Endocrine Abstracts

Introduction: In 7–20% of cases, Cushing’s disease is due to ACTH-producing macroadenoma. Aim of the study was to present our observations concerning etiologic, clinical, and therapeutic diversity of Cushing’s disease in patients with macrocorticotropinomas.

Material and methods: Retrospective analysis of medical records of nine patients (5F; 4M) hospitalized in 2002–2015.

Results: Four of the patients (2F; 2M) were diagnosed before they were 40 years old (mean age 28.5) and five (3F; 2M), >40 (mean age 50). In 3F tumour was initially discovered as microadenoma. A woman (23) first underwent bilateral adrenalectomy because of inability to neurosurgery and 5 years later Nelson’s syndrome developed. In two consecutive females (49 and 52) at the moment of diagnosis MRI revealed microadenomas 9×7 and 5×4 mm respectively. In the first female 2 years after initial neurosurgery consecutive four transsphenoidal reoperations, stereotactic radiotherapy, and bilateral adrenalectomy were conducted. Then she developed Nelson’s syndrome. Propter a consecutive rapid tumour’s invasion, themozolomide and bevacuzimab were introduced. She died 7 years after the diagnosis. In the second patient tumour regrowth was observed 6 years after the first neurosurgery and because of failure of second adrenalectomy and mild hypercortisolism she was qualified to pasireotyd therapy. In six patients (2F; 4M) the pituitary tumours were initially diagnosed as macroadenomas. One patient with 5 cm tumour and overt hypercortisolism died after craniotomy. Another patient presented cyclic Cushing’s syndrome with pituitary macroadenoma discovered after 7 years’ observation. In the next patient unique cause of Cushing’s disease was Crooke’s macroadenoma, refractory to two neurosurgeries with good response to themozolomide. In two cases (1F; 1M) macrocorticotropinomas were discovered as incidentalomas and operated as clinically inactive (silent) tumours. In these patients the tumour recurred 5 years after the first neurosurgery and visible hypercortisolism developed.

Conclusion: Macrocorticotropinomas are very heterogenic group of pituitary tumours with high aetiologic, clinical and therapeutic diversity.