がん診断ジャーナル

抽象的な

Intravascular Lymphoma: Look for the Less Obvious!

Antonella Bianchi, Lorenza Falcone, Lorenzo Ricci, Mario Tombini, Ombretta Annibali

Intravascular large B cell lymphoma (IVBCL) is a rare subtype of extranodal large B-cell lymphoma with a distinct presentation. Anatomically the disease is characterized by the proliferation of clonal lymphocytes within small vessels with relative sparing of the surrounding tissue. The clinical symptoms of the disease are dependent on the specific organ involvement, which often includes the central nervous system and skin. Because of the various modes of presentation and the rarity of IVBCL, the diagnosis is challenging and made post-mortem in most cases. The tumor is often disseminated at the time of diagnosis and prognosis is poor, even with aggressive chemotherapy. The spectrum of neurological presentations of IVBCL can be heterogeneous. We report a case of intravascular lymphoma diagnosed on autopsy.