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| Reference | Clinical features | Pathologic features | Treatment | Outcome |
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| Rehman et al. [13] | Syncope with central pulmonary artery filling defect in a 61-year-old woman | Grossly: tan, well-circumscribed mass within pulmonary trunk | N/A | Deceased during attempt at ultrasound-guided intravascular biopsy |
| Morsolini et al. [14] | Dry cough with FDG-avid mass infiltrating right pulmonary artery in a 62-year-old man | Grossly: white-yellow mass of solid, fleshy tissue.
Microscopically: large, histiocyte-like cells with emperipolesis in background of mature plasma cells, small lymphocytes, and foamy histiocytes | Pulmonary artery endarterectomy | Stable and disease-free at 9 months |
| Walters et al. [15] | Progressive dyspnea and lower extremity edema with FDG-avid masses nearly completely obstructing her main pulmonary artery, right pulmonary artery and left pulmonary artery in a 22-year-old woman | Gross: Tan-white, solid, finely granular specimen.
Microscopically: emperipolesis (in 5–10% of histiocytes) and histiocytic proliferation among an inflammatory infiltrate of plasma cells and lymphocytes set in a fibrous stroma | Debulking operation on cardiopulmonary bypass | No disease recurrence at 5 months from operation |
| Prendes et al. [16] | Progressive dyspnea on exertion with cor pulmonale and bilateral pulmonary artery narrowing due to a mediastinal mass in a 42-year-old woman | Microscopic: Two inconclusive biopsies revealing lymph and fibroadipose tissue, respectively, prior to successful biopsy with pathognomonic features of RDD | Median sternotomy with full cardiopulmonary bypass for resection and reconstruction of the great vessels with multiple grafts placed in the aorta and pulmonary artery | Normalization of right ventricular size with improved function and clinically stable without symptoms or recurrence at 12 months from operation |
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