Pulmonary Intravascular Lymphomatosis: Clinical, CT, and PET Findings, Correlation of CT and Pathologic Results, and Survival Outcome

Abstract

).q RSNA, 2016 Purpose:To describe clinical, computed tomographic (CT), and positron emission tomographic (PET) features, correlation of CT and pathologic results, and survival of patients with pulmonary intravascular lymphomatosis. Materials and Methods:The institutional review board approved this retrospective study with waiver of patient consent. Forty-two patients with pulmonary intravascular lymphomatosis were identified, 11 (26%) of whom showed lung involvement. CT features were correlated with histopathologic results. Clinical and survival outcomes were compared between patients with and those without pulmonary involvement by adopting the x 2 , Student t, or Kaplan-Meier analysis with log-rank tests. Results:At clinical presentation, all 11 patients showed B symptoms (systemic symptoms of fever, night sweats, and weight loss), 10 had respiratory and four had neurologic symptoms, and two had skin lesions. Patients received cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy with (n = 5) or without (n = 6) rituximab, and seven (64%) patients died. Patients with lung involvement showed reduced overall and recurrence-free survival (median; 10.8 and 18.9 months, respectively) compared with those without lung involvement (median, 18.4 and 31.0 months, respectively) (P = .338 and .065, respectively). The most common CT abnormality was bilateral ground-glass opacity (GGO, n = 10), with increased fluorodeoxyglucose uptake at PET/CT (seven of seven patients). GGO correlated histopathologically with the expanded alveolar septal vasculatures and perivascular spaces filled with neoplastic lymphoid cells. Conclusion:Pulmonary intravascular lymphomatosis appeared as bilateral GGO on CT images, with increased fluorodeoxyglucose uptake on PET/CT images. GGO on CT images correlated with the area of expanded alveolar septae because of distended vessels filled with neoplastic lymphoid cells.q RSNA, 2016

Pulmonary Intravascular Large B-cell Lymphoma in a Patient Administered Methotrexate for Rheumatoid Arthritis

“…These CT features have been reported to correlate with thickened alveolar septa owing to the distension of small vessels filled with neoplastic lymphoid cells (19). The clinical, radiological, and pathological findings seen in our case were consistent with those described in previous reports (19)(20)(21)(22).…”

“…The common patterns of parenchymal abnormality seen on CT images include bilateral diffuse ground-glass opacity, and in a few cases, peribronchovascular and interlobular septal thickening, nodules, and consolidation (19)(20)(21)(22)(23). These CT features have been reported to correlate with thickened alveolar septa owing to the distension of small vessels filled with neoplastic lymphoid cells (19). The clinical, radiological, and pathological findings seen in our case were consistent with those described in previous reports (19)(20)(21)(22).…”

“…The main sites of involvement in patients with IVLBCL are the central nervous system, skin, bone marrow, and lymph nodes, and involvement of the lungs has also been re-ported (10,18,19). Clinical manifestations of IVLBCL in the lungs have included a fever, cough, hypoxemia, and progressive dyspnea (18)(19)(20)(21)(22)(23).…”

“…The main sites of involvement in patients with IVLBCL are the central nervous system, skin, bone marrow, and lymph nodes, and involvement of the lungs has also been re-ported (10,18,19). Clinical manifestations of IVLBCL in the lungs have included a fever, cough, hypoxemia, and progressive dyspnea (18)(19)(20)(21)(22)(23). The common patterns of parenchymal abnormality seen on CT images include bilateral diffuse ground-glass opacity, and in a few cases, peribronchovascular and interlobular septal thickening, nodules, and consolidation (19)(20)(21)(22)(23).…”

“…Clinical manifestations of IVLBCL in the lungs have included a fever, cough, hypoxemia, and progressive dyspnea (18)(19)(20)(21)(22)(23). The common patterns of parenchymal abnormality seen on CT images include bilateral diffuse ground-glass opacity, and in a few cases, peribronchovascular and interlobular septal thickening, nodules, and consolidation (19)(20)(21)(22)(23). These CT features have been reported to correlate with thickened alveolar septa owing to the distension of small vessels filled with neoplastic lymphoid cells (19).…”