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Linfangioleiomiomatosis (LAM)

Vista determinista de la entidad fuente YAML. La autoridad clínica sigue con los ID de fuente citados y estado de firma del revisor.

IDDIS-LAM
TipoEnfermedad
Aliaces
LAMLinfangioleiomiomatosisLAM pulmonarEVALUACIÓN ESporádicaLAM asociada a la esclerosis tuberosaЛімфангіолейоміоматоз (ЛАМ)
Situaciónreviewed 2026-05-04 | pending_clinical_signoff
EnfermedadesNinguna declarada
FuentesSRC-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.

Fuente 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)

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Notas

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.

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