Pheochromocytoma Paraganglioma Syndrome 7 C5193116
An inherited disorder increasing risk for neuroendocrine tumors. Patients need ongoing monitoring and coordinated care involving surgery and hormonal management.
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.
Surgical tumor resection
Complete surgical removal of pheochromocytoma or paraganglioma tumors is the primary curative treatment approach. Preoperative alpha-blockade is essential to control blood pressure and prevent hypertensive crisis during surgery. Laparoscopic techniques are preferred when anatomically feasible.
Alpha-blockade therapy
Alpha-adrenergic blockers such as phenoxybenzamine are initiated 10-14 days before surgery to control hypertension and prevent catecholamine surge complications. Beta-blockers may be added afterward for tachycardia control.
Additional treatment options
Long-term surveillance program
Regular biochemical testing (plasma metanephrines, urinary catecholamines) and imaging studies are essential for detecting tumor recurrence or metachronous lesions. Surveillance protocols are tailored based on genetic mutation status and tumor characteristics.
monitoringGenetic counseling services
Comprehensive genetic counseling is recommended for patients and family members due to the hereditary nature of this syndrome. Counseling addresses inheritance patterns, risk assessment, and implications for family planning.
psychologicalEmergency preparedness planning
Patients are educated on recognizing signs of catecholamine crisis and maintaining emergency medical information. Avoidance of triggers such as certain medications, physical stress, and extreme temperatures is emphasized.
lifestyleWhen 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 7 C5193116?
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, Buffet A. Molecular Genetics of Pheochromocytoma and Paraganglioma Syndromes. Ann Endocrinol. 2022.
Burnichon N, Mazzella JM, Billharz C. The Genetics of Paraganglioma and Pheochromocytoma Syndromes. Endocrinol Metab Clin North Am. 2017.
Bayley JP, van der Tuin K, Hannema G. Clinical Presentation and Genetics of MAX-associated Pheochromocytoma/Paraganglioma. Fam Cancer. 2020.
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