Endocrine Abstracts

A 27-year-old male patient during past 10 years noted a gradual change in his appearance, but began to seek a medical attention only 1.5 years ago due to reduced vision. Hormonal tests from March 2013 revealed marked elevation of GH to 106 mIU/ln (ref. values <20), and IGF1 to 567 ng/ml (121–336), decrease in testosterone levels to 1.91 ng/ml (3–12), with cortisol, LH, FSH, PRL, TSH values within the reference range; MRI of the brain showed a 4×7 mm adenoma of the anterior part of the pituitary, CT brain scan with contrast described poliostic dysplasia of the skull bones. From September 2013 octreotide depot injections therapy was initiated 10 mg per 28 days, with further increase to 20 mg per 28 days. In January 2014 levels of GH and IGF1 were still high in spite of medical treatment – 119 mIU/l and 1033 ng/ml respectively. In Apr 2014, at admission to Endocrinology Research Centre at the age of 27 years the patient was 205 cm tall and weighed 124 kg (BMI 29.5 kg/m²). ‘Café au lait’ pigmentation of the skin was noted at the chest, back, and abdomen. His facial features were acromegaloid with sloped towered skull. Lab tests confirmed the presence of the active acromegaly (GH – 117 mIU/l, IGF1 – 1412 ng/ml), brain MRI with contrast showed a marked increase in the size of previously described adenoma (17×23×14 mm), and progression of the fibrous dysplasia (predominantly hypointense on T1) of the skull base, parietal, temporal bones, scales of the frontal and occipital bones, hypopneuma-tization of frontal sinus and ethmoidal labyrinth, narrowing of the internal and the external auditory canals on the left. All these symptoms allowed us to suspect a McCune-Albright (MAS) syndrome. The progressive clinical course of the disease, insensitivity to octreoride treatment was the basis for the choice of further surgical treatment despite the pronounced fibrous dysplasia of the skull base. In Oct 2014 at Burdenko Neurosurgical Research Institute the patient underwent endoscopic endonasal removal of tumor using navigation BrainLab. Postoperatively levels of GH and IGF1 decreased to – 27 mIU/l and 856 ng/ml, visual function had markedly improved. He was then followed on depo octreotide injections 30 mg per 28 days and cabergoline 2 mg per week with later dose adjustments.

Conclusions: The treatment of acromegaly in the setting of MAS is characterized by multiple challenges that require the participation of a team of experienced endocrinologists and neurosurgeons.

Disclosure: This work was supported by the Grant of the President of the Russian Federation (awarded to E G Przyjalkowski : grant Number: MK-5411.2014.7).