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Vol. 71. Issue 6.
Pages 263-270 (June - July 2024)
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Vol. 71. Issue 6.
Pages 263-270 (June - July 2024)
Review article
Carcinoid crisis: The challenge is still there
Crisis carcinoide: el desafío sigue ahí
Fernando Guerrero-Péreza,b,
Corresponding author
, Inmaculada Peiróc,d, José L. Vercher-Conejerob,e, Alex Teuléf, Carles Villabonaa,b
a Department of Endocrinology, Bellvitge University Hospital, Barcelona, Spain
b Biomedical Research Institute of Bellvitge (IDIBELL), Barcelona, Spain
c Clinical Nutrition Unit, Catalan Institute of Oncology, Barcelona, Spain
d Unit of Nutrition and Cancer-IDIBELL, Barcelona, Spain
e Department of Nuclear Medicine-PET Unit, Bellvitge University Hospital, Barcelona, Spain
f Department of Oncology, Catalan Institute of Oncology, L’Hospitalet de Llobregat, Barcelona, Spain
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Table 1. Carcinoid crisis criteria, prophylaxis and treatment used in different studies.
Abstract

Carcinoid crisis (CC) has classically been considered the extreme end of the spectrum of carcinoid syndrome (CS). However, this presumption and other aspects of CC remain poorly understood. Consequently, current clinical guidelines are based on a low quality of evidence. There is no standard definition of CC and its incidence is unknown. Patients with florid CS and elevated serotonin (or its derivatives) which develop CC have been reported during decades. Nevertheless, the hypothesis that CC is due to the sudden massive release of serotonin or other vasoactive substances is unproven. Many triggers of CC (surgery, anaesthesia, peptide receptor radionuclide therapy, tumour biopsy or liver-directed treatments) have been proposed. However, data from studies are heterogeneous and even contradictory. Finally, the role of octreotide in the prevention of CC has been questioned. Herein, we report a clinical case and perform a critical review of the evidence available today on this topic.

Keywords:
Carcinoid crisis
Carcinoid syndrome
Neuroendocrine tumours
Resumen

La crisis carcinoide (CC) se ha considerado clásicamente el espectro clínico extremo del síndrome carcinoide (SC). Sin embargo, esta presunción y otros aspectos de la CC siguen siendo poco conocidos. En consecuencia, las guías clínicas actuales se basan en una evidencia de baja calidad. No existe una definición estándar de CC y se desconoce su verdadera incidencia. Pacientes con SC florido y niveles elevados de serotonina (o sus derivados) que han desarrollado CC han sido reportados durante décadas. Sin embargo, la hipótesis de que la CC se deba a la súbita liberación de serotonina u otras sustancias vasoactivas no ha sido probada. Se han propuesto diferentes desencadenantes de la CC (cirugía, anestesia, radionúclidos, biopsias o tratamientos dirigidos de las metástasis hepáticas). Sin embargo, los datos de los diferentes estudios son heterogéneos e incluso contradictorios. Finalmente, el papel del octreótide en la prevención de la CC ha sido seriamente cuestionado. A continuación presentamos un caso clínico no publicado y realizamos una revisión crítica de la evidencia disponible sobre este tema.

Palabras clave:
Crisis carcinoide
Syndrome carcinoide
Tumores neuroendocrinos

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