RESUMO
Central nervous system involvement by mycosis fungoides (MF) is rare and is usually seen in advanced stages of the disease. We describe a patient with early-stage follicular MF who presented with changes in mental status. Despite an initial diagnosis of vasculitis based on clinical and brain biopsy results, the postmortem examination revealed extensive infiltration of MF cells throughout the brain with leptomeningeal involvement. This case in addition to the accompanied review of literature illustrates the importance of the awareness of central nervous system involvement by MF and highlights the need for an urgent neurologic evaluation in patients with a history of MF now presenting with neurologic signs or symptoms.
Assuntos
Neoplasias Encefálicas/patologia , Micose Fungoide/patologia , Neoplasias Cutâneas/patologia , Evolução Fatal , Humanos , Masculino , Pessoa de Meia-IdadeRESUMO
Intravascular lymphoma (IVL) is a rare and fatal disease, typically of B-cell origin. Most of the reported cases have been for primary IVL, and only a minority of cases are of recurrent IVL. In addition, recurrent IVL occurring after treatment of anaplastic large T-cell lymphoma (ALCL) by contrast is extraordinarily rare. In this article, we present 3 cases of recurrent cutaneous IVL (2 men and 1 woman) and compare these with 1 case of primary IVL. The patients ranged in age from 56 to 73 years and were encountered in the routine dermatopathology and consultative practices of one of the authors. In 2 of the cases, the patients had intravascular cutaneous ALCL. In regard to the remaining 2 patients, 1 patient had a recurrent intravascular cutaneous follicular lymphoma in the context of a history of diffuse large B-cell lymphoma. The fourth patient had a primary intravascular ALCL because there was no antecedent history. In all cases, the skin biopsies showed large aggregates of atypical cells within the blood vessels. Phenotypic studies revealed variable staining results with CD29 and CD54 in cases of recurrent IVL compared with those of primary IVL. Recurrent cutaneous IVL represents a somewhat heterogeneous group of lymphoproliferative disorders with a distinct variant being in the context of intravascular ALCL; the mechanisms of intravascular localization in recurrent IVL are likely different from those of primary IVL.