Endocrine Abstracts

Introduction: Most pituitary adenomas are benign. However, there are invasive forms with rapid growth rate and particular histological signs (atypical adenomas), making them considered pituitary carcinomas without metastases.

Observation: A 63-year-old patient was followed for 10 years for Cushing’s disease in the pituitary macrodenoma. Clinical evaluation found Cushing syndrome, pituitary tumor syndrome associated with diplopia, and trigeminal neuralgia. At the endocrine level, it presents substituted thyrotropic and gonadotropic insufficiency. Metabolically, severe osteoporosis complicated by vertebral fracture. He was operated on by the transphenoidal route and irradiated three times by gamma-knife radiosurgery without clear remission. Immunohistochemistry expressed only ACTH. Currently, the patient has persistent Cushing’s disease with eight times normal urinary free cortisol (UFC). MRI showing an invasive pituitary macrodenoma with extension to the cavernous sinus. Anticortisolic treatment (ketoconazole) was started with an improvement in CLU to 3 times normal. For the treatment of macroadenoma, surgical treatment has been discussed but rejected by surgeons. So, we indicated a somatostatin analogue ‘pasireotide‘, but it is not available in Morocco.

Discussion: Aggressive pituitary adenomas (AHA) are said to be atypical and have suprasellar and parasellar extension with invasion of the cranial nerves, cavernous sinus. Usually, they are resistant to conventional treatments (surgery and radiation therapy). It is important to differentiate them from pituitary carcinomas according to histological criteria. Treatment of these forms of aggressive adenoma consists of a combination of several surgical, pharmacological, and radiotherapy therapies. Pasireotide, which is an analogue of somatostatin, has particularly demonstrated efficacy in corticotropic pituitary adenomas. New therapeutic prospects based on chemotherapy using temozolomide have also been shown to be effective. These pituitary tumors must be recognized and aggressively treated to prevent complications.

References: 1) Steven W. J. Lamberts Å Leo J. Hofland. Future treatment strategies of aggressive pituitary tumors Pituitary (2009) 12 :261–264. 2) McCormack A, Wass A. and Grossman A. Aggressive pituitary tumours: the role of temozolomide and the assessment of MGMT status. Eur J Clin Invest 2011; 41 (10): 1133–114. 3) Annamaria Colao, Stephen. Petersenn and al. A 12-month phase 3 study of pasireotide in cushing’s disease. N Engl J Med 2012;366:914-24.