Primary Cutaneous CD30-Positive T-cell Lymphoma of the Eyelids

摘要

A case of a cutaneous CD30-positive pleomorphic T-cell lymphoma primarily involving both the upper and lower left eyelids of a 50-year-old man is reported. A firm, partly ulcerated mass was the initial and only clinical sign. Histopathologic examination of a shave biopsy revealed atypical neoplastic cells in close relation with epidermal appendages, suggesting epithelial derivation of the lesion. Nevertheless, the results of the immunohistochemical testing disclosed diffuse reactivity for T-cells and CD30 positivity in more than 70% of neoplastic cells, thus supporting the diagnosis of a CD30-positive lymphoma, T-cell phenotype. The diagnosis of this entity is particularly challenging, especially at such a site, since primary ocular adnexal non-Hodgkin lymphomas are virtually of B-cell type. The differential diagnosis and a review of the relevant literature are presented. Introduction Most of ocular adnexa lymphomas bear a close relationship to MALT lymphoma and are low-grade B-cell tumors deriving from the marginal zone B-cell. High-grade lymphomas of the ocular adnexa are distinctly uncommon. T-cell origin in primary lymphomatous lesions of the eyelid is indeed rare1. CD30 represents a differentiation antigen whose expression is associated with activation of lymphoid cells; its immunohistochemical staining with the recent antibody of choice in diagnostic pathology (ie, monoclonal antibody Ber-H2) usually results in specific, circumferential membranous and paranuclear dot-like positivity. CD30-positive primary cutaneous anaplastic or pleomorphic large cell lymphoma (C-ALCL) belongs to the spectrum of CD30-positive T-cell lymphoproliferative disorders, originating from transformed or activated CD30-positive T-lymphocytes. Some of these lesions have ambiguous clinical and histological features and are referred as borderline lesions1. Case report A 59-year-old man was admitted to the hospital for evaluation of a severe erythematous swelling of both the upper and lower left eyelids and the cheek, which had been rapidly enlarged for the last 2-3 weeks. Medical and family histories were unremarkable.In the clinical examination, there was a large (1.8X1.5X0.7 cm), raised, firm, nodular mass located mainly in the lower left eyelid, with extensive ulceration and hemorrhage. It was surrounded by severe edema. Visual acuity was unaffected and bulbar movements were not significantly restricted; the eye did not appear to be infiltrated by the mass. Physical examination did not reveal any peripheral lymphadenopathy or hepatosplenomegaly. Before the final diagnosis was made, material from the ulcerated area was cultured and fungi of Candida Albicans type was found. According to this, the patient went under amphoterikine.Biopsies from both eyelids were taken. Hematoxylin and eosin sections showed partly necrotic and ulcerated, severely inflammed skin specimens. In the dermis, there was a diffuse infiltration of solid sheets of atypical cells, displaying foci of angiocentric growth pattern (Figure 1). The cells were large, pleomorphic, with hyperchromatic nuclei, while atypical mitotic figures were frequent. Some foci of keratinization, perineural distribution and sebaceous differentiation (Figure 1) were suggested. The above features were indicative of a neoplasm deriving from the sebaceous glands.