Skin Involvement of Mantle Cell Lymphoma May Mimic Primary Cutaneous Diffuse Large B-cell Lymphoma, Leg Type

Abstract

Mantle cell lymphoma (MCL) is a B-cell neoplasm with a variable and generally aggressive clinical course. So far our knowledge of skin involvement of MCL is limited. To understand the clinical and histopathologic features of MCL with skin involvement, the files of the Lymph Node Registry Kiel were screened for MCL diagnosed in the skin. Over a period of 13 years, 1321 biopsy specimens were diagnosed as MCL; among them, 14 patients (1%) showed skin involvement. Of these, skin was the initial site of manifestation in 6/11 (55%) cases. One patient presented with a skin-limited lymphoma. Furthermore, 7/12 (58%) patients presented with lesions on the leg. The lymphomas were highly proliferative with blastoid cytology in 12/14 (86%) cases. Moreover, the immunophenotype with expression of BCL2 (100%), MUM-1/IRF4 (83%), and IgM (82%) and lack of CD10 (25%) and BCL6 (0%) closely resembled the features of primary cutaneous diffuse large B-cell lymphoma, leg type. Solely the expression of cyclin D1 (100%) and the presence of t(11;14) (100%) allowed a distinction from cases of primary cutaneous diffuse large B-cell lymphoma, leg type. Only 2 MCL cases with skin involvement presented with classical cytology. Interestingly, in these 2 cases skin involvement occurred simultaneously in a lesion of coexisting primary cutaneous marginal zone lymphoma. Our data suggest that clinical presentation on the leg and blastoid cytology along with high proliferation and expression of Bcl2, Mum-1/IRF4, and IgM are typical for MCL involving the skin. Lymphomas with these features might be erroneously diagnosed as diffuse large B-cell lymphoma, leg type, if cyclin D1 staining is not performed.

Mantle Cell Lymphoma Relapsing as Disease of Skin, Orbit and CNS: An Extremely Rare Presentation and a Review of Literature

“…Commonly observed skin lesions include nodules, macules, tumoral plaques and subcutaneous infiltrations [8,14]. The average age of the patients with cutaneous MCL was 64.4 years (range: 22 -89 years), with male to female ratio of 19: 5 [14].…”

“…Most commonly affected areas were the trunk followed by the face, arms, legs, thighs and the abdomen [8,9]. Commonly observed skin lesions include nodules, macules, tumoral plaques and subcutaneous infiltrations [8,14].…”

“…2018;7(1):38-42 toid and pleomorphic variants have been generally associated with morbid prognosis and incurability [7]. The blastoid variant is found in 10-30% of those diagnosed with MCL and has a shorter median survival time than classical type (15 vs. 45 months) [5,8,9]. The skin involvement, even if rare, has been more frequently reported in the blastoid variant of MCL [2].…”

Skin manifestation of mantle cell lymphoma – case report and literature review

“…The differentiation between both diseases was only possible on the basis of cyclin-D1 and translocation t (11;14) analysis. On that basis, the authors recommended routine tests to detect cyclin-D1 expression in cases of suspected mantle cell lymphoma [14].…”

“…Jedynie ekspresja cykliny D1 oraz translokacji t(11;14) umożliwiła zróżnicowanie tych dwóch chorób. Na tej podstawie zalecono rutynowe poszukiwanie ekspresji cykliny D1 przy podejrzeniu chło-niaka z komórek płaszcza [14].…”