Pheochromocytoma Paraganglioma Syndrome 2 C1866552
An inherited condition increasing risk of neuroendocrine tumors. Family members may benefit from genetic testing. Surveillance and early detection guide 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.
Tumor Excision with Function Preservation
Surgical removal of pheochromocytoma or paraganglioma tumors with emphasis on organ-sparing techniques when appropriate. SDHB mutations may be associated with more aggressive tumors requiring complete excision with wider margins.
Preoperative Alpha Blockade Protocol
Systematic alpha-adrenergic blockade initiated well before any surgical or invasive procedure to prevent life-threatening hypertensive episodes during tumor manipulation. Phenoxybenzamine or alternative agents are commonly used.
Additional treatment options
Malignancy Surveillance Protocol
Enhanced monitoring protocol given higher malignant potential of SDHB-associated tumors, including periodic imaging of chest, abdomen, and pelvis alongside biochemical markers. May include FDG-PET scanning for early metastatic detection.
monitoringHypertensive Crisis Management
Emergency protocols and medications for acute blood pressure elevation, including intravenous phentolamine and nicardipine. Patients and caregivers should have access to emergency care plans and appropriate medical contacts.
medicationComprehensive Genetic Counseling Services
In-depth genetic counseling addressing SDHB mutation implications, inheritance patterns, family screening recommendations, and psychological support. Includes discussion of tumor risks and surveillance options for identified carriers.
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 2 C1866552?
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;99(6):1915-1942.
Burnichon N, Mazzella JM, Drui D, et al. Genetics of Pheochromocytoma Paraganglioma Syndromes: State of the Art. Ann Endocrinol. 2018;79(3):134-142.
Neumann HP, Erlic Z, Boedeker CC, et al. Clinical Predictors of Germline SDHx Mutations in Pheochromocytoma Paraganglioma Patients. Eur J Clin Invest. 2019;49(5):e13092.
Favier J, Amar L, Gimenez-Roqueplo AP. Paraganglioma and Phaeochromocytoma: From Genetics to Personalized Medicine. Nat Rev Endocrinol. 2015;11(2):101-107.
Medication Comparisons
Compare medications used to treat Pheochromocytoma Paraganglioma Syndrome 2 C1866552. 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