Department of Pathology, Charles University, 3rd Faculty of Medicine, Charles University and Kralovske Vinohrady University Hospital, Prague, Czech Republic.
Department of Pathology and Molecular Medicine, 2nd Faculty of Medicine, Charles University and Motol University Hospital, Prague, Czech Republic.
Diagn Pathol. 2019 Jul 17;14(1):80. doi: 10.1186/s13000-019-0854-9.
Panniculitis-like T-cell lymphoma is an uncommon type of non-Hodgkin lymphoma, occurring usually in the form of nodules within the subcutaneous fat tissue of the extremities or trunk. In the literature, subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is described as a distinct type of T-cell lymphoma with a variable clinical behavior, depending on molecular phenotype of T-cell receptor (TCR) and on the presence or absence of hemophagocytic syndrome.
We present a bioptic and autoptic case of a 65-years old Caucasian man with panniculitic T-cell lymphoma with morphological and immunohistochemical features of SPTCL, limited to the retroperitoneal and mesenteric mass, i.e. without any cutaneous involvement, and associated with severe hemophagocytic lymphohistiocytosis.
A panniculitic T-cell lymphoma with morphological and molecular features of SPTCL, which is limited to mesentery, i.e. does not involve subcutaneous fat, seems to be exceedingly rare.
脂膜炎样 T 细胞淋巴瘤是非霍奇金淋巴瘤的一种罕见类型，通常以四肢或躯干皮下脂肪组织内结节的形式出现。在文献中，皮下脂膜炎样 T 细胞淋巴瘤（SPTCL）被描述为一种独特的 T 细胞淋巴瘤，其临床表现具有变异性，取决于 T 细胞受体（TCR）的分子表型以及是否存在噬血细胞综合征。
我们报告了一例 65 岁白人男性的脂膜炎样 T 细胞淋巴瘤活检和尸检病例，具有 SPTCL 的形态学和免疫组织化学特征，仅限于腹膜后和肠系膜肿块，即没有任何皮肤受累，并且伴有严重的噬血细胞性淋巴组织细胞增生症。
形态学和分子学特征符合 SPTCL 的脂膜炎样 T 细胞淋巴瘤，仅限于肠系膜，即不涉及皮下脂肪，似乎极为罕见。
