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Open AccessFeature PaperReview

The Pathological Spectrum of Systemic Anaplastic Large Cell Lymphoma (ALCL)

Institute of Pathology and Neuropathology and Comprehensive Cancer Center Tübingen, Eberhard-Karls-University, Liebermeisterstraße 8, 72076 Tübingen, Germany
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Cancers 2018, 10(4), 107; https://doi.org/10.3390/cancers10040107
Received: 12 March 2018 / Revised: 30 March 2018 / Accepted: 2 April 2018 / Published: 4 April 2018
Anaplastic large cell lymphoma (ALCL) represents a group of malignant T-cell lymphoproliferations that share morphological and immunophenotypical features, namely strong CD30 expression and variable loss of T-cell markers, but differ in clinical presentation and prognosis. The recognition of anaplastic lymphoma kinase (ALK) fusion proteins as a result of chromosomal translocations or inversions was the starting point for the distinction of different subgroups of ALCL. According to their distinct clinical settings and molecular findings, the 2016 revised World Health Organization (WHO) classification recognizes four different entities: systemic ALK-positive ALCL (ALK+ ALCL), systemic ALK-negative ALCL (ALK− ALCL), primary cutaneous ALCL (pC-ALCL), and breast implant-associated ALCL (BI-ALCL), the latter included as a provisional entity. ALK is rearranged in approximately 80% of systemic ALCL cases with one of its partner genes, most commonly NPM1, and is associated with favorable prognosis, whereas systemic ALK− ALCL shows heterogeneous clinical, phenotypical, and genetic features, underlining the different oncogenesis between these two entities. Recognition of the pathological spectrum of ALCL is crucial to understand its pathogenesis and its boundaries with other entities. In this review, we will focus on the morphological, immunophenotypical, and molecular features of systemic ALK+ and ALK− ALCL. In addition, BI-ALCL will be discussed. View Full-Text
Keywords: anaplastic large cell lymphoma (ALCL); ALK (anaplastic lymphoma kinase); breast implant-associated ALCL; lymphoma; morphology anaplastic large cell lymphoma (ALCL); ALK (anaplastic lymphoma kinase); breast implant-associated ALCL; lymphoma; morphology
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Montes-Mojarro, I.A.; Steinhilber, J.; Bonzheim, I.; Quintanilla-Martinez, L.; Fend, F. The Pathological Spectrum of Systemic Anaplastic Large Cell Lymphoma (ALCL). Cancers 2018, 10, 107.

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