Epidermotropic diffuse large B‐cell lymphoma infiltrating the umbilicus, presenting as Sister Mary Joseph's nodule

Abstract

EditorSister Mary Joseph's nodule is an umbilical metastatic nodule from an intra-abdominal tumour.1 The primary origin is usually the gastrointestinal or genital system, but on rare occasions, this pathology originates from lymphoma. [2][3][4] We describe herein a case of diffuse large B-cell lymphoma (DLBCL) infiltrating only in the umbilicus, histologically presenting as epidermotropism, as an atypical histological presentation of B-cell lymphoma.A 36-year-old Japanese man consulted with us about a 1-week history of a painful nodule on the umbilicus. He had been treated for DLBCL developing in the pancreas with a regimen of R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone). On the first visit, physical examination disclosed an infiltrated, reddish nodule on the umbilicus (Fig. 1a). Computed tomography (CT) of the abdomen revealed significant enlargement of a tumour at the umbilicus (Fig. 1b). A biopsy specimen from the navel revealed atypical large cells densely infiltrating through the dermal papillae to the subcutaneous tissue, and epidermotropism with involvement of the overlying epidermis (Fig. 2a). Immunohistochemical staining revealed that these large cells were positive for CD10 (Fig. 2b), CD20 (Fig. 2c), CD45, CD79a (Fig. 2d), bcl2 and c-myc and negative for CD3, CD5, CD56, bcl6, and multiple myeloma oncogene-1. From the above data, we diagnosed DLBCL on the umbilicus, presenting as Sister Mary Joseph's nodule.Because the histological finding of epidermotropism in B-cell lymphoma is extremely rare, we employed immunohistochemical staining for matrix metalloproteinase (MMP)-2, MMP-9, and E-cadherin to investigate the underlying mechanisms. Immunohistochemical staining revealed a lack of E-cadherin in tumour-infiltrated areas of epidermis (Fig. S1a, Supporting Information). Moreover, lymphoma cells were positive for MMP-9 (Fig. S1c, Supporting Information) and negative for MMP-2 (Fig. S1b, Supporting Information).In this report, we have described a case of DLBCL infiltrating only the umbilicus, presenting as a Sister Mary Joseph's nodule and showing epidermotropism histologically. To inves-

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“…Although this case was initially interpreted as a DLBCL, the distinctive clinical presentation with a disseminated skin rash resembling pityriasis rosea was unusual for DLBCL. In addition, the orderly multinodular non-effacing growth pattern around nerves, blood vessels, and the eccrine coil without diffuse infiltration and the presence of epidermotropism, which has been only rarely reported in DLBCL, were not compatible with DLBCL [15,16]. Moreover, there were many admixed smaller neoplastic B cells.…”

Epstein-Barr Virus Positive Diffuse Large B-Cell Lymphoma with Epidermotropism

“…The mechanism of epidermotropism of B-cell lymphomas is unknown, but Kakinuma et al 16 suggested a distinct interaction between the neoplastic B cells and particular epidermal chemokines and growth factors, similar to the mechanisms of epidermal involvement in T-cell lymphomas. Furudate et al 17 described a case of DLBCL with epidermotropism involving the umbilicus and found that malignant lymphoid cells expressed matrix metalloproteinase-9 in relation to decreased E-cadherin expression in the surrounding epidermis. This suggest that these changes promote migration of neoplastic lymphocytes into the epidermis.…”

Epidermotropic Epstein–Barr virus-Positive Diffuse Large B-Cell Lymphoma: A Series of 3 Cases of a Very Unusual High-Grade Lymphoma

“…26,29 More uncommonly, cases of DLBCLs with involvement of the epidermis have been described. 30,31 Clinically, these cases presented as solitary plaques or nodules and were sometimes associated with epidermal ulceration. Furudate et al 30 demonstrated in their case of epidermotropic DLBCL involving the umbilicus that lymphoma cells expressed matrix metalloproteinase-9 in association with diminished E-cadherin expression in the surrounding epidermis and postulated that these changes facilitated migration of malignant lymphocytes into the epidermis.…”