A Rare Case of McCune-Albright Syndrome in Association with Acromegaly: Treatment Options and a Review of the Literature

Abstract

McCune-Albright syndrome constitutes of fibrous dysplasia, café-au-lait macules, and various endocrinopathies such as thyroid, cortisol, prolactin and growth hormone hypersecretion. Growth hormone excess as a manifestation of endocrine hyperfunction is uncommon. We present a case of McCune-Albright syndrome with compressive optic neuropathy due to fibrous dysplasia and acromegaly which was resistant to different therapies. A 33-year-old male patient was admitted to our center due to loss of vision and a progressively growing mass in the right parietal region. Magnetic resonance image yielded a contrast enhanced lesion, measuring 10x15 cm in size. The patient underwent decompression surgery due to optic nerve and chiasm compression and the histopathologic evaluation was compatible with fibrous dysplasia. The patient was diagnosed with acromegaly after laboratory evaluation. Surgical treatment was not preferred. Medical therapy with octreotide LAR 10 mg once a month was recommended. Cabergoline was added to his therapy and the doses of these two medicines were gradually escalated. Pegvisomant therapy was planned because IGF-1 level was not normalized. As a rarely encountered syndrome, it is important to recognize this syndrome and evaluate for a wide range of endocrinopathies. There are various treatment options and further research is still warranted.

Keywords

• acromegaly
• cabergoline
• fibrous dysplasia
• mccune-albright syndrome
• pegvisomant
• somatostatin receptor analogues