Paraganglioma (PGL)
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | DIS-PARAGANGLIOMA |
|---|---|
| Type | Disease |
| Aliases | Chemodectoma (historical)Extra-adrenal paragangliomaGlomus tumor (historical, head/neck)Head and neck paraganglioma (HNPGL)PGLParagangliomaThoracoabdominal paragangliomaПарагангліома (PGL) |
| Status | reviewed 2026-05-20 | pending_clinical_signoff |
| Diseases | None declared |
| Sources | 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) |
Notes
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.
Used By
No reverse references found in the YAML corpus.