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Primary cutaneous follicle center lymphoma

Authors
Eric Jacobsen, MD
Arnold S Freedman, MD
Rein Willemze, MD
Section Editors
Timothy M Kuzel, MD, FACP
John A Zic, MD
Deputy Editor
Alan G Rosmarin, MD

INTRODUCTION

Primary cutaneous B cell lymphoma (PCBCL) refers to those cases of B cell lymphoma that present in the skin when there is no evidence of extracutaneous disease at the time of diagnosis and after the completion of an initial staging evaluation. The revised 2016 World Health Organization (WHO) classification system defines three main subtypes of PCBCL [1]:

Primary cutaneous follicle center lymphoma (PCFCL)

Primary cutaneous large B cell lymphoma, leg type

Primary cutaneous marginal zone lymphoma (PCMZL), which is included in the broader category of extranodal marginal zone B cell lymphoma

These appear to be unique entities with differing clinical presentations, pathologic features, and prognosis. The approach to treatment also differs between subtypes. This topic review will discuss PCFCL. This entity was previously known as primary cutaneous follicle center cell lymphoma (PCFCCL) under the European Organization for Research and Treatment of Cancer (EORTC) classification and contains cases that may have previously be classified as cutaneous follicle center lymphoma or diffuse large B cell lymphoma under the 2001 WHO classification.

                       

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Literature review current through: Nov 2016. | This topic last updated: Wed Sep 14 00:00:00 GMT 2016.
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