaly can also be part of multiple endocrine neoplasia-type 1 (MEN1) with an association with endocrine tumors of the parathyroids and pancreas tumors principally. X-linked acrogigantism (X-LAG) is responsible for an early gigantism with a median age of onset of 12 months in boys. Ectopic growth hormone-releasing hormone (GHRH) secretion from a neuroendocrine bronchial or pancreatic is another rare etiology of acromegaly. The MRI feature is that of a characteristic T2W hypointense pituitary hyperplasia.