Pheochromocytoma Paraganglioma Syndrome 6 C5193112
A genetic syndrome causing tumors in the adrenal glands and paraganglia. Management involves regular imaging, tumor removal, and genetic counseling.
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
Bilateral Adrenal-Sparing Surgery
For patients requiring bilateral adrenalectomy, cortical-sparing techniques attempt to preserve adrenal function while removing tumorous tissue, reducing lifelong steroid dependency.
Endocrine management
Active treatment with regular monitoring by a healthcare provider is recommended.
Glucocorticoid Replacement Therapy
Lifelong adrenal hormone replacement with glucocorticoids for patients post-bilateral adrenalectomy, requiring stress-dose adjustments during illness or surgery.
Additional treatment options
Cardiovascular Monitoring
Regular echocardiography and blood pressure monitoring given the association with underlying predisposition to cardiomyopathy and vascular disease in this syndrome subtype.
monitoringCardiac Rehabilitation
Structured exercise program designed for patients with cardiovascular involvement, improving functional capacity while avoiding excessive catecholamine stimulation.
physical_therapyChronic Illness Coping Support
Psychological intervention addressing the chronic nature of hereditary cancer syndromes, including body image concerns, family guilt, and long-term health anxiety.
psychologicalWhen 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 6 C5193112?
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: Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2014;99(6):1915-1942.
Favier J, Amar L, Stratakis CA. The genetics of paragangliomas and pheochromocytomas. Nat Rev Endocrinol. 2018;14(10):587-598.
Burnichon N, Briere JJ, Libe R, et al. SDHA is a tumor suppressor gene in pheochromocytomas and paragangliomas. PLoS One. 2010;5(7):e10023.
Bayley JP, Kunst HP, Morreau H, et al. Systematic analysis of SDHx mutations in sporadic and familial pheochromocytomas and paragangliomas. Endocr Relat Cancer. 2012;19(3):455-465.
Medication Comparisons
Compare medications used to treat Pheochromocytoma Paraganglioma Syndrome 6 C5193112. 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