ABSTRACT
BACKGROUND: Extranodal involvement is not unusual in mantle cell lymphoma (MCL) which accounts for 6% of non-Hodgkin's lymphomas. Simultaneous localization in the eyelid and in the breast, as observed in our case, is however exceptional. CASE REPORT: Chronic lymphoid leukemia (CLL) was suspected in a 71-year-old woman with asthenia, a cervical and axillary nodal enlargement and elevated lymphocyte count. Blood immunoflow cytometry analysis, occurrence of rapidly growing tumors involving the two breasts and eyelids and cytogenetic and molecular features led to the diagnosis of MCL. A very good partial remission was obtained with second-line polychemotherapy composed of cytarabin, cisplatin and dexamethasone, but lasted only 3 months after the end of 6 cycles. DISCUSSION: Primary breast and eyelid lymphomas are rare. Such localizations are exceptional in MCL and are signs of aggressive disease. Before extra-nodal involvement, MCL may simulate banal CLL. Therefore, systematic immunohistochemistry and if necessary molecular analysis are useful for early diagnosis of MCL. Prognosis is particularly poor. Conventional chemotherapy cannot provide cure of MCL and median survival is 48 months. For this reason, high-dose chemotherapy with stem cell graft has to be discussed in young patients. MCL is currently characterized by Bcl1 rearrangement, t(11-14) translocation and cyclin D1 overexpression among small B-cell lymphomas in recent REAL- and WHO-classifications.
