What is carcinoid crisis?

1.

Wolin, Edward M., Jensen, Robert T. (2024). In Goldman-Cecil Medicine (pp. 1561). DOI: 10.1016/B978-0-323-93038-3.00213-6

Diarrhea of secretory type is a major feature of the carcinoid syndrome. Stool volume is typically greater than 1L/day and persists with fasting (when on intravenous fluids). No osmotic gap is present when stool osmolality is measured. Flushing from carcinoid syndrome involves attacks of skin redness, which may be associated with burning and warmth without sweating. Redness is typically noted on the face and neck, sometimes extending to the trunk and abdomen. Psychiatric manifestations of tryptophan deficiency occur when a large percentage of dietary tryptophan is shunted to produce serotonin, thereby leaving less tryptophan to form nicotinic acid and protein. Pellagra (nicotinic acid deficiency;Chapter 199) can be seen when the urinary excretion of 5-HIAA is greater than 100mg/day as a result of this shunting. Carcinoid heart disease (Chapter 47) is endomyocardial fibrosis caused by serotonin.The serotonin level is typically greater than 1000pg/mL, and the urinary 5-HIAA is typically greater than 57mg/24hours. Cardiac fibrotic manifestations commonly include tricuspid valve regurgitation (65%), pulmonary valve regurgitation (20%), and right ventricular myocardial restrictive disease, but about 10% of patients may develop left-sided cardiac involvement. Symptoms of right-sided heart failure include swelling in legs and abdomen, shortness of breath, and fatigue. Diagnosis often is easily made by echocardiography. Intraperitoneal and retroperitoneal fibrosis, which also results from the high level of serotonin, causes intense peritoneal fibrosis that leads to bowel obstruction in about 50% of patients, as well as mesenteric vessel occlusion and bowel ischemia. Retroperitoneal fibrosis can cause ureteral obstruction. Carcinoid crisis, which is an attack of severe and sustained flushing with life-threatening hemodynamic compromise and bronchoconstriction, is precipitated by anesthesia, surgery, tumor necrosis, or catecholamine infusion.

2.

Neuroendocrine Tumors of the Gastrointestinal Tract, Lung, and Thymus (Carcinoid Tumors), Elsevier ClinicalKey Clinical Overview

Complications and Prognosis Carcinoid heart disease Carcinoid heart disease occurs in 25% to 50% of patients with carcinoid syndrome and is associated with reduced overall survival Elevated levels of hormones such as serotonin result in plaque formation, primarily on right‐sided heart valves and endocardial surfaces Left‐sided valve involvement may occur: In the presence of a patent foramen ovale or a functioning lung neuroendocrine tumor In cases with very high circulating levels of vasoactive substances Right-sided heart disease is characterized by tricuspid insufficiency, pulmonary stenosis, and ensuing pulmonary hypertension Left-sided heart disease is reported in 10% of patients and is characterized by coronary vasospasm and angina Carcinoid crisis Potentially fatal condition caused by sudden release of high levels of serotonin and/or other biologically active amines and peptides from a neuroendocrine tumor Characterized by profuse diarrhea, intense flushing, hypotension, confusion, and dyspnea Can occur spontaneously but is more frequently triggered by tumor manipulation (ie, biopsy, resection), stress, anesthesia, cytolytic therapies (hepatic embolization), or peptide receptor radionuclide therapy Pellagra (niacin deficiency) Caused by shift in metabolism of tryptophan from niacin to serotonin Results in sun‐sensitive dermatitis, diarrhea, and dementia Cushing syndrome Due to ectopic secretion of corticotropin by some pulmonary carcinoids Bowel obstruction Can result from small bowel tumor growth or intussusception secondary to mesenteric fibrosis and contraction Secondary malignancies Patients with carcinoids have higher risk

3.

Bardasi C, Benatti S, Luppi G, et al. Cancers. 2022;14(3):662. doi:10.3390/cancers14030662. Copyright License: CC BY

Publish date: January 5, 2022.

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.

4.

Maxwell JE, Naraev B, Halperin DM, Choti MA, Halfdanarson TR. Annals of Surgical Oncology. 2022;29(5):3072-3084. doi:10.1245/s10434-022-11371-0.

Publish date: May 0, 2022.

Carcinoid crisis is a potentially fatal condition characterized by various symptoms, including hemodynamic instability, flushing, and diarrhea. The incidence of carcinoid crisis is unknown, in part due to inconsistency in definitions across studies. Triggers of carcinoid crisis include general anesthesia and surgical procedures, but drug-induced and spontaneous cases have also been reported. Patients with neuroendocrine tumors (NETs) and carcinoid syndrome are at risk for carcinoid crisis. The pathophysiology of carcinoid crisis has been attributed to secretion of bioactive substances, such as serotonin, histamine, bradykinin, and kallikrein by NETs. The somatostatin analog octreotide has been considered the standard of care for carcinoid crisis due to its inhibitory effect on hormone release and relatively fast resolution of carcinoid crisis symptoms in several... (truncated preview)

5.

Wonn SM, Ratzlaff AN, Pommier SJ, McCully BH, Pommier RF. Surgery. 2022;171(1):88-93. doi:10.1016/j.surg.2021.03.063.

Publish date: January 6, 2022.

BACKGROUND: Carcinoid crises, defined as the sudden onset of hemodynamic instability in patients with neuroendocrine tumors undergoing operation, are associated with significantly increased risk of postoperative complications. Octreotide has been used prophylactically to reduce crisis rates as well as therapeutically to treat crises that still occur. However, studies using octreotide still report crisis rates of 3.4% to 35%, leading to the questioning of its efficacy. METHODS: Patients with neuroendocrine tumors undergoing operation between 2017 to 2020 with no perioperative octreotide were prospectively studied. Clinicopathologic data were compared by χ test for discrete variables and by Mann-Whitney U test for continuous variables. RESULTS: One hundred and seventy-one patients underwent 195 operations. Crisis was documented in 49 operations (25%), with a mean duration of 3 minutes. Crisis was more likely to occur in patients with small bowel primary tumors (P = .012), older age (P = .015), and carcinoid syndrome (P < .001). Those with crises were more likely to have major postoperative complications (P = .003). CONCLUSION: Completely eliminating perioperative octreotide resulted in neither increased rate nor duration compared with previous studies using octreotide. We conclude perioperative octreotide use may be safely stopped, owing to inefficacy, though the need for an effective medication is clear given continued higher rates of complications.