Mantle cell lymphoma (MCL), an uncommon and aggressive form of non-Hodgkin
lymphoma, typically involves lymph nodes. It usually only secondarily invol
ves extranodal sites. We describe an unusual case of a MCL that presented a
nd relapsed in the earlobes. Light microscopic findings were initially rega
rded as suggestive of small lymphocytic lymphoma, al though subsequent anal
ysis of fresh tissue by flow cytometry led to the diagnosis of MCL. Retrosp
ective application of a broad panel of recently developed markers suitable
for analysis of routinely processed tissue yielded results that also permit
ted a diagnosis of MCL. If these results had been available at the time of
initial presentation, they would have obviated the need for rebiopsy. Great
er awareness not only of the phenotypic criteria by which lymphomas are cla
ssified but of the lymphoma markers available for evaluation of routinely p
rocessed tissue should facilitate the accurate diagnosis of diseases like M
CL and minimize the risk of misdiagnosis as an indolent disorder.