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Diagnostic Pathology
Published in: Diagnostic Pathology 1/2019

Open Access 01-12-2019 | Lymphoma | Case Report

Panniculitis-like T-cell-lymphoma in the mesentery associated with hemophagocytic syndrome: autopsy case report

Authors: Jan Hrudka, Václav Eis, Josef Heřman, Zuzana Prouzová, Andreas Rosenwald, František Duška

Published in: Diagnostic Pathology | Issue 1/2019

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Abstract

Background

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.

Case presentation

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.

Conclusion

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.
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Metadata
Title
Panniculitis-like T-cell-lymphoma in the mesentery associated with hemophagocytic syndrome: autopsy case report
Authors
Jan Hrudka
Václav Eis
Josef Heřman
Zuzana Prouzová
Andreas Rosenwald
František Duška
Publication date
01-12-2019

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