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Endocrine Abstracts (2017) 49 EP954 | DOI: 10.1530/endoabs.49.EP954

1Department of Neuroendocrinology, Clinic for Endocrinology, Diabetes and Metabolic Diseases, Clinical Center of Serbia, Belgrade, Serbia; 2Belgrade University School of Medicine, Belgrade, Serbia; 3Institute of Pathology, Belgrade, Serbia; 4Barts and the London School of Medicine, London, UK; 5Institute de pathologie multu-site des HCL, Lyon, France.

40-year-old female patient presented with acromegaly in 2008 (GH 61 (g/L, IGF-1 774 ng/ml PRL 1500 mU/L). Macro-adenoma invading the right cavernous sinus was found on MRI and she underwent two pituitary surgeries revealing sparsely granulated GH adenoma with scattered PRL cells, low Ki 67 and negative p53 immuno-staining. Her second cousin was treated for macro prolactinoma. Both patients tested negative for germline mutations in the AIP and menin genes. Treatment with somatostatin analogue and dopamine agonist was initiated. She was well controlled (GH 1.1 (g/L and IGF-1 291 ng/ml) until 2011 (IGF-1 576\..608 ng/ml, GH 4.1 (g/L) when she received 20 Gy gamma knife radio surgery, to the right para-sellar rest and clivus with good response (2012 IGF-1 235-306 ng/ml). In 2014 she became symptomatic with headache, VI cranial nerve palsy and biochemical deterioration (GH 5.3 (g/L and IGF 1 549 ng/ml). In November 2015 she developed VII & VIII cranial nerve palsy and biochemically deteriorated (GH 13 (g/L and IGF-1 857 ng/ml). MRI disclosed tumour progression from the right para-sellar to the infra-sellar region infiltrating clivus, sphenoid and temporal bone and posterior cranial fossa with progressive rise in GH 16(g/L and IGF-1 909 ng/ml levels despite treatment with somatostatin analogue and dopamine agonist. Avid tracer uptake was noted in the pituitary tumour and neck lymph node on the left side on FDG PET CT and SST2 scintigraphy. Third surgery using the posterior cranial fossa approach was performed. Tumour biology revealed sparsely granulated GH adenoma with high proliferative activity Ki 67 17.2%, positive p53 and 2/10 mitoses. Revised immunohistochemistry revealed recurrent sparsely granulated GH adenoma expressing SSTR2 and Pit1 with low proliferation (Ki 67: 0, mitoses: 0) positive p53 (1%) grade 2a. This case illustrates our AIP negative FIPA patient with sparsely granulated GH adenoma and aggressive behaviour refractory to multimodality treatment during long term follow up.

Volume 49

19th European Congress of Endocrinology

Lisbon, Portugal
20 May 2017 - 23 May 2017

European Society of Endocrinology 

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