Fibrosing Mediastinitis: Managing Active Symptoms and Progressive Mediastinal Compression After Rituximab Has Not Achieved Disease Control
This protocol addresses patients with fibrosing mediastinitis who present with ongoing symptoms or significant progressive compression of vital mediastinal structures, and in whom prior treatment with Rituximab did not achieve adequate control of the disease.
Clinical Scenario
Patients with symptoms of fibrosing mediastinitis, or significant progressive compression of vital mediastinal structures, who require escalation of care. In this population, therapies are typically divided into medical versus interventional approaches.
Prior Treatment Failure
The previous treatment line — Rituximab — did not achieve the required goals: reduction in symptom burden, reduction in PET avidity, reduction in lesion volume, and no evidence of disease progression. This protocol defines the structured next step following that failure.
Treatment goals: Improvement in WHO functional class, six-minute walk test, and clinical symptoms.
References
DOI: 10.1007/s13665-025-00382-3
- In patients with symptoms or significant progressive compression of vital structures, therapies are typically divided into medical versus interventional.
- Non-surgical interventions include either pulmonary artery and/or endobronchial angioplasty and stenting.
- Surgical interventions can include excision of tissue via pneumonectomy or lobectomy or even surgical debulking/decompression.
- In patients with SVC syndrome, SVC bypass, or vascular reconstruction using saphenous spiral vein graft, polytetrafluoroethylene (PTFE) or Dacron graft have been used.
- Less commonly performed are pulmonary vein balloon angioplasty; the largest study examining pulmonary vein stenosis/angioplasty involved 31 patients, with noted improvement in WHO functional class, six-minute walk test, and clinical symptoms.