Carcinoid crisis: The challenge is still there

Area of Science:

• Endocrinology
• Oncology
• Gastroenterology

Background:

• Carcinoid crisis (CC) is poorly understood and often considered the most severe form of carcinoid syndrome (CS).
• Current clinical guidelines for CC are based on limited evidence due to a lack of standardized definitions and unknown incidence.
• The exact pathophysiology, including the role of sudden massive release of vasoactive substances, remains unproven.

Purpose of the Study:

• To critically review the existing evidence on carcinoid crisis (CC).
• To evaluate proposed triggers of CC, such as surgery, anesthesia, and various therapies.
• To question the established role of octreotide in CC prevention.

Main Methods:

• A comprehensive literature review of carcinoid crisis (CC) and carcinoid syndrome (CS).
• Critical analysis of heterogeneous and contradictory data regarding CC triggers.
• Case report presentation to illustrate clinical aspects of CC.

Main Results:

• Evidence supporting the classical definition and incidence of CC is lacking.
• Proposed triggers for CC (e.g., surgery, PRRT, biopsy) have heterogeneous and sometimes contradictory supporting data.
• The efficacy of octreotide in preventing CC is questionable based on current literature.

Conclusions:

• Carcinoid crisis (CC) requires a standardized definition and further research to understand its pathophysiology and incidence.
• The proposed triggers of CC need more robust investigation due to inconsistent evidence.
• The role of octreotide in CC prevention warrants re-evaluation.