Introduction: McCune-Albright Syndrome constitiutes of fibrous dysplasia, café-au-lait macules, and various endocrinopaties 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.
Case Representation: A 33 year old male patient was admitted to our centre due to loss of vision anda progressively growing mass in 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 optic 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 commnenced. Cabergoline added to his therapy and the doses of these two medicines was gradually escalated. Pegvisomant therapy was planned because IGF-1 level was not normalized.
Conclusion: 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.
Key words: fibrous dysplasia, acromegaly, McCune-Albright Syndrome, surgery, somatostatin receptor analogues, cabergoline, pegvisomant
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