title: Gigantism treated by pure endoscopic endonasal approach in a case of McCune-Albright syndrome with sphenoid fibrous dysplasia: a case report.
abstract: McCune-Albright syndrome (MAS) is an uncommon polyostotic manifestation of fibrous dysplasia in association with at least one endocrinopathy that is mostly associated with precocious puberty and hyperpigmented skin macules named café-au-lait spots. We present an atypical manifestation of McCune-Albright syndrome in a 19-year-old man with the uncommon association of polyostotic fibrous dysplasia and gigantism in the absence of café-au-lait spots and precocious puberty. He presented with a height increase to 202 cm in the previous 3 years, which had become more progressive in the few months prior. Physical examination revealed only a mild facial asymmetry; however, a computed tomography (CT) scan discovered vast areas of voluminous bones with ground-glass density and thickening involving the craniofacial bones and skull base. Magnetic resonance imaging (MRI) found a right stalk shift of the pituitary with a 20 mm pituitary adenoma. We describe the diagnostic and endoscopic endonasal transsphenoidal approach for excision of the tumor.
authors: Department of Neurosurgery, Loghman Hakim Hospital, Shaheed Beheshti University of Medical Sciences, Tehran, Islamic Republic of Iran.
authors: Guive
authors: G
authors: Sharifi
authors: Endoscopic Pituitary and Skull Base Surgery Unit, ENT Head and Neck Research Center and Department, Hazrat Rasoul Akram Hospital, Tehran University of Medical Sciences, Tehran, Islamic Republic of Iran.
authors: Maryam
authors: M
authors: Jalessi
authors: Research Institute for Endocrine Sciences, Taleghani Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Islamic Republic of Iran.
authors: Farzaneh
authors: F
authors: Sarvghadi
authors: Endoscopic Pituitary and Skull Base Surgery Unit, ENT Head and Neck Research Center and Department, Hazrat Rasoul Akram Hospital, Tehran University of Medical Sciences, Tehran, Islamic Republic of Iran.
authors: Mohammad
authors: M
authors: Farhadi
mesh_terms: D000236:Adenoma / Q000601:surgery; D050071:Bone Density Conservation Agents / Q000627:therapeutic use; D004724:Endoscopy; D005357:Fibrous Dysplasia of Bone / Q000150:complications* / Q000473:pathology; D005359:Fibrous Dysplasia, Polyostotic / Q000150:complications*; D005877:Gigantism / Q000209:etiology* / Q000601:surgery*; D049912:Growth Hormone-Secreting Pituitary Adenoma / Q000601:surgery; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009296:Nasal Cavity / Q000601:surgery*; D015282:Octreotide / Q000627:therapeutic use; D010024:Osteoporosis / Q000188:drug therapy; D013100:Sphenoid Bone / Q000473:pathology*; D025321:Surgery, Computer-Assisted; D055815:Young Adult
chemical_list: D050071:Bone Density Conservation Agents; D015282:Octreotide