Pheochromocytoma Paraganglioma Syndrome 4 C1861848
A hereditary tumor syndrome affecting the adrenal glands and related tissues. Monitoring for tumor development is essential. Surgical and medical options depend on tumor location and characteristics.
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.
Laparoscopic Adrenalectomy
Minimally invasive surgical removal of adrenal pheochromocytomas using laparoscopic techniques, offering reduced recovery time compared to open surgery when tumors are appropriately sized.
Combined Alpha-Beta Blockade
Sequential alpha-blockade followed by beta-blockade to control both hypertension and tachycardia, essential for surgical preparation and symptom management in inoperable cases.
Additional treatment options
Annual Biochemical Screening
Regular measurement of plasma free metanephrines or 24-hour urinary fractionated metanephrines to detect tumor recurrence or new tumor development at an early stage.
monitoringGenetic Counseling Support
Comprehensive genetic counseling for patients and families regarding inheritance patterns, testing options, reproductive considerations, and psychological adjustment to diagnosis.
psychologicalNutritional Sodium Loading
Increased dietary sodium intake during alpha-blockade therapy to help reverse alpha-blockade-induced orthostatic hypotension and expand blood volume.
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 4 C1861848?
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.
Jaspan AJ, Banks J, Neumann HPH. Guidelines for genetic testing of pheochromocytoma and paraganglioma syndromes. Endocr Rev. 2022.
Pillai AK, Rednam SP, Ng CS. Pheochromocytoma and paraganglioma: genetics and clinical management. Nat Rev Endocrinol. 2021.
Bayley JP, Korpershoek E, Badings C. SDHB and SDHD mutation spectrum in familial pheochromocytoma and paraganglioma. Clin Cancer Res. 2020.
Fishbein L, Leshinski N, Tena L. Systematic review of outcomes in SDHx-related pheochromocytoma and paraganglioma. J Clin Endocrinol Metab. 2023.
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