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Endocrine Abstracts (2014) 35 P226 | DOI: 10.1530/endoabs.35.P226

ECE2014 Poster Presentations Clinical case reports Pituitary/Adrenal (50 abstracts)

Treatment of aggressive corticotropinoma with temozolomide and bevacizumab in patient with Nelson's syndrome

Joanna Malicka 1 , Maria Kurowska 1 , Grzegorz Zielinski 2 , Jerzy S Tarach 1 , Maria Maksymowicz 3 & Piotr Denew 1


1Department of Endocrinology, Medical University, Lublin, Poland; 2Department of Neurosurgery, Military Medical Institute, Warsaw, Poland; 3Department of Pathology, M. Sklodowska-Curie Memorial Cancer Centre and Institute of Oncology, Warsaw, Poland.


Introduction: Aggressive corticotropinomas are more invasive than other pituitary tumors. Recent reports have documented the efficacy of temozolomide alone or in combination with an anti-angiogenic agent –bevacizumab in invasive pituitary adenomas treatment.

Objective: Presentation a case of 56-year-old woman initially diagnosed as Cushing’s disease and the analysis of treatment procedures with special concentration on temozolomide and bevacizumab.

Case study: At the moment of diagnosis she presented a clinical picture of severe hypercortisolism with high ACTH concentration, loss of cortisol circadian rhythm, fivefold increased urinary cortisol excretion and a lack of cortisol suppression after low and high dexamethasone dose. The ectopic source of ACTH was excluded. MRI revealed 9×7 mm microadenoma. The patient underwent selective transsphenoidal adenomectomy followed by a 27-month-long period of remission. A postoperative exploration revealed a sparsely granulated corticotroph adenoma with MIB-1 index of about 40%. Due to a re-growth of the tumor she underwent two transsphenoidal reoperations followed by stereotactic radiotherapy. Afterwards, because of treatment failures, bilateral total adrenalectomy was performed. Then the patient developed Nelson’s syndrome. A fourth transsphenoidal adenomectomy was performed, but there was a rapid recurrence of the tumor with an expansion to the right cavernous sinus and suprasellar region with a compression of the optic chiasm causing blindness of the right eye. Five months later she underwent a right fronto-temporal craniotomy with subtotal suprasellar adenomectomy. Propter a rapid re-growth of the tumor the patient was disqualified for gamma-knife and received cabergoline and somatostatin analogue for some time. Only nine cycles of TMZ resulted in marked clinical, biochemical and radiological improvement. After 6 months pause in temozolomide a consecutive relapse occurred. Then bevacizumab was introduced. Until now the clinical stabilization of the disease has been observed.

Conclusion: Temozolomide alone or in combination with bevacizumab can be an effective treatment option in invasive corticotropinomas.

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