Mycosis fungoides with extracutaneous localization in the breast

Abstract

We report a patient with mycosis fungoides (MF) and transformation to anaplastic (CD30+) lymphoma, who developed an unusual manifestation in the breast. Cutaneous and extracutaneous tumour cells both showed marked intraepithelial migration, but had distinct expression patterns of epitheliotropism-associated integrins. Intraepidermal and dermal lymphoma cells in the skin expressed most of the integrins that are presumed to be involved in epitheliotropism, such as leucocyte functional antigen-1 (LFA-1), alpha 1 beta 1, alpha 3 beta 1, alpha 4 beta 1 and alpha 6 beta 1. In the breast, most of the extraepithelial tumour cells only expressed the laminin-5 ligand alpha 3 beta 1 (strong) and the collagen ligand alpha 1 beta 1 (weak). However, on the intraepithelial tumour cells in the mammary ducts and glands LFA-1, alpha 4 and alpha E could also be detected. In view of these findings we propose epitheliotropism of the MF cells in the breast to be a multistep process in which tumour cell-epithelial basement membrane (EBM) interaction, predominantly mediated by alpha 3 beta 1 and laminin-5, is the primary event. Adhesion to and subsequent infiltration of the neoplastic lymphoid cells into the EBM may, either directly or indirectly, lead to upregulation and/or activation of other integrins that amplify interaction with epithelial cells.

Clinicopathological spectrum of mycosis fungoides

“…9, 16 On very rare occasions, MF involves extracutaneous compartments such as the oral cavity (tongue, oral mucosae), oesophagus, breast, eyes and the central nervous system before large-cell transformation. [159][160][161][162][163][164][165][166][167][168][169][170][171] As typical skin lesions are usually also present, the diagnosis should be straightforward. In the oral cavity, both tongue and mucosae may be affected.…”

γδ T‐cell lymphoma in a cow

“…15 In a report of human mycosis fungoides, the lymphoma cells had epitheliotropism-associated integrins such as α3β1 and α4β1. 16 In our case, the presence of CD11c and CD18 (integrin αXβ2) and CD49d (integrin α4) might have been related to intraepithelial migration of lymphoma cells.…”

Bilateral breast involvement in Sézary syndrome

“…Either primary or secondary, nonHodgkin's lymphoma of the breast is B cell in phenotype in most of cases [8,9,10]. Cutaneous T-cell lymphoma involving the breast has been documented in few cases; however, they were not the cases of SØzary syndrome [2,4,5,6]. The extracutaneous spread of CTCL occurs via the hematogenous or lymphatic pathways.…”

“…Cutaneous T-cell lymphoma is confined to the skin for a variable period of time and extracutaneous dissemination is generally considered to be a late occurrence; however, dissemination may occur at any time during the course of the disease, and almost every organ has been shown to have a potential for involvement [3]. The parenchymal involvement of the breast has been reported in mycosis fungoides [2,4,5,6]; however, no prior report has described breast involvement in SØzary syndrome. In this report, we describe the clinical and radiological findings of bilateral breast involvement in a case of SØzary syndrome.…”