Sporadic pheochromocytoma/secreting…

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Sporadic 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

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

DNMT3ADisease-causing germline mutation(s) (gain of function)
EPAS1Disease-causing somatic mutation(s)
RETMajor susceptibility factor
SDHBMajor susceptibility factor
SDHDMajor susceptibility factor
VHLMajor susceptibility factor

ICD-10 codes

C74.1filed under a broader ICD-10 category — shared with 1 other rare disease
D35.0filed under a broader ICD-10 category — shared with 2 other rare diseases

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

MONDO 0017190UMLS C4707333

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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