Gigantism with Pituitary Macroadenoma: An Unusual Variant of McCune-Albright Syndrome

Abstract

Extreme physical size and stature (gigantism) is a rare condition in childhood, and when it is present it is rarely due to growth hormone (GH) excess, but more frequently of genetic etiology (familial tall stature, Klinefelter syndrome, Sotos syndrome, Marfan syndrome, etc.). GH hypersecretion is usually by pituitary adenomas, even though other cases such as somatotroph hyperplasia may be involved. Endocrine gigantism typically occurs as an isolated disorder, but it may also be a feature of some syndromes such as neurofibromatosis, multiple endocrine neoplasia type 1 (MEN1) or McCune-Albright syndrome (MAS). In its classic form, MAS is characterised by precocious puberty, caf\ue8-au-lait skin spots, and polyostotic fibrous dysplasia. An unusual form of this complex, heterogeneous disorder may arise in conjunction with GH excess and/or other endocrinopathies and abnormalities. In the literature there are very few case reports of MAS associated with GH excess that are caused by secreting pituitary adenomas. Most are reported as acromegaly since they are diagnosed in the third decade of life. Paediatric reports are even more uncommon. Herein we describe the case of an 11 year-old boy with GH hypersecretion