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Case Reports
. 2022 May 12;29(5):3494-3498.
doi: 10.3390/curroncol29050282.

Successful Treatment with Selpercatinib for Ectopic Cushing's Syndrome Due to Medullary Thyroid Cancer

Oskar Ragnarsson  1 , Marta Piasecka  1 , Andreas Hallqvist  2
Affiliations
Case Reports

Successful Treatment with Selpercatinib for Ectopic Cushing's Syndrome Due to Medullary Thyroid Cancer

Oskar Ragnarsson et al. Curr Oncol. .

Abstract

Selpercatinib, a RET kinase inhibitor, is an effective treatment for patients with medullary thyroid cancer with RET mutations. In this paper, we present the case of a 62-year-old man with ectopic Cushing’s syndrome due to medullary thyroid cancer who received treatment with selpercatinib. Six months later, all the cushingoid features had resolved, and s-calcitonin had decreased from 580 pmol/L to 3.5 pmol/L (normal < 3). After further 6 months, s-calcitonin had normalized (1.5 pmol/L), and radiological evaluation showed a profound tumour volume reduction. We are aware of two other cases where treatment with selpercatinib has also been successful. Thus, selpercatinib may be a promising treatment alternative in patients with ectopic Cushing’s syndrome due to medullary thyroid cancer, especially when other treatment options are ineffective or not tolerated.

Keywords: ectopic Cushing’s syndrome; hypercortisolism; medullary thyroid cancer; paraneoplastic syndrome.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Computed tomography showing mediastinal and abdominal lymph node metastases before (a,c) and 12 months after (b,d) treatment with selpercatinib was started. Of additional note is a hyperplastic adrenal gland (arrows) before treatment that decreased in volume following treatment.
Figure 1
Figure 1
Computed tomography showing mediastinal and abdominal lymph node metastases before (a,c) and 12 months after (b,d) treatment with selpercatinib was started. Of additional note is a hyperplastic adrenal gland (arrows) before treatment that decreased in volume following treatment.
Figure 2
Figure 2
Schematic summary of the effects of tyrosine kinase inhibitors for metastatic medullary thyroid cancer. Vandetanib and cabozantinib are multi-targeted inhibitors. Vandetanib is mainly an inhibitor of vascular endothelial growth factor receptor-2 (VEGFR2), epidermal growth factor receptor (EGFR), and RET tyrosine kinase. Cabozantinib is mainly an inhibitor of VEGFR2, RET tyrosine kinase, and tyrosine-protein kinase Met (c-MET). Selpercatinib is a selective RET tyrosine kinase inhibitor, making it less toxic and less likely to cause side effects.
See this image and copyright information in PMC

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