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Feocromocitoma y paraganglioma (PPGL)

Vista determinista de la entidad fuente YAML. La autoridad clínica sigue con los ID de fuente citados y estado de firma del revisor.

IDDIS-PHEOCHROMOCYTOMA
TipoEnfermedad
Aliaces
Tumor medular adrenalTumor de secretación de catecolaminaPPGLParagangliomaEl tratamiento con el medicamento se realizará de forma que se pueda obtener un tratamiento de la salud de los animales.Феохромоцитома та парагангліома (PPGL)
Situaciónreviewed 2026-05-19 | pending_clinical_signoff
EnfermedadesNinguna declarada
FuentesSRC-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.

Fuente 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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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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