Carcinoid crisis

As early as the 19th century, O. Lubarsch (1860 - 1933) and W. B. Ransom (1860 - 1909) described unusual tumors in the small intestine whose cancer entity differed from other tumor types. The pathologist S. Oberndorfer (1876 - 1944) was the first to coin the term "carcinoid" for this type of tumor (Schulz 2016).

Octreotide was first successfully used in 1985 by Kvols et al. in a patient with NET in the context of a carcinoid crisis as two i.v. boluses of 50 µg (Bardasi 2022).

In 2001, Kinney et al. found in a study that the best prophylaxis to prevent CC is the intraoperative administration of octreotide. However, the actual role of this drug has not yet been sufficiently clarified, and available data are partly contradictory (Bardasi 2022).

Carcinoid crisis (CC) is an immediately life-threatening complication of carcinoid syndrome (Kasper 2015).

A carcinoid crisis is a sudden onset of hemodynamic instability that is a life-threatening event (Bardasi 2022).

Carcinoid crisis occurs more frequently in patients who:

- Have severe symptoms

- Whose 5- HIAA level in the urine is greatly increased, i.e. > 200 mg / d (Kasper 2015)

The crisis can be triggered by:

• Biopsy
• Endoscopy
• chemotherapy
• surgery
• anesthesia
• Radiological examinations such as hepatic artery embolization, vascular catheterization
• Stress
• Frequent palpation of the tumor
• Spontaneous (Kasper 2015)

Carcinoid crisis is a rare but extremely dangerous complication that can occur in patients with neuroendocrine tumors (NET) (Bardasi 2022).

In a carcinoid crisis, there is an excessive release of vasoactive substances such as histamine, serotonin and bradykinin (Larsen 2022), with the massive release of serotonin playing a decisive role (Payot 2020).

Patients experience:

• Prolonged severe drop in blood pressure (Herold 2023)
• severe hot flushes
• abdominal pain
• diarrhea
• Cardiac abnormalities such as tachycardia, arterial hypertension or hypotension
• Confusion, stupor (Kasper 2015)

In a carcinoid crisis, octreotide, a potent inhibitor peptide (Bardasi 2022), should be administered subcutaneously or intravenously to alleviate the severity of symptoms and prevent the further release of vasoactive substances (Payot 2020).

To date, however, there is no standard regimen or information on the duration of treatment and dosage, as the available data is limited (Bardasi 2022).

If the carcinoid crisis is not treated appropriately, it can be fatal (Kasper 2015).

The carcinoid crisis still poses a major clinical challenge today, as the pathophysiology and treatment are still poorly understood (Guerrero-Perez 2024).

High doses of octreotide (a synthetic analog of somatostatin) are recommended perioperatively during surgery for neuroendocrine tumors to prevent a carcinoid crisis (Rinke 2018 / Werner 2020).

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