Exceptional Response of Nelson’s Syndrome to Pasireotide LAR in the Long-Term Follow-up of 9 Years

Fill, S; Popp, Kathrin; Stalla, Günter; Athanasoulia-Kaspar, Anastasia P.

doi:10.1055/a-1158-9214

Cited by 1 publication

(1 citation statement)

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“…Data on the efficacy of this agent in patients with NS are extremely scarce. Pasireotide has reduced ACTH levels in some case reports [51][52][53], and it has induced tumour shrinkage in one of them [53]. In a recent case series, 7 patients with NS were treated with pasireotide for a 28-week administration period; reduction of ACTH was reported in all of them, but with no significant change in the tumour volume [54].…”

Section: Somatostatin Analoguesmentioning

confidence: 99%

Management of Nelson’s Syndrome

Fountas

Karavitaki

2022

Medicina

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Nelson’s syndrome is a potentially severe condition that may develop in patients with Cushing’s disease treated with bilateral adrenalectomy. Its management can be challenging. Pituitary surgery followed or not by radiotherapy offers the most optimal tumour control, whilst pituitary irradiation alone needs to be considered in cases requiring intervention and are poor surgical candidates. Observation is an option for patients with small lesions, not causing mass effects to vital adjacent structures but close follow-up is required for a timely detection of corticotroph tumour progression and for further treatment if required. To date, no medical therapy has been consistently proven to be effective in Nelson’s syndrome. Pharmacotherapy, however, should be considered when other management approaches have failed. A subset of patients with Nelson’s syndrome may develop further tumour growth after primary treatment, and, in some cases, a truly aggressive tumour behaviour can be demonstrated. In the absence of evidence-based guidance, the management of these cases is individualized and tailored to previously offered treatments. Temozolomide has been used in patients with aggressive Nelson’s with no consistent results. Development of tumour-targeted therapeutic agents are an unmet need for the management of aggressive cases of Nelson’s syndrome.

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