XXXX . An aggressive lymphoma distinct from mycosis fungoides.

@entity1 @entity706 @entity39 @entity2617 @entity784 @entity3 @entity272
Among 37 consecutive cases of @entity706 in which the skin was either the only site of disease or one of the prominent sites of initial involvement, 19 cases had a distinctive histological appearance. These cases corresponded to what has been termed " @entity706 ," and all were found to exhibit a T-cell phenotype either by frozen-section immunohistochemistry or by using monoclonal antibodies reactive in paraffin sections. There were nine @entity1 and 10 @entity1 ; the mean age was 48.2 years. The lesions were nodular and were either ulcerated or had intact skin. One case presented with involvement of one anatomical region of skin, five had involvement of multiple regions of skin, and 13 had concurrent extracutaneous disease. The disease pursued an aggressive course and was not uncommonly resistant to treatment. Histologically, the @entity2617 occurred predominantly in the mid to deep dermis with involvement of the subcutaneous layer. The pattern was mainly perivascular and peri-adnexal with or without confluence; the overlying epidermis and papillary dermis were often spared. A prominent feature was invasion of small or large blood vessels by @entity706 cells. Eight cases showed @entity39 of the neoplastic and normal tissues, and 12 cases showed @entity3 . The neoplastic lymphoid cells consisted of either a monomorphous population or a variable mixture of small, medium-sized, and large cells with stippled chromatin and distinct nucleoli. Although the nuclei were often irregularly folded, few exhibited a cerebriform configuration. The cytoplasm was pale to clear. These cases exhibit a remarkable histological similarity to the @entity784 of the nasal/nasopharyngeal region; in addition, there are features that overlap with so-called @entity272 .
