Pheochromocytoma Paraganglioma Syndrome 5 C3279992
A hereditary condition predisposing to tumors of the adrenal glands and nervous system. Affected individuals require lifelong tumor surveillance and surgical treatment when needed.
Key facts at a glance
- ICD-10
- E34.9
- ICD-11
- 5A00.0
- Body system
- endocrine
- Severity
- severe
- Chronic
- true
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Fatigue or weight changes
(endocrine)Significant symptoms that substantially affect daily functioning.
Frequency: Common at all stages
Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Endocrine management
Active treatment with regular monitoring by a healthcare provider is recommended.
Metastatic Disease Debulking
Surgical resection of metastatic lesions when feasible, particularly for solitary or limited metastatic deposits, combined with systemic therapies for comprehensive management.
Tyrosine Kinase Inhibitor Therapy
Targeted therapy using medications that inhibit tumor angiogenesis and growth pathways, particularly for progressive metastatic disease not amenable to other treatments.
Additional treatment options
Radiofrequency Ablation
Image-guided thermal ablation of localized metastatic tumors, particularly useful for bone metastases causing pain or threatening structural integrity.
procedureWhole-Body MRI Surveillance
Comprehensive imaging protocol using whole-body MRI to detect and monitor metastatic disease throughout the body without radiation exposure.
monitoringCatecholamine-Trigger Avoidance
Dietary and environmental modifications to avoid substances that can stimulate catecholamine release, including tyramine-rich foods, caffeine, and intense physical exertion.
dietaryWhen to seek medical help
Recognize when you need immediate medical attention.
See a doctor within 24 hours
Moderate to severe symptoms that may indicate worsening of this condition. Prompt medical evaluation is recommended.
Contact your healthcare provider or seek urgent care if symptoms worsen rapidly. Go to the emergency department for life-threatening symptoms.
Frequently asked questions
Common questions about this topic.
What is Pheochromocytoma Paraganglioma Syndrome 5 C3279992?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Lenders JW, Duh QY, Eisenhofer G, et al. Pheochromocytoma and Paraganglioma: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2014.
Favier J, Amar L, Gimenez-Roqueplo AP. Paraganglioma and phaeochromocytoma: from genetics to personalized medicine. Nat Rev Endocrinol. 2015.
Burnichon N, Cascón A, Schiavi F, et al. MAX mutations cause hereditary and sporadic pheochromocytoma and paraganglioma. Clin Cancer Res. 2012.
Jiang JH, Guo M, Luo H, et al. Comprehensive analysis of SDHx, VHL, and MAX genes in patients with pheochromocytoma and paraganglioma: A systematic review and meta-analysis. Endocr Relat Cancer. 2020.
Medication Comparisons
Compare medications used to treat Pheochromocytoma Paraganglioma Syndrome 5 C3279992. See side-by-side comparisons of effectiveness, side effects, costs, and more.
View Medication ComparisonsThis information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy