Resolution of Cyclicity After Pasireotide LAR in a Patient With Cushing Disease

Márcio Carlos Machado; Valter Angelo Sperling Cescato; Maria Candida Barisson Villares Fragoso; Marcello Delano Bronstein

doi:10.1016/j.aace.2021.02.007

Resolution of Cyclicity After Pasireotide LAR in a Patient With Cushing Disease

AACE Clin Case Rep. 2021 Mar 9;7(4):277-281. doi: 10.1016/j.aace.2021.02.007. eCollection 2021 Jul-Aug.

Authors

Márcio Carlos Machado^{1

2

3}, Valter Angelo Sperling Cescato⁴, Maria Candida Barisson Villares Fragoso⁵, Marcello Delano Bronstein^{1

2}

Affiliations

¹ Neuroendocrine Unit, Division of Endocrinology and Metabolism, University of Sao Paulo Medical School, Sao Paulo, SP, Brazil.
² Laboratory of Cellular and Molecular Endocrinology/LIM25, University of Sao Paulo Medical School, Sao Paulo, SP, Brazil.
³ Endocrinology Service, AC Camargo Cancer Center, Sao Paulo, SP, Brazil.
⁴ Neurosurgery Service, University of Sao Paulo Medical School, Sao Paulo, SP, Brazil.
⁵ Adrenal Unit, Division of Endocrinology and Metabolism, University of Sao Paulo Medical School, Sao Paulo, SP, Brazil.

Abstract

Objective: The cyclicity (CIC) of cortisol spontaneously occurs in a minority of patients with Cushing syndrome (CS). When it arises, diagnostic and therapeutic approaches become more challenging. This study aimed to report a patient with Cushing disease (CD) who achieved normalization of cortisol and CIC pattern with pasireotide long-acting release (pasi/LAR).

Methods: A 43-year-old female patient related an 8-month history of CS. An 8-mm pituitary nodule depicted by magnetic resonance imaging, serum cortisol suppression of >50% after 8 mg of dexamethasone therapy, and the absence of other lesions were compatible with a CD diagnosis. The patient presented with a CIC pattern with 1 episode before and 17 episodes after an unsuccessful pituitary surgery.

Results: Medical treatment with cabergoline alone up to 3.5 mg/wk and a combined treatment with ketoconazole 400 mg/d did not improve CIC CS. Pasi/LAR was initiated at a dose of 20 mg/mo. A few days after the first dose, the patient experienced symptoms suggestive of adrenal insufficiency. The medication and dose were maintained for 24 months. During this period, there was a normalization of UFC levels and progressive clinical improvement. Additionally, new episodes of CIC were not observed.

Conclusion: A CD patient with a challenging issue of CIC was reported. The condition was not controlled after pituitary surgery and by the combined treatment with cabergoline and ketoconazole, although hypercortisolism was abated by the continuous use of pasi/LAR. To our knowledge, this is the first report as regards the use of this medication to control CIC in a patient with CD.

Keywords: ACTH, adrenocorticotropic hormone; AI, adrenal insufficiency; BIPSS, bilateral and simultaneous petrosal sinus sampling; CAB, cabergoline; CD, Cushing disease; CIC, cyclicity; CS, Cushing syndrome; Cushing disease; Cushing syndrome; KTC, ketoconazole; LNSC, late-night salivary cortisol; MRI, magnetic resonance imaging; R, reference; UC, urine cortisol; cyclicity; pasi/LAR, pasireotide long-acting release; pasireotide; pasireotide LAR.

Publication types

Case Reports