Approach to the Management of Gastrointestinal Manifestations in Patients With Phaeochromocytoma and Paraganglioma.

in Clinical endocrinology by Monica Majumder, Cherie Chiang, Grace Kong, Michael Michael, Nirupa Sachithanandan, Emma Boehm

TLDR

  • The study reviewed the management of gastrointestinal symptoms in patients with phaeochromocytoma and paraganglioma (PPGL) and developed recommendations for minimizing the risk of catecholaminergic crisis.

Abstract

Managing gastrointestinal symptoms in patients with phaeochromocytoma and paraganglioma (PPGL) is challenging due to the risk of catecholaminergic crisis with many commonly prescribed medications, especially in functional tumours. We reviewed gastrointestinal symptom management and outcomes in PPGL patients at our centre and developed recommendations based on a literature review and our experience. A single-centre retrospective analysis of the management of gastrointestinal symptoms in patients with PPGL between 2019 and 2024 was completed. A literature review of gastrointestinal manifestations in PPGL was undertaken. Twenty-four individuals with PPGL admitted for radionuclide therapy, chemotherapy, surgery or other medical illness were included. Eighteen (75%) had metastatic disease. Fifty administration events of antiemetics for nausea or vomiting occurred. Two patients had acute colonic pseudo-obstruction. Dopamine antagonists (metoclopramide) and corticosteroids (dexamethasone) were administered to 10 and 9 patients, respectively, the majority of whom were alpha-blocked (n = 7) or had a dopaminergic/biochemically silent phenotype (n = 10). A patient with noradrenergic PPGL experienced a hypertensive episode following high-dose dexamethasone. No patients with biochemically negative/dopaminergic phenotypes or on alpha blockade experienced an antiemetic-related adverse event. Published evidence of dopamine antagonists and corticosteroids precipitating catecholaminergic crisis was mostly limited to case reports. While low-risk antiemetics (serotonin, histamine or neurokinin antagonists) are preferable, we found higher-risk antiemetics (dexamethasone and metoclopramide) can be cautiously administered in patients with a biochemically negative/dopaminergic phenotype or in those on adequate alpha blockade. Limited case reports demonstrated anti-cholinergic agents were beneficial for the management of acute colonic pseudo-obstruction. Optimal management of gastrointestinal symptoms in PPGL should consider disease characteristics such as primary location, secretory profile, alpha blockade and medication profile.

Overview

  • The study aimed to investigate the management of gastrointestinal symptoms in patients with phaeochromocytoma and paraganglioma (PPGL) while minimizing the risk of catecholaminergic crisis.
  • The study reviewed gastrointestinal symptom management and outcomes in 24 patients with PPGL, including a literature review and retrospective analysis of patient data.
  • The study aimed to develop recommendations for managing gastrointestinal symptoms in PPGL patients based on disease characteristics, medication profiles, and case reports.

Comparative Analysis & Findings

  • The study found that dopamine antagonists (metoclopramide) and corticosteroids (dexamethasone) were administered to 10 and 9 patients, respectively, with the majority being alpha-blocked or having a dopaminergic/biochemically silent phenotype.
  • However, a patient with noradrenergic PPGL experienced a hypertensive episode following high-dose dexamethasone, highlighting the need for caution in administering higher-risk antiemetics.
  • No patients with biochemically negative/dopaminergic phenotypes or on alpha blockade experienced antiemetic-related adverse events, suggesting that these patients may be candidates for higher-risk antiemetics under cautious circumstances.

Implications and Future Directions

  • The study highlights the importance of considering disease characteristics, medication profiles, and case reports when managing gastrointestinal symptoms in PPGL patients to minimize the risk of catecholaminergic crisis.
  • Future research should explore the optimal management of gastrointestinal symptoms in PPGL patients, including the use of low-risk antiemetics and anti-cholinergic agents, and the role of alpha blockade in preventing catecholaminergic crisis.
  • The study's findings suggest that further case reports and controlled trials are needed to confirm the safety and efficacy of antiemetics and anti-cholinergic agents in PPGL patients.
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