Abstract
Purpose
Pasireotide is the only pituitary targeted medication registered for the treatment of Cushing’s disease. Drug efficacy data are largely based on a major prospective study in which the vast majority of patients had microadenomas. The purpose of this study was to summarize results of pasireotide treatment of ACTH secreting macroadenomas from our center.
Methods
Retrospective review of data extracted from clinical files.
Results
Three patients presented with large and invasive macroadenomas that required several surgical interventions and radiotherapy treatments. Patient 1 is a 57 year-old male who developed an extreme (27-fold) paradoxical response of urinary free cortisol (UFC) levels as measured 2 weeks after pasireotide institution, which increased further (71-fold) in response to dose increment but decreased to baseline levels after treatment interruption. Patient 2 is a 44 year old woman with a long standing (26 years) ACTH-secreting carcinoma metastatic to bone and after bilateral adrenalectomy. After an initial excellent response to pasireotide treatment, ACTH levels escaped suppression and a further rebound was noted 6 weeks after treatment interruption. Patient 3 is a 53 year old man that after escape from temozolomide therapy was started on pasireotide and rapidly responded by almost normalizing UFC excretion after 4 weeks, but returned to baseline UFC levels after four additional weeks of treatment.
Conclusions
We describe as yet unreported atypical responses to pasireotide treatment in patients with aggressive ACTH-secreting tumors. Increased vigilance is recommended during pasireotide treatment of such patients.
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YG has received research grants from Novartis, speaker honorarium from Novartis and Medison, and has participated in advisory boards for Novartis and Pfizer. NS has nothing to disclose.
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Greenman, Y., Stern, N. Paradoxical and atypical responses to pasireotide in aggressive ACTH-secreting pituitary tumors. Pituitary 19, 605–611 (2016). https://doi.org/10.1007/s11102-016-0755-9
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DOI: https://doi.org/10.1007/s11102-016-0755-9