Abstract
Simple SummaryIn this review, the Authors are going to discuss the main highlights of the Carcinoid Crisis, an uncommon manifestation related to neuroendocrine tumors, focusing on the potential etiopathogenetic mechanisms, clinical implications, potential treatments and prophylaxis.Carcinoid Crisis represents a rare and extremely dangerous manifestation that can occur in patients with Neuroendocrine Tumors (NETs). It is characterized by a sudden onset of hemodynamic instability, sometimes associated with the classical symptoms of carcinoid syndrome, such as bronchospasm and flushing. Carcinoid Crisis seems to be caused by a massive release of vasoactive substances, typically produced by neuroendocrine cells, and can emerge after abdominal procedures, but also spontaneously in rare instances. To date, there are no empirically derived guidelines for the management of this cancer-related medical emergency, and the available evidence essentially comes from single-case reports or dated small retrospective series. A transfer to the Intensive Care Unit may be necessary during the acute setting, when the severe hypotension becomes unresponsive to standard practices, such as volemic filling and the infusion of vasopressor therapy. The only effective strategy is represented by prevention. The administration of octreotide, anxiolytic and antihistaminic agents represents the current treatment approach to avoid hormone release and prevent major complications. However, no standard protocols are available, resulting in great variability in terms of schedules, doses, ways of administration and timing of prophylactic treatments.
Highlights
Neuroendocrine Tumors (NETs) are a heterogeneous family of neoplasms that can arise from any district of the body and can occur with an extremely wide range of symptoms and clinical manifestations, due to hormonal secretion
Carcinoid Syndrome (CS) is the most typical and common clinical presentation in functional NETs and is characterized by diarrhea, gastrointestinal discomforts, such as cramps and nausea, facial flushing with apparent peripheral cyanosis, eventual right-sided valvular heart disease preceded by palpitation and dyspnea with bronchospasm
Patients with NETs can exhibit a life-threatening occurrence known as Carcinoid Crisis (CC), generally described as a sudden onset of hemodynamic instability, sometimes accompanied by characteristics of carcinoid syndrome, such as prolonged flushing, wheezing and hyperthermia
Summary
Neuroendocrine Tumors (NETs) are a heterogeneous family of neoplasms that can arise from any district of the body and can occur with an extremely wide range of symptoms and clinical manifestations, due to hormonal secretion. Patients with NETs can exhibit a life-threatening occurrence known as Carcinoid Crisis (CC), generally described as a sudden onset of hemodynamic instability (prolonged hypertension or severe hypotension, unresponsive to standard practices), sometimes accompanied by characteristics of carcinoid syndrome, such as prolonged flushing, wheezing and hyperthermia. Theoretically every kind of tumor stress can cause CC, it typically occurs during invasive procedures, such as surgery and liver embolization [4], but it can arise during clinical examination, biopsy, mammography [5], transesophageal echocardiography [6] or induction of anesthesia. The reported incidence of CC is about 7% in patients with NETs [9] undergoing abdominal surgery [10,11], but more recent works observed a higher number of cases, with a maximum incidence of 24–30% [12]
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