Multidisciplinary care key in updated pheochromocytoma, paraganglioma guideline
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People with metastatic pheochromocytomas and paragangliomas should be closely monitored and undergo routine cross-sectional imaging while also being evaluated by a multidisciplinary team, according to a guideline published in Pancreas.
A North American Neuroendocrine Tumor Society panel of 18 experts from multiple fields drafted a set of consensus guidelines for the treatment of metastatic pheochromocytomas and paragangliomas after a meeting held in October 2019. The recommendations focus on the diagnosis, management, treatment and surveillance of metastatic and primary pheochromocytomas and paragangliomas and consider some of the recent advances in diagnosing and treating the disease.
“The guidelines review all treatments that are typically used to treat metastatic pheochromocytomas and paragangliomas from cytotoxic chemotherapy to targeted therapies to radionuclide therapies,” Lauren Fishbein, MD, PhD, MTR, associate professor of medicine in the department of endocrinology, metabolism and diabetes at University of Colorado Anschutz School of Medicine, told Healio. “Azedra (iobenguane I-131, Progenics Pharmaceuticals) is the only FDA-approved therapy for metastatic or unresectable pheochromocytomas and paragangliomas at this time. There are several potential treatments in clinical trials right now, so hopefully more data will be available soon.”
Monitoring, testing with primary disease
One area of focus was monitoring and testing for people with primary pheochromocytomas and paragangliomas. According to the guideline, up to 40% of primary pheochromocytomas and paragangliomas are seen in people with a germline pathogenic variant in at least one of 12 well-studied susceptibility genes, and about 40% to 45% of people with metastatic pheochromocytomas and paragangliomas have a known heritable pathogenic variant. Researchers recommended all people with primary or metastatic pheochromocytomas and paragangliomas have clinical germline genetic testing performed. Additionally, family cascade testing is recommended if a susceptibility gene pathogenic variant is found.
Upon diagnosis of primary pheochromocytomas and paragangliomas, an evaluation for metastatic disease should be considered and presurgical characteristics assessed to help determine surgical options. Fishbein said those with primary pheochromocytomas and paragangliomas should be monitored for life due to the risk for metastatic disease. People with primary disease should undergo annual testing of plasma-free or 24-hour urine fractionated metanephrines to detect recurrence or metastatic disease.
Annual imaging is not recommended for all patients, but those with nonsecreting primary tumors or who are otherwise at higher risk for metastatic disease should consider yearly imaging.
Treating metastatic disease
Frequent monitoring is recommended for people with secreting metastatic pheochromocytomas and paragangliomas, according to the guideline. At minimum, patients should be assessed for symptoms, have blood pressure and heart rate measured and plasma metanephrine levels checked every 3 to 6 months. Symptoms and signs of anxiety, mood changes, severe constipation and new-onset or worsening diabetes should be monitored.
“Patients with secreting metastatic pheochromocytomas and paragangliomas should be monitored for hemodynamic and other consequences of high catecholamine production, which impacts quality of life, morbidity and mortality,” Fishbein said.
Surveillance imaging with anatomical cross-sectional imaging with either CT or MRI every 3 to 6 months is recommended for people with metastatic pheochromocytomas and paragangliomas in the first year of disease. If disease stabilizes, imaging can be conducted every 6 to 12 months. However, those on systemic therapies should continue with imaging every 3 to 6 months.
The researchers wrote there is a lack of evidence for any therapy treating metastatic pheochromocytomas and paragangliomas, and follow-up is limited in retrospective studies. Because of this, treatment decisions should be made by a multidisciplinary team.
“Multidisciplinary care at an expert center is critical for patients with metastatic pheochromocytoma and paragangliomas to receive the most up-to-date recommendations and treatments from providers who are experienced in treating this rare cancer,” Fishbein said.
Additionally, Fishbein said more multicenter collaborative research is needed to study the biology of tumors, biomarkers for high-risk tumors and therapeutic options in metastatic pheochromocytomas and paragangliomas.
For more information:
Lauren Fishbein, MD, PhD, MTR, can be reached at lauren.fishbein@cuanschutz.edu.
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