Cutaneous mantle cell lymphoma: a clinicopathologic review of 10 cases

Cutaneous mantle cell lymphoma (MCL) is exceedingly rare, almost always occurring as a dissemination of systemic MCL. To date, only 32 cases have been described. We report a series of 10 cases of MCL in the skin, and provide a comprehensive clinicopathologic review with clinical follow-up. Our cases occurred in older individuals (mean age = 70) and were more frequently in men (90%). Half of them presented in the head and neck region as a mass/nodule, and the remainder in the trunk and extremities as nodules. All patients have stage IV disease. In two of the 10 cases (20%) the cutaneous lesions preceded the diagnosis of disseminated disease. In two of the seven cases with available clinical follow-up information (33%) the skin was immediately involved after the diagnosis of MCL, and in three of the seven the skin was the first site of recurrence (mean interval = 57 months). The mean time to recurrence of the disease was 45.4 months and the overall survival was 66.3 months. Histologically six out of 10 cases (60%) had either pleomorphic or blastoid morphology (four out of 10 and two out of 10, respectively). The mean number of mitoses per 10 high-power fields was 18.44. While nine out of 10 cases expressed cyclin-D1, one case was not positive for cyclin-D1 but did label with SOX-11. Limited cytogenetic data showed trisomy 14 in one case, in addition to the t(11;14) translocation.