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Primary Cutaneous Anaplastic Lymphoma Kinase–Positive Large B-Cell Lymphoma

Kempf, Werner MD*; Torricelli, Rocco MD; Zettl, Andreas MD; Zimmermann, Anne-Katrin MD*; Berisha, Arbeneshe BSc*; Ghielmini, Michele MD§

The American Journal of Dermatopathology: August 2019 - Volume 41 - Issue 8 - p 602–605
doi: 10.1097/DAD.0000000000001348
Extraordinary Case Report

Abstract: Large B-cell lymphomas include several subtypes. Recently, anaplastic lymphoma kinase (ALK)-positive large B-cell lymphoma has been delineated as a distinct entity involving mostly lymph nodes and rarely affecting extranodal sites. We describe the first case of a primary cutaneous ALK-positive large B-cell lymphoma in a 48-year-old man with a solitary nodule on the back, and describe the histologic and phenotypic features. Accurate staging confirmed the absence of other lesions, and so surgical excision and postoperative local radiation therapy were initiated and resulted in complete remission. Two years later, extracutaneous spread with involvement of axillary lymph nodes occurred. Complete remission was achieved again by multiagent chemotherapy. Our case demonstrates that a primary cutaneous form of ALK-positive large B-cell lymphoma exists. The immunophenotypic analysis of cutaneous large B-cell lymphomas affecting the skin primarily or secondarily should include the assessment of ALK expression.

*Kempf und Pfaltz, Histologische Diagnostik, Zurich, Switzerland;

Dermatology Practice, Lugano-Paradiso, Switzerland;

Viollier Pathology, Allschwil, Switzerland; and

§Oncologia Ospedale Regionale Bellinzona e Valli, Bellinzona, Switzerland.

Correspondence: Werner Kempf, MD, Kempf und Pfaltz, Histologische Diagnostik, CH-8050 Zürich, Switzerland (e-mail:

The authors declare no conflicts of interest.

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