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Start free with EleplanSporadic pheochromocytoma/secreting paraganglioma
ORPHA:276621Disease
What it is
A rare, isolated, non-familial pheochromocytoma/paraganglioma tumor arising from neuroendocrine chromaffin cells of the adrenal medulla (pheochromocytoma) or from extra-adrenal chromaffin tissue (paraganglioma). The majority of these tumors are benign and the presenting symptoms are typically caused by the increased catecholamine production of the tumor, including hypertension (often paroxysmal), tachycardia, anxiety and/or excessive sweating.
Key facts
- Age of onset
- Adolescent, Adult, Childhood
- Inheritance
- Not applicable
- Classified as
- Disease
Signs and symptoms
Common30–79%
28- Cerebral hemorrhage
- Chest pain
- Dysphonia
- Elevated urinary dopamine
- Elevated urinary epinephrine
- Elevated urinary norepinephrine
- Episodic abdominal pain
- Episodic hyperhidrosis
- Episodic paroxysmal anxiety
- Fatigue
- Flushing
- Glomerulosclerosis
- Hypercalcemia
- Hypertension
- Hypertension associated with pheochromocytoma
- Hypertensive retinopathy
- Nausea
- Orthostatic hypotension
- Palpitations
- Paraganglioma of head and neck
- Paroxysmal vertigo
- Positive regitine blocking test
- Proteinuria
- Pulsatile tinnitus
- Recurrent paroxysmal headache
- Sinus tachycardia
- Syncope
- Weight loss
Sometimes5–29%
15- Abdominal mass
- Cardiomyopathy
- Conductive hearing impairment
- Congestive heart failure
- Constipation
- Cranial nerve compression
- Hematuria
- Hyperglycemia
and 7 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Genes
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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