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Sex differences in carcinoid syndrome: A gap to be closed

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Abstract

The incidence of neuroendocrine neoplasms and related carcinoid syndrome (CS) has markedly increased over the last decades and women seem to be more at risk than men for developing CS. Nevertheless, very few studies have investigated sex differences in clinical presentation and outcomes of CS. However, as per other tumours, sex might be relevant in influencing tumour localization, delay in diagnosis, clinical outcomes, prognosis and overall survival in CS. The present review was aimed at evaluating sex differences in CS, as they emerge from an extensive search of the recent literature. It emerged that CS occurs more frequently in female than in male patients with NENs and women seem to have a better prognosis and a slight advantage in overall survival and response to therapy. Moreover, the disease likely impacts differently the quality of life of men and women, with different psychological and social consequences. Nevertheless, sex differences, even if partially known, are deeply underestimated in clinical practice and data from clinical trials are lacking. There is urgent need to increase our understanding of the sex-related differences of CS, in order to define tailored strategies of management of the disease, improving both the quality of life and the prognosis of affected patients.

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Acknowledgements

This review is part of the ‘Neuroendocrine Tumours Innovation Knowledge and Education’ project led by Prof. Annamaria Colao and Prof. Antongiulio Faggiano, which aims at increasing the knowledge on neuroendocrine tumours. We would like to acknowledge all the Collaborators of this project: Manuela Albertelli, Luigi Barrea, Nazarena Betella, Severo Campione, Federica Campolo, Giuseppe Cannavale, Roberta Centello, Alessia Cozzolino, P. Crivelli, Federica de Cicco, Andrea Dicitore, S. Di Meglio, Sergio Di Molfetta, Valentina Di Vito, Andrea Dotto, Giuseppe Fanciulli, Tiziana Feola, Francesco Ferraù, Emanuele Filice, Marco Gallo, Elisa Giannetta, Federica Grillo, Elia Guadagno, Andrea Isidori, Anna La Salvia, Valentina Guarnotta, E. Lavezzi, Fabio Lo Calzo, Pasqualino Malandrino, Chiara Martini, Andrea Lania, R. Mazzilli, Erika Messina, N. Mikovic, Roberta Modica, Giovanna Muscogiuri, S. Olana, Genoveffa Pizza, A. Plebani, Gabriella Pugliese, Giulia Puliani, A. Ragni, Manila Rubino, Franz Sesti, A. Veresani , Giovanni Vitale, Isabella Zanata, Maria Chiara Zatelli.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

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Authors and Affiliations

  1. Department of Clinical and Experimental Medicine, Unit of Endocrinology, University of Messina, Messina, Italy

    Rosaria M. Ruggeri

  2. Division of Endocrinology and Diabetes, Department of Internal Medicine I, University Hospital, University of Würzburg, Würzburg, Germany

    Barbara Altieri

  3. S.C. Endocrinologia, ASST Grande Ospedale Metropolitano Niguarda, Milan, Italy

    Erika Grossrubatcher

  4. Department of Clinical Medicine and Surgery, Endocrinology Unit, University Federico II, Naples, Italy

    Roberto Minotta & Anna Maria Colao

  5. Department of Experimental Medicine, Sapienza University of Rome, Rome, Italy

    Maria Grazia Tarsitano & Andrea MIsidori

  6. Endocrinology Unit, Department of Clinical and Molecular Medicine, Sant’Andrea Hospital, Sapienza University of Rome, Rome, Italy

    Virginia Zamponi & Antongiulio Faggiano

  7. Cattedra Unesco “Educazione Alla Salute E Allo Sviluppo Sostenibile”, University Federico II, Naples, Italy

    Anna Maria Colao

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  1. Rosaria M. Ruggeri
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  8. Antongiulio Faggiano
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Contributions

R.M.R., B.A., E.G., R.M., M.G.T., and V.Z. made substantial contributions to the conception of the article, literature search and wrote the manuscript. A.I., A.F., and A.C. critically revised the work for important intellectual content. All authors read and approved the final manuscript.

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Correspondence to Rosaria M. Ruggeri.

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Ruggeri, R.M., Altieri, B., Grossrubatcher, E. et al. Sex differences in carcinoid syndrome: A gap to be closed. Rev Endocr Metab Disord 23, 659–669 (2022). https://doi.org/10.1007/s11154-022-09719-8

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