Lymphangioleiomyomatosis (LAM)
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | DIS-LAM |
|---|---|
| Type | Disease |
| Aliases | LAMLymphangioleiomyomatosispulmonary LAMsporadic LAMtuberous sclerosis-associated LAMЛімфангіолейоміоматоз (ЛАМ) |
| Status | reviewed 2026-05-04 | pending_clinical_signoff |
| Diseases | None declared |
| Sources | SRC-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.
| icd_o_3_morphology | 8894/1 |
|---|---|
| icd_o_3_topography | ["C34"] |
| icd_10 | J84.81 |
| who_classification | WHO Classification of Thoracic Tumours 5th ed (2021): Lymphangioleiomyomatosis (PEComa family) |
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.
Used By
Actionability
BMA-TSC1-LAM- Sirolimus (rapamycin), an mTORC1 inhibitor, is FDA-approved (May 2015) and EMA-approved (...BMA-TSC1-TSC-RENAL-AML- Everolimus (mTOR inhibitor) is FDA-approved (2012) for renal angiomyolipoma (AML) associa...
Algorithms
ALGO-LAM-1L- ALGO-LAM-1L
Indication
IND-LAM-1L-SIROLIMUS- IND-LAM-1L-SIROLIMUS
Questionnaires
QUEST-LAM-1L-STUB- Lymphangioleiomyomatosis — first line
Red flag
RF-TSC-CONFIRMED-CARRIER- Confirmed germline pathogenic / likely-pathogenic variant in TSC1 (hamartin) or TSC2 (tub...