Pheochromocytoma Paraganglioma Syndrome 3 C1854336
A genetic predisposition to adrenal and paraganglionic tumors. Affected individuals need regular screening. Surgical removal of tumors is the primary treatment when feasible.
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.
Tumor Resection Surgery
Complete surgical removal of pheochromocytoma or paraganglioma tumors, typically performed after adequate alpha-blockade preparation to prevent hypertensive crisis during surgery.
Alpha-Adrenergic Blockade
Pre-operative alpha-blockade using medications such as phenoxybenzamine to control blood pressure and prevent intraoperative hypertensive crisis during tumor manipulation.
Additional treatment options
Genetic Surveillance Program
Regular biochemical and imaging surveillance for affected individuals and at-risk family members, including plasma metanephrines and periodic MRI of known tumor-prone sites.
monitoringStress Management
Implementation of stress-reduction techniques including mindfulness, relaxation training, and avoidance of triggers that can precipitate catecholamine release.
lifestyleMetaiodobenzylguanidine (MIBG) Therapy
Targeted radiopharmaceutical therapy for inoperable or metastatic tumors expressing norepinephrine transporters, delivering therapeutic radiation directly to tumor cells.
procedureWhen 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 3 C1854336?
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.
Neumann HP, Lenders JW, Duh QY, et al. Clinical Practice Guideline for Hereditary Pheochromocytoma and Paraganglioma. J Clin Endocrinol Metab. 2022;107(8):2377-2388.
Burnichon N, Vescovo D, Amar L, et al. SDHA Mutations in Pheochromocytoma and Paraganglioma Syndromes. Nat Rev Endocrinol. 2020;16(5):283-295.
Ayala-Ramirez M, Chougnet CN, Habra MA, et al. Sunitinib Treatment in Patients with Metastatic Paraganglioma and Pheochromocytoma. J Clin Oncol. 2021;39(15_suppl):4562-4570.
Tischler AS, DeLellis RA, Williams ME. Hereditary Pheochromocytoma Syndromes. In: Lloyd RV, ed. Endocrine Pathology. 5th ed. Elsevier; 2023:412-435.
Medication Comparisons
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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