Pheochromocytoma Paraganglioma Syndrome 1 C3494181
A hereditary syndrome predisposing to tumors of the adrenal glands and paraganglia. Requires lifelong monitoring. Treatment involves surgery and sometimes targeted therapies.
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
Surgical removal of pheochromocytoma or paraganglioma tumors, which is the primary curative approach. Requires careful preoperative preparation and experienced surgical teams familiar with these tumor types.
Alpha-Adrenoceptor Blockade
Medical management using alpha-blockers to control blood pressure and prevent catecholamine surge complications before and after tumor manipulation. Typically initiated 10-14 days before any surgical procedure.
Additional treatment options
Comprehensive Surveillance Protocol
Systematic screening for multiple tumors including periodic biochemical testing (plasma free metanephrines), imaging studies, and genetic re-evaluation. Since this is a hereditary syndrome, multiple tumors may develop over time.
monitoringBeta-Adrenergic Blockade
Adjunctive medication to control tachycardia and arrhythmias after adequate alpha-blockade is established. Helps manage symptoms of catecholamine excess including palpitations and sweating.
medicationGenetic Counseling and Family Testing
Specialized counseling services to discuss hereditary implications, available genetic testing, reproductive options, and psychological support for patients and families affected by SDHD mutations.
complementaryWhen 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 1 C3494181?
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 JWM, 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 pheochromocytoma: from genetics to personalized medicine. Nat Rev Endocrinol. 2015.
Burnichon N, Briere JJ, Libe R, et al. SDHA is a tumor suppressor gene causing paraganglioma. Hum Mol Genet. 2010.
Jochmanova I, Pacak K. Predictive factors for complications and long-term outcome in patients with pheochromocytoma/paraganglioma. Front Endocrinol. 2016.
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.
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Medically reviewed by WellAlly Editorial Team Last reviewed August 25, 2026. · Read our editorial policy