Paradoxical and atypical responses to pasireotide in aggressive ACTH-secreting pituitary tumors
- 328 Downloads
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.
Retrospective review of data extracted from clinical files.
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.
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.
KeywordsCushing’s disease Atypical tumor Pasireotide Paradoxical response Pituitary carcinoma
No specific funding was provided for this work.
Compliance with ethical standards
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.
- 5.Simeoli C, Auriemma RS, Tortora F, De Leo M, Iacuaniello D, Cozzolino A, De Martino MC, Pivonello C, Mainolfi CG, Rossi R, Cirillo S, Colao A, Pivonello R (2015) The treatment with pasireotide in Cushing’s disease: effects of long-term treatment on tumor mass in the experience of a single center. Endocrine 50:725–740CrossRefPubMedGoogle Scholar
- 10.Cannavo S, Messina E, Albani A, Ferrau F, Barresi V, Priola S, Esposito F, Angileri F (2016) Clinical management of critically ill patients with Cushing’s disease due to ACTH-secreting pituitary macroadenomas: effectiveness of presurgical treatment with pasireotide. Endocrine 52:481–487CrossRefPubMedGoogle Scholar
- 12.Rajendran R, Naik S, Sandeman DD, Nasruddin AB (2013) Pasireotide therapy in a rare and unusual case of plurihormonal pituitary macroadenoma. Endocrinol Diabet Metab Case Rep 2013:130026Google Scholar
- 17.Mohamed A, Blanchard MP, Albertelli M, Barbieri F, Brue T, Niccoli P, Delpero JR, Monges G, Garcia S, Ferone D, Florio T, Enjalbert A, Moutardier V, Schonbrunn A, Gerard C, Barlier A, Saveanu A (2014) Pasireotide and octreotide antiproliferative effects and sst2 trafficking in human pancreatic neuroendocrine tumor cultures. Endocr Relat Cancer 21:691–704CrossRefPubMedGoogle Scholar