Primary cutaneous diffuse large B-cell lymphoma, leg type with central nervous system involvement

Abstract

Introduction: Primary cutaneous diffuse large B-cell lymphoma, leg type is a new entity of actual World Health Organization (WHO) classification of lymphoid neoplasms with distinctive clinical, mor-phological and immunophenotipic features. This very rare subtype of lymphoma is characterized by the primary occurence in the skin of the lower extremities, an aggressive clinical course and high relapse rate under different therapeutic regimens. Case report: A 71-year-old female patient pre-sented with bluish-red multicentric in part ulcera-ted nodular skin tumors of the left leg, with 2-5 cm in diameter. She noticed tumor-related chan-ges two months earlier. The excision biopsy of a tumor was performed. The diagnosis of primary cutaneous diffuse large B-cell lymphoma, leg type was obtained on review of the hematoxylin and eo-sin slides and immunophenotypic data. Treatment with 8 cycles of rituximab combined with doxo-rubicin, cyclophosphamide, vincristine, and pred-nisone, resulted in partial remission. Five months later she developed neurological symptoms, and PCR analysis confirmed the presence of monoclo-nal B-lymphoid population in the peripheral blo-od, bone marrow, and cerebrospinal fluid. Therapy for CNS lymphoma localisation remained without effect. She died 16 months after initial diagnosis. Conclusion: Leptomeningeal central nervous system involvement as extracutaneous dissemina-tion is a relatively uncommon for primary cuna-teous diffuse large B-cell lymphoma, leg type. An aggressive course of this rare subtype of non-Hodgkin lymphoma often requires the use of com-bined modality treatment, with ongoing testing of some of them.