Familial cases represent 5% of PitNEts, which are increasingly recognized as clinicians become more acquainted with familial syndromes, such as familial isolated pituitary adenomas (FIPA), multiple endocrine neoplasia types 1 and 4, X-linked acrogigantism, Carney complex, 3PAs, DICER1, and CABLES1 [13]. This evidence concerns the gene DICER1 and familial isolated pituitary adenoma.