Onco Wiki / Maladie

Lymphangioléiomiomatose (LAM)

Vue déterministe de l'entité source de la LJAM. L'autorité clinique demeure avec les ID de la source cités et l'état d'approbation de l ́examinateur.

IDDIS-LAM
TypeMaladie
Alias
LAMLymphangioléiomyomatoseLAM pulmonaireLAM sporadiqueLAM associé à la sclérose en tuberieЛімфангіолейоміоматоз (ЛАМ)
Étatreviewed 2026-05-04 | pending_clinical_signoff
MaladiesAucune déclaration
SourcesSRC-MILES-MCCORMACK-2011 SRC-NCCN-SARCOMA

Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.

Source YAML

icd_o_3_morphology8894/1
icd_o_3_topography["C34"]
icd_10J84.81
who_classificationWHO Classification of Thoracic Tumours 5th ed (2021): Lymphangioleiomyomatosis (PEComa family)

Capacités

Notes

LAM is a rare, progressive cystic lung disease caused by TSC1/TSC2 loss-of-function and consequent mTORC1 hyperactivation. Sirolimus (rapamycin) is FDA/EMA-approved for LAM (MILES trial, 2011: improved FEV1 trajectory, reduced VEGF-D). Everolimus is used for TSC-related manifestations including SEGA and renal AML. Disease is estrogen-sensitive: exacerbations during pregnancy, use of hormonal contraceptives discouraged. Diagnosis: high-resolution CT (diffuse bilateral thin-walled cysts) ± serum VEGF-D ≥800 pg/mL (diagnostic without biopsy in right clinical context). LAM Foundation guidelines (NHLBI/ERS) primary reference; NCCN does not cover LAM.

Utilisé par

Pertinence thérapeutique

Algorithmes

Indication

Questionnaires

Signal d’alerte