J Korean Soc Endocrinol.  1998 Mar;13(1):108-114.

A Case of McCunt-Albright Syndrome Associated with Acremegaly and Fibrous Dysplasia

• Kim JG
• Ha SW
• Chung SW
• Koo SM
• Lee JT
• Lee KB
• Kim YS
• Kwon S
• Kim BW

Abstract

Acromegaly and hyperprolactinemia have been described in association with polyostotic fibrous dysplasia. The pathogenic mechanisms of this endocrinopathy are not clear. We experienced a 19-year-old male with hypersecretion of GH, hyperprolactinemia and fibrous dysplasia. He was referred for evaluation of suspected acromegaly. He had no skin pigmentation. Plasma GH, PRL, somatomedin-C, LH, FSH, testosterone, estradiol, progesterone, T3, T4, TSH and cortisol were measured. Among those, the levels of plasma GH, PRL and somatomedin-C were high. Serum alkaline phosphatase was increased. OGTT did not suppress plasma OH concentration and GH showed paradoxical response to TRH and LHRH. GH was suppressed after a test-dose of somatastatin and bromocriptine. Brain MRI demonstrated a mass lesion in sella turcica and another mass lesions in nasal cavity and posterior occipital bone. Whole body bone scan revealed increased uptake in skull, nasal bone, both 9th posterior rib, both femurs, both tibias, left scapular and pelvic bone. These fmdings were consistent with bone tumor such as fibrous dysplasia. We report a case with incomplete MeCune-Albright syndrome including acromegaly, hyperprolactinemia and polyostotic fibrous dysplasia.

MeSH Terms

Acromegaly
Alkaline Phosphatase
Brain
Bromocriptine
Estradiol
Femur
Fibrous Dysplasia, Polyostotic
Glucose Tolerance Test
Gonadotropin-Releasing Hormone
Humans
Hydrocortisone
Hyperprolactinemia
Magnetic Resonance Imaging
Male
Nasal Bone
Nasal Cavity
Occipital Bone
Pelvic Bones
Plasma
Progesterone
Ribs
Sella Turcica
Skin Pigmentation
Skull
Testosterone
Tibia
Young Adult
Alkaline Phosphatase
Bromocriptine
Estradiol
Gonadotropin-Releasing Hormone
Hydrocortisone
Progesterone
Testosterone