Onco Wiki / Disease

Pheochromocytoma and paraganglioma (PPGL)

Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.

IDDIS-PHEOCHROMOCYTOMA
TypeDisease
Aliases
Adrenal medullary tumorCatecholamine-secreting tumorPPGLParagangliomaPheochromocytomaФеохромоцитома та парагангліома (PPGL)
Statusreviewed 2026-05-19 | pending_clinical_signoff
DiseasesNone declared
SourcesSRC-NCCN-GENETIC-FAMILIAL-BREAST-OVARIAN-2025 SRC-NCCN-NET-2025

Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.

Source YAML

icd_o_3_morphology8700/0
icd_o_3_topography["C74.1"]
icd_10D35.0
who_classificationWHO Endocrine and Neuroendocrine Tumours 5th ed (2022): Pheochromocytoma and extra-adrenal paragangliomas

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Notes

Pheochromocytoma is a catecholamine-secreting neuroendocrine tumor of adrenal medullary origin; extra-adrenal counterparts (paragangliomas) arise from sympathetic / parasympathetic ganglia (head/neck, thoracic, abdominal). They are biologically and clinically a single spectrum (PPGL); this entity is anchored to the adrenal subset for ICD-10 cleanliness but cross-references SDHx-driven extra-adrenal paragangliomas in prevention workstream notes. Hereditary fraction is the highest known among adult cancers (~30–40%). NCCN and Endocrine Society recommend germline panel testing (RET, VHL, SDHB/C/D/AF2, NF1, TMEM127, MAX, FH) for ALL patients with PPGL — not restricted by family history. This makes PPGL a sentinel disease for hereditary cancer surveillance pathways (RET → MEN2 prophylactic thyroidectomy; VHL → multi-organ surveillance; SDHB → lifetime PPGL + RCC surveillance). Classical clinical triad: headache + palpitations + sweating; many patients present with paroxysmal or sustained hypertension. Biochemical diagnosis: plasma free metanephrines or 24-h urinary fractionated metanephrines (preferred over catecholamines). Functional imaging (MIBG, Ga-68 DOTATATE PET) for staging and...

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