Mantle Cell Lymphoma Involving Skin A Clinicopathologic Study of 37 CasesKim, Do Hwan MD*; Medeiros, L. Jeffrey MD*; Aung, Phyu P. MD, PhD†; Young, Ken H. MD*; Miranda, Roberto N. MD*; Ok, Chi Young MD*The American Journal of Surgical Pathology: October 2019 - Volume 43 - Issue 10 - p 1421–1428 doi: 10.1097/PAS.0000000000001312 Original Articles Buy Abstract Author InformationAuthors Article MetricsMetrics Mantle cell lymphoma (MCL) rarely involves the skin and the histologic and immunohistochemical features of this neoplasm at this site are under described. In this study, we report 37 skin specimens involved by MCL, representing 1.4% of total MCL biopsy specimens in our institution. The median age at time of skin involvement was 66 years (range, 36 to 85 y) and there was a male predilection of 2.7 to 1. The most frequently involved site was the skin of extremities, in 59.3% of patients, and 30 (81.1%) patients had advanced stage (III/IV) disease. Eleven (29.7%) patients presented with skin lesions as the first manifestation of MCL and 26 (70.3%) patients presented as relapse or progression of previously documented MCL and despite therapy for systemic MCL. Multiple skin lesions were more common (81.8%) in the former group whereas a solitary skin lesion was more frequent (65.4%) in the relapse/progression group (P=0.01). Thirty (81.1%) patients had skin nodules. Microscopically, the epidermis was spared with a grenz zone in all cases. A diffuse pattern of involvement was the most common architectural pattern (66.7%). In 27 (72.9%) patients, the MCL was either blastoid or pleomorphic variant, in 9 (24.3%) patients classic variant, and the disease was not further classified in 1 (2.7%) patient. The Ki-67 proliferation rate was higher in aggressive variants as compared with classic variant MCL (median 90% vs. 20%, P <0.01). In patients who presented skin lesions as a manifestation of disease relapse or progression, 16 patients initially had classic variant MCL and in 10 of the patients the MCL evolved over time (median interval: 4.1 y) to an aggressive variant at progression or relapse. The overall survival of patients with aggressive variant MCH was inferior to that of patients with classic variant MCL (median: 59 vs. 155.8 mo, P<0.05). In summary, MCL rarely involves the skin and correlates with relapse or progression of disease, aggressive morphologic features, and a poorer prognosis. Departments of *Hematopathology †Pathology, The University of Texas M.D. Anderson Cancer Center, Houston, TX Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article. Correspondence: Chi Young Ok, MD, Department of Hematopathology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030-4009 (e-mail: firstname.lastname@example.org). Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.