Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Elderly
- Status
- active
A rare pulmonary neuroendocrine tumor, most often presenting in women over 50 years and no smokers, characterized by long-lasting cough (>10 years), and less frequently by dispnea or wheezing. A proportion of patients are asymptomatic, the disease being an incidental finding during routine check-ups. Diffuse pulmonary nodules and mosaic attenuation can be detected by CT-scan however only histopathology can confirm the diagnosis detecting a multifocal hyperplasia of pulmonary neuroendocrine cells (carcinoid tumorlets). Ventilatory dysfunction can be obstructive and less often restrictive, mixed or non-specific. Symptoms can progress slowly but sometimes lead to respiratory failure. It is considered as a precursor for pulmonary carcinoid tumor.
Names in Orphadata languages
10 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia | |
| fr | Hyperplasie neuroendocrine pulmonaire diffuse idiopathique | |
| de | Diffuse idiopathische pulmonale neuroendokrine Zellhyperplasie | DIPNECH |
| es | Hiperplasia difusa idiopática de células neuroendocrinas pulmonares | |
| it | Iperplasia diffusa idiopatica delle cellule neuroendocrine del polmone | |
| nl | Diffuse idiopathische pulmonale hyperplasie van neuro-endocriene cellen | |
| pt | Hiperplasia difusa idiopática das células neuroendócrinas pulmonares | |
| pl | Rozlany idiopatyczny przerost komórek neuroendokrynnych płuc | |
| cs | Difuzní idiopatická hyperplázie plicních neuroendokrinních buněk | |
| uk | Дифузна ідіопатична нейроендокринна клітинна гіперплазія легень |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C34.9 | NTBT | attributed | validated |
| ICD-11 | 2F00.Y | NTBT | index | validated |
| MONDO | 0022986 | E | validated | |
| UMLS | C1333291 | E | validated | |
| GARD | 10780 | E | validated | |
| MedDRA | 10082591 | 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:101945Rare bronchopulmonary and pleural cavity tumors
rare respiratory diseases; rare neoplastic diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 100 cases | Worldwide | Case(s) | Validated | 30593169[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | 30593169[PMID]_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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/617916
- This record as JSON: /data/d/617916.json