Paraganglioma (PGL)
Visão determinística da entidade fonte YAML. A autoridade clínica permanece com os IDs de fonte citados e estado de assinatura do revisor.
| ID | DIS-PARAGANGLIOMA |
|---|---|
| Tipo | Doença |
| Alegais | Quemodectoma (histórico)Paraganglioma extra-adrenalTumor glótico (histórico, cabeça/colo)Paraganglioma de cabeça e pescoço (HNPGL)PGLParagangliomaParaganglioma torácico-abdominalПарагангліома (PGL) |
| Estado | reviewed 2026-05-20 | pending_clinical_signoff |
| Doenças | Nenhuma declaração |
| Fontes | SRC-NCCN-GENETIC-FAMILIAL-BREAST-OVARIAN-2025 |
Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.
| icd_o_3_morphology | 8693/1 |
|---|---|
| icd_o_3_topography | ["C75.4", "C75.5", "C49"] |
| icd_10 | D44.7 |
| who_classification | WHO Endocrine Tumours 5th ed (2022): Paraganglioma — extra-adrenal counterpart of pheochromocytoma; subtypes by anatomic site (head/neck = parasympathetic, non-secreting; thoracoabdominal = sympathetic, often catecholamine-secreting) |
Notas
Distinct from pheochromocytoma (DIS-PHEOCHROMOCYTOMA) by anatomic site (PGL = extra-adrenal). Treatment philosophy: complete surgical resection where feasible; malignant/unresectable disease — somatostatin- analog (lutathera 177Lu-DOTATATE) per ESMO/ENS@T; tyrosine kinase inhibitors (cabozantinib, sunitinib) in clinical trials for SDHx- mutant unresectable PGL; 131I-MIBG for MIBG-avid disease (limited efficacy in SDHB-mutant). Hereditary testing recommended for ALL PGL diagnoses regardless of age or family history.
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