J Korean Neurosurg Soc.  2014 Sep;56(3):281-283. 10.3340/jkns.2014.56.3.281.

Endoscopic Decompression for Optic Neuropathy in McCune-Albright Syndrome

• Noh JH
• Kong DS
• Seol HJ
• Shin HJ

• KMID: 2339973
• DOI: http://doi.org/10.3340/jkns.2014.56.3.281

Abstract

McCune-Albright syndrome (MAS) is characterized by a triad of poly/monostotic fibrous dysplasia, cafe-au-lait macules and hyperfunctioning endocrinopathies including human growth hormone excess. Acromegaly as a manifestation of endocrine hyperfunction with MAS is uncommon. Surgical excision may be challenging due to the associated severe fibrous dysplasia of the skull base. Through the endoscopic procedures, we treated a case of MAS presenting with compressive optic neuropathy due to fibrous dysplasia and acromegaly caused by growth hormone secreting pituitary adenoma. We reviewed the literature on GH excess in MAS to highlight its surgical and medical challenges.

Keyword

Fibrous dysplasia; McCune-Albright syndrome; Acromegaly

MeSH Terms

Acromegaly
Decompression*
Fibrous Dysplasia, Polyostotic*
Growth Hormone
Human Growth Hormone
Optic Nerve Diseases*
Pituitary Neoplasms
Skull Base
Growth Hormone
Human Growth Hormone

Figure

• 

  Fig. 1 Ostiometal unit (OMU) CT scan before and after optic nerve decompression. A : The non-contrast OMU CT scan demonstrates an expanded left basiocciput and greater sphenoid wing, typical for fibrous dysplasia. B : After endoscopic transnasal transsphenoidal and transethmoidal approach, postoperative OMU CT shows relief of left optic canal by removal of the fibrous lesion (arrows).

• 

  Fig. 2 Sellar magnetic resonance (MR) images performed before and after transsphenoidal tumor removal. A : Sellar magnetic resonance (MR) image performed before transsphenoidal tumor removal show slightly prominent posterior pituitary gland with newly developed mass-like lesion in the left side of pituitary gland which is measured about 7 mm, without definite enhancement (red arrow). B : The follow-up sellar MR image 1-year post-operatively demonstrates no residual tumor in the pituitary gland and no significant interval change in polyostotic fibrous dysplasia (red arrow).

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  Fig. 3 Histopathology of pituitary adenoma. A : Histological examination revealed tumor cells with cytoplasmic, uniformly round, secretory granules of widely varying sizes on light microscopy, ×400. B : Immunohistochemistry for growth hormone (GH) with light hematoxylin counterstain, ×400. The majority of adenoma cells manifested GH immunoreactivity.

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