Non-functioning paraganglioma
Synonyms: Non-secreting paraganglioma
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adolescent, Adult, Childhood, Elderly
- Status
- active
A rare neuroendocrine tumor arising from neural crest-derived paraganglion cells (most often in the para-aortic region at the level of renal hilia, organ of Zuckerkandl, thoracic paraspinal region, bladder, and carotid body) not associated with catecholamine secretion. These tumors are usually clinically silent and symptoms, if present, are nonspecific and depend on the location of the tumor. Association with certain hereditary cancer-predisposing syndromes, such as multiple endocrine neoplasia, neurofibromatosis type 1 or von Hippel lindau syndrome, may be observed.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Non-functioning paraganglioma | Non-secreting paraganglioma |
| fr | Paragangliome non sécrétant | |
| de | Paragangliom, nicht-funktionelles | Paragangliom, nicht-sezernierendes |
| es | Paraganglioma no funcionante | Paraganglioma no secretante |
| it | Paraganglioma non secernente | |
| nl | Niet-functioneel paraganglioom | Niet-secreterend paraganglioom |
| pt | Paraganglioma não-secretor | |
| pl | Przyzwojak nieczynny hormonalnie | = en |
| cs | Nesekretující paragangliom | = en |
| uk | Нефункціонуюча парагангліома | Несекретуюча парагангліома |
| zh | 无功能性副神经节瘤 | 非分泌性副神经节瘤 |
| tr | İşlevsel olmayan paraganglioma | Salgılamayan paragangliom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D44.7 | NTBT | attributed | validated |
| ICD-11 | 2F9A | NTBT | attributed | validated |
| MONDO | 0019788 | E | validated | |
| UMLS | C4707263 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:573163Pheochromocytoma-paraganglioma
rare endocrine diseases; rare neoplastic diseases
No children.
Codes that Orphanet moved or referred to this one:
- ORPHA:101106OBSOLETE: Non-secreting chemodectoma referred to · inactive
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
31 annotations · validated 2018-01-29| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0002668 | Paraganglioma | |
| Frequent (79-30%) | ||
| HP:0001342 | Cerebral hemorrhage | |
| HP:0100749 | Chest pain | |
| HP:0001618 | Dysphonia | |
| HP:0011979 | Elevated urinary dopamine | |
| HP:0003639 | Elevated urinary epinephrine | |
| HP:0003345 | Elevated urinary norepinephrine | |
| HP:0002574 | Episodic abdominal pain | |
| HP:0001069 | Episodic hyperhidrosis | |
| HP:0012378 | Fatigue | |
| HP:0031284 | Flushing | |
| HP:0003072 | Hypercalcemia | |
| HP:0002640 | Hypertension associated with pheochromocytoma | |
| HP:0001095 | Hypertensive retinopathy | |
| HP:0002018 | Nausea | |
| HP:0001962 | Palpitations | |
| HP:0002864 | Paraganglioma of head and neck | |
| HP:0010532 | Paroxysmal vertigo | |
| HP:0003574 | Positive regitine blocking test | |
| HP:0008629 | Pulsatile tinnitus | |
| HP:0002331 | Recurrent paroxysmal headache | |
| HP:0011703 | Sinus tachycardia | |
| HP:0001824 | Weight loss | |
| Occasional (29-5%) | ||
| HP:0000405 | Conductive hearing impairment | |
| HP:0001635 | Congestive heart failure | |
| HP:0001293 | Cranial nerve compression | |
| HP:0000790 | Hematuria | |
| HP:0000980 | Pallor | |
| HP:0025269 | Panic attack | |
| HP:0001337 | Tremor | |
| HP:0001605 | Vocal cord paralysis | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 17200167[PMID]_24893135[PMID]_19628817[PMID]_22948026[PMID]_17102067[PMID]_9509062[PMID]_17940110[PMID]_12000821[PMID]_17940110[PMID]_20503330[PMID]_12574179[PMID]_11502790[PMID]_9509062[PMID]_5015920[PMID]_20260947[PMID]_4340974[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/94080
- This record as JSON: /data/d/94080.json