OpenOnco · Treatment Plan

Treatment plan — Lymphangioleiomyomatosis

PLAN-COVERAGE-LAM-001-V1 · v1 · 2026-05-13

Patient

COVERAGE-LAM-001 · Algorithm: ALGO-LAM-1L

DiagnosisLymphangioleiomyomatosis

MOH / ICD-10J84.81

ICD-O-38894/1; C34

Clinical significance of mutations (ESCAT)

Tumor-board context — the engine does not use these tiers to rank tracks

Biomarker	Variant	ESCAT	Evidence	Clinical significance	Drugs	Sources
No clinically actionable variants matched in this profile.

Primary current-line option

Standard plan

★ DEFAULT

Indication

IND-LAM-1L-SIROLIMUS

Regimen

Sirolimus monotherapy (progressive LAM)

Drugs + NSZU

Sirolimus (DRUG-SIROLIMUS) 2 mg PO once daily initially; titrate to target trough 5–15 ng/mL · Continuous; long-term treatment; monitor trough levels · PO ⚠ Out-of-pocket

Reason

Primary current-line option selected by ALGO-LAM-1L at step 2.

Pre-treatment investigations

Investigations before treatment start · critical / standard / desired · merged across tracks

ID	Name	Priority	Category	Where to order	Needed for
TEST-CBC	Complete Blood Count with Differential	Critical	lab	—	all tracks
TEST-CMP	Comprehensive Metabolic Panel	Critical	lab	—	all tracks

What NOT to do

Explicit prohibitive rules, each grounded in a regimen / supportive care / contraindication entity

Standard plan (IND-LAM-1L-SIROLIMUS)

Do NOT ignore sirolimus trough level monitoring — check 1–2 weeks post dose change; target 5–15 ng/mL
Do NOT initiate during active infections — mTOR inhibition impairs immune defense
Do NOT ignore new respiratory symptoms — non-infectious pneumonitis is a class effect
Do NOT co-administer with grapefruit juice or strong CYP3A4 inhibitors/inducers — markedly alters drug levels
Do NOT use during pregnancy — teratogenicity

Timeline

Treatment timeline — derived from regimen + monitoring schedule

Standard plan

Induction · Sirolimus monotherapy (progressive LAM)

28-day cycles × Long-term continuous

MDT brief

Discussion questions (1, 1 blocking)

BLOCKING OQ-BIOMARKER-TSC1
What is the status of TSC1 loss-of-function mutation (hamartin) (BIO-TSC1)? It is required by track(s): IND-LAM-1L-SIROLIMUS. Expected value: TSC1 or TSC2 mutation (diagnostic of LAM in appropriate context; identifies mTOR-activating mutation — not required for eligibility when clinical + CT diagnosis is established).
A treatment-track biomarker requirement is missing from the patient profile; the MDT should verify the test result, method, specimen, and date before relying on this option.
→ molecular_geneticist

MDT talk tree (2 steps)

#	Owner	Topic	Action
1	molecular_geneticist	Biomarker status BLOCKING	What is the status of TSC1 loss-of-function mutation (hamartin) (BIO-TSC1)? It is required by track(s): IND-LAM-1L-SIROLIMUS. Expected value: TSC1 or TSC2 mutation (diagnostic of LAM in appropriate context; identifies mTOR-activating mutation — not required for eligibility when clinical + CT diagnosis is established).
2	social_worker_case_manager	Specialist review	Plan includes drugs without NSZU reimbursement — patient access pathway must be assessed.

Skills (recommended) — for consideration (2)

Molecular geneticist / molecular oncologist recommended
Indication references an actionable genomic biomarker — mutation / target / actionability interpretation needed.
Owns: OQ-BIOMARKER-TSC1
Social worker / case manager recommended
Plan includes drugs without NSZU reimbursement — patient access pathway must be assessed.

Data quality

Incomplete for default-track review. Default-track review is incomplete until required biomarker gaps are resolved.

Biomarker coverage: 0/1 known (0%), 1 missing, 1 default-track gaps

Missing biomarker	Label	MDT owner	Default track	Required by	Next action
`BIO-TSC1`	TSC1 loss-of-function mutation (hamartin)	molecular_geneticist	yes	IND-LAM-1L-SIROLIMUS	Verify result, method, specimen, and report date before sign-off. Expected/constraint: TSC1 or TSC2 mutation (diagnostic of LAM in appropriate context; identifies mTOR-activating mutation — not required for eligibility when clinical + CT diagnosis is established)

Technical MDT skill metadata (2/16 activated in this plan)

All registered virtual specialists. ✓ — activated for this case; ○ — not activated (available for other clinical scenarios).

Specialist	skill_id	Version	Last reviewed	Domain
Cellular therapy specialist (CAR-T)	`cellular_therapy_specialist`	v0.1.0	2026-04-25	cellular_therapy
Clinical pharmacist	`clinical_pharmacist`	v0.1.0	2026-04-25	clinical_pharmacy
Hematologist / oncohematologist	`hematologist`	v0.1.0	2026-04-25	hematology_oncology
Hematopathologist (lymphoma / leukemia / myeloma)	`hematopathologist`	v0.1.0	2026-04-25	hematopathology
Infectious disease / hepatology	`infectious_disease_hepatology`	v0.1.0	2026-04-25	infectious_diseases
Medical oncologist (solid-tumor chemotherapist)	`medical_oncologist`	v0.1.0	2026-04-25	solid_oncology
Molecular geneticist / molecular oncologist	`molecular_geneticist`	v0.1.0	2026-04-25	molecular_oncology
Palliative care	`palliative_care`	v0.1.0	2026-04-25	palliative_care
Pathologist (general)	`pathologist`	v0.1.0	2026-04-25	pathology
Primary care / family physician	`primary_care`	v0.1.0	2026-04-25	primary_care
Psycho-oncologist	`psychologist`	v0.1.0	2026-04-25	psychosocial
Radiation oncologist	`radiation_oncologist`	v0.1.0	2026-04-25	radiation_oncology
Radiologist	`radiologist`	v0.1.0	2026-04-25	diagnostic_imaging
Social worker / case manager	`social_worker_case_manager`	v0.1.0	2026-04-25	psychosocial
Surgical oncologist	`surgical_oncologist`	v0.1.0	2026-04-25	surgical_oncology
Transplant specialist (BMT)	`transplant_specialist`	v0.1.0	2026-04-25	cellular_therapy

Sources cited

SRC-MILES-MCCORMACK-2011: MILES: Sirolimus for Lymphangioleiomyomatosis (McCormack et al., NEJM 2011) ()

Experimental options (clinical trials)

Third plan track — open-enrollment trials from ClinicalTrials.gov. Render-time metadata; engine selection is not affected by this block (CHARTER §8.3). Last synced: 2026-05-13.

NCT	Title	Phase	Status	Sponsor	UA	Signals
NCT06160310	Tuberous Sclerosis Complex and Lymphangioleiomyomatosis Pregnancy Registry (TSC-LAM Registry)	N/A	RECRUITING	David M. Ritter	—	Single country
NCT00001465	Study of the Disease Process of Lymphangioleiomyomatosis	N/A	RECRUITING	National Heart, Lung, and Blood Institute (NHLBI)	—	Single country
NCT06889168	Evaluating the Long-term Safety and Tolerability of Imatinib in Patients With Lymphangioleiomyomatosis (LAM)	PHASE1	RECRUITING	Columbia University	—	Phase 1 only Small N (<50) Single country
NCT07304856	Role of Extracellular Vesicles as Biomarkers of Pulmonary Involvement in Patients With Lymphangioleiomyomatosis and Tuberous Sclerosis Complex	N/A	RECRUITING	University of Milan	—	Single country
NCT02432560	Safety and Durability of Sirolimus for Treatment of LAM	N/A	RECRUITING	University of Cincinnati	—	Single country
NCT03193892	A National Registry on Chinese Patients With Lymphangioleiomyomatosis	N/A	RECRUITING	Peking Union Medical College Hospital	—	Single country
NCT06405997	The Genotype and Phenotype of Lymphangioleiomyomatosis	N/A	RECRUITING	National Taiwan University Hospital	—	Single country
NCT05676099	TSC Biosample Repository and Natural History Database	N/A	RECRUITING	National Tuberous Sclerosis Association	—	—
NCT01484236	National Lymphangioleiomyomatosis Registry, France	N/A	RECRUITING	Vincent COTTIN	—	Single country
NCT01799538	Nebulized or Inhaled Albuterol for Lymphangioleiomyomatosis	PHASE1 / PHASE2	RECRUITING	National Heart, Lung, and Blood Institute (NHLBI)	—	Single country

Verify recruitment status directly with the trial site. ctgov data can lag behind current UA-site status.

Option availability in Ukraine

Per-track UA registration · NSZU · cost · access pathway. Render-time metadata; engine selection does not depend on these fields (CHARTER §8.3).

Option	UA registration	NSZU	Cost orientation	Access pathway
Standard plan Sirolimus monotherapy (progressive LAM) (REG-SIROLIMUS-LAM) 1/1 component drug(s) not on NSZU formulary	✓ registered	✗ out-of-pocket	₴-? — verify pathway	not recorded
Trial · NCT06160310 Tuberous Sclerosis Complex and Lymphangioleiomyomatosis Pregnancy Registry (TSC-LAM Registry) No UA site listed — international referral required	— unknown	— unknown	self-pay: ₴0/course	Trial sponsor
Trial · NCT00001465 Study of the Disease Process of Lymphangioleiomyomatosis No UA site listed — international referral required	— unknown	— unknown	self-pay: ₴0/course	Trial sponsor
Trial · NCT06889168 Evaluating the Long-term Safety and Tolerability of Imatinib in Patients With Lymphangioleiomyomatosis (LAM) No UA site listed — international referral required	— unknown	— unknown	self-pay: ₴0/course	Trial sponsor
Trial · NCT07304856 Role of Extracellular Vesicles as Biomarkers of Pulmonary Involvement in Patients With Lymphangioleiomyomatosis and Tuberous Sclerosis Complex No UA site listed — international referral required	— unknown	— unknown	self-pay: ₴0/course	Trial sponsor
Trial · NCT02432560 Safety and Durability of Sirolimus for Treatment of LAM No UA site listed — international referral required	— unknown	— unknown	self-pay: ₴0/course	Trial sponsor
Trial · NCT03193892 A National Registry on Chinese Patients With Lymphangioleiomyomatosis No UA site listed — international referral required	— unknown	— unknown	self-pay: ₴0/course	Trial sponsor
Trial · NCT06405997 The Genotype and Phenotype of Lymphangioleiomyomatosis No UA site listed — international referral required	— unknown	— unknown	self-pay: ₴0/course	Trial sponsor
Trial · NCT05676099 TSC Biosample Repository and Natural History Database No UA site listed — international referral required	— unknown	— unknown	self-pay: ₴0/course	Trial sponsor
Trial · NCT01484236 National Lymphangioleiomyomatosis Registry, France No UA site listed — international referral required	— unknown	— unknown	self-pay: ₴0/course	Trial sponsor
Trial · NCT01799538 Nebulized or Inhaled Albuterol for Lymphangioleiomyomatosis No UA site listed — international referral required	— unknown	— unknown	self-pay: ₴0/course	Trial sponsor

Cost information is orientation. Verify with a specific pharmacy / foundation / trial site. Status updated: 2026-05-13.

plan_id: PLAN-COVERAGE-LAM-001-V1 | version: 1 | generated: 2026-05-13T10:01:51.316437+00:00

Per FDA non-device CDS positioning (CHARTER §15): Outpatient oncology treatment planning to support tumor-board discussion. Not a medical device; not for autonomous clinical decision-making.

Per FDA non-device CDS positioning (CHARTER §15): Both treatment options below are presented for review. The engine's selection of a 'recommended' default does not constitute a clinical decision. Final treatment selection requires HCP judgment incorporating information not available to the system.

This document is an informational resource supporting tumor-board discussion (per CHARTER §11). It is not a system that makes clinical decisions. Every recommendation must be verified by the treating physician.