BACKGROUND: Extranodal involvement is not unusual in mantle cell lymphoma (
MCL) which accounts for 6% of non-Hodgkin's lymphomas, Simultaneous localiz
ation 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 elevate
d lymphocyte count Blood immunoflow cytometry analysis, occurrence of rapid
ly growing tumors involving the two breasts and eyelids and cytogenetic and
molecular features led to the diagnosis of MCL. A very good partial remiss
ion was obtained with second-line polychemotherapy composed of cytarabin, c
isplatin and dexamethasone, but lasted only 3 months after the end of 6 cyc
les.
DISCUSSION: Primary breast and eyelid lymphomas are rare. Such localization
s are exceptional in MCL and are signs of aggressive disease. Before extra-
nodal involvement, MCL. may simulate banal CLL. Therefore, systematic immun
ohistochemistry and if necessary molecular analysis are useful for early di
agnosis of MCF. Prognosis is particularly poor. Conventional chemotherapy c
annot 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 Bcl 1 rearrangement t(11-14) tr
anslocation and cyclin D 1 overexpression among small B-cell lymphomas in r
ecent REAL- and WHO-classifications.