. 2024 Nov 26;25:416. doi: 10.1186/s12931-024-03040-5

Table 2.

treatments and outcome

Case	Patient 1	Patient 2	Patient 3	Patient 4	Patient 5	Patient 6	Patient 7	Patient 8	Patient 9	Patient 10	Patient 11	Patient 12
Therapy	Sirolimus	NIV, lung-transplantation	Surgery with mediastinal cyst removal and thoracic duct ligation	Thoracoscopic pericardial fenestration, removal of the mediastinal tumor, Ductus thoracic ligature and talcum pleurodesis	Thoracoscopic pericardial fenestration, followed by sirolimus one months later which was paused after the patient became pregnant	Thoracoscopic talcum pleurodesis, thoracic duct ligation without success, followed by thoracic radiation due to persistent chylothorax and sirolimus	Sirolimus	Thoracoscopic talcum pleurodesis, thoracic radiation, sirolimus, oxygen therapy	Sirolimus	Sirolimus recommended	Sirolimus + propranolol only for 9 months Followed by pericardiectomy, adhesiolysis and talcum pleurodesis due to deterioration, continuation with sirolimus	Sirolimus
Outcome	Clinical and radiological improvement after 3 months of therapy with sirolimus, stable disease in the following 25 months, afterwards loss of follow up	Death 8 years after diagnosis due to a complication after lung transplantation which was performed due to a progressive disease	Loss of follow-up	1 month after surgery no symptoms anymore, afterwards loss of follow up	Improvement of pericardial effusion 12 months after sirolimus therapy, stable disease during pregnancy and time afterwards until now (another 26 months)	Clinical and radiological improvement and stabilization 6 months of sirolimus, treatment for 45 months, afterwards loss of follow up	Clinical and radiological improvement for 11 months after initiating sirolimus, loss of follow up after a treated infection	Regressive chylothorax after 3 months of sirolimus treatment, discontinuation due to phlebitis for one months, followed by reuptake, afterwards loss of follow up	Clinical improvement, discreet progression in CT scan (initiation of sirolimus was delayed to concerns of the patient in terms of adverse event and started after progression of symptoms)	Stable situation for 3 months, then loss of follow-up	Clinical and radiological improvement for approx. 3 months; after combining pharmacological and surgical treatments improvement of symptoms, lung function, X-ray findings and 6MWT	Stable clinical and radiological situation after 6 months

Case

Patient 1

Patient 2

Patient 3

Patient 4

Patient 5

Patient 6

Patient 7

Patient 8

Patient 9

Patient 10

Patient 11

Patient 12

Therapy

Sirolimus

NIV, lung-transplantation

Surgery with mediastinal cyst removal and thoracic duct ligation

Thoracoscopic pericardial fenestration, removal of the mediastinal tumor, Ductus thoracic ligature and talcum pleurodesis

Thoracoscopic pericardial fenestration, followed by sirolimus one months later which was paused after the patient became pregnant

Thoracoscopic talcum pleurodesis, thoracic duct ligation without success, followed by thoracic radiation due to persistent chylothorax and sirolimus

Sirolimus

Thoracoscopic talcum pleurodesis, thoracic radiation, sirolimus, oxygen therapy

Sirolimus

Sirolimus recommended

Sirolimus + propranolol only for 9 months

Followed by pericardiectomy, adhesiolysis and talcum pleurodesis due to deterioration, continuation with sirolimus

Sirolimus

Outcome

Clinical and radiological improvement after 3 months of therapy with sirolimus, stable disease in the following 25 months, afterwards loss of follow up

Death 8 years after diagnosis due to a complication after lung transplantation which was performed due to a progressive disease

Loss of follow-up

1 month after surgery no symptoms anymore, afterwards loss of follow up

Improvement of pericardial effusion 12 months after sirolimus therapy, stable disease during pregnancy and time afterwards until now (another 26 months)

Clinical and radiological improvement and stabilization 6 months of sirolimus, treatment for 45 months, afterwards loss of follow up

Clinical and radiological improvement for 11 months after initiating sirolimus, loss of follow up after a treated infection

Regressive chylothorax after 3 months of sirolimus treatment, discontinuation due to phlebitis for one months, followed by reuptake, afterwards loss of follow up

Clinical improvement, discreet progression in CT scan (initiation of sirolimus was delayed to concerns of the patient in terms of adverse event and started after progression of symptoms)

Stable situation for 3 months, then loss of follow-up

Clinical and radiological improvement for approx. 3 months; after combining pharmacological and surgical treatments improvement of symptoms, lung function, X-ray findings and 6MWT

Stable clinical and radiological situation after 6 months