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Preoperative management of patients with ectopic Cushing’s syndrome caused by ACTH-secreting pheochromocytoma: a case series and review of the literature

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Abstract

Purpose

ACTH-secreting pheochromocytoma is a rare cause of ectopic Cushing’s syndrome, posing a clinical challenge for the severity of its clinical presentation, the difficulty in the prevention and the management of surgical complications. Sparse data are currently available about the optimal preoperative management of the severe symptoms due to both hypercortisolism and catecholamine excess, especially regarding the role and timing of medical therapies.

Methods

We present a series of three patients with ACTH-secreting pheochromocytoma. A brief review of the available literature evidence on the preoperative management of this rare clinical condition is also conducted.

Discussion

Patients with ACTH-secreting pheochromocytoma show peculiarities as compared to other forms of ACTH-dependent Cushing’s syndrome, in terms of clinical presentation, preoperative management, and peri- and post-surgical short-term outcome. Pheochromocytoma should be ruled out in patient with ectopic CS of unknown origin because of the high anesthesiologic risk of proceeding to surgery with an undiagnosed pheochromocytoma. Proper preoperative recognition of complications of both hypercortisolism and catecholamines excess is the key to prevent the morbidity and mortality of an ACTH-producing pheochromocytoma. In these patients the absolute priority is to control excessive cortisol secretion since the rapid correction of the hypercortisolism is the most effective treatment of all the related comorbidities and it is mandatory to prevent severe complications during surgery, opting if necessary for a “block-and-replace” regimen.

Conclusion

Our additional cases and this literature review could provide a better understanding of the complications to be evaluated at diagnosis and some suggestions on their management during the preoperative period.

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Data availability

The dataset genereted and analyzed during the study are available from the corresponding author on reasonable request.

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Author notes

  1. M. F. Birtolo and E. M. Grossrubatscher have contributed equally to the manuscript and must be considered as co-first authors.

Authors and Affiliations

  1. Department of Biomedical Sciences, Humanitas University, Via Rita Levi Montalcini 4 Pieve Emanuele, 20090, Milan, Italy

    M. F. Birtolo, S. Antonini, G. Mazziotti & A. G. Lania

  2. Endocrinology, Diabetology and Medical Andrology Unit, IRCCS Humanitas Research Hospital, Via Manzoni 56 Rozzano, 20089, Milan, Italy

    M. F. Birtolo, S. Antonini, G. Mazziotti & A. G. Lania

  3. Endocrine Unit, ASST Grande Ospedale Metropolitano Niguarda, Piazza Ospedale Maggiore 3, 20162, Milan, Italy

    E. M. Grossrubatscher & I. Chiodini

  4. Endocrinology, Clinica San Carlo, Via Ospedale 21, 20037 Paderno Dugnano, Milan, Italy

    P. Loli

  5. Department of Medical Biotechnology and Translational Medicine, University of Milan, 20122, Milan, Italy

    I. Chiodini

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  1. M. F. Birtolo
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Correspondence to A. G. Lania.

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Birtolo, M.F., Grossrubatscher, E.M., Antonini, S. et al. Preoperative management of patients with ectopic Cushing’s syndrome caused by ACTH-secreting pheochromocytoma: a case series and review of the literature. J Endocrinol Invest 46, 1983–1994 (2023). https://doi.org/10.1007/s40618-023-02105-4

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