Peripheral T cell lymphomas (PTCLs) are highly heterogeneous tumors, displaying distinct clinical and biological features. The pathogenesis and normal counterpart of such entities have been elusive for decades. Recent studies have, however, disclosed key mechanisms of peripheral T cell transformation, including (a) the deregulation of signaling pathways controlling T cell development, differentiation, and maturation; (b) the remodeling of the peritumor microenvironment; and (c) the virus-mediated rewiring of T cell biology. Uncovering the molecular mechanisms of T cell transformation will help elucidate the peculiar clinical and pathological features of each PTCL entity and will lead to the characterization of novel antitumor therapies. These therapies will combine conventional and new-generation compounds with immune-modulating agents to ablate both the neoplastic cells and the tumor-supporting microenvironment. This review addresses the pathogenic mechanisms of PTCLs, with special attention paid to novel therapeutic strategies for the clinical management of such aggressive tumors.

中文翻译：

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外周T细胞淋巴瘤的发病机制

外周T细胞淋巴瘤（PTCL）是高度异质性肿瘤，表现出独特的临床和生物学特征。此类实体的发病机制和正常对应物已经消失了数十年。但是，最近的研究已经揭示了外周T细胞转化的关键机制，包括（a）控制T细胞发育，分化和成熟的信号通路的失调；（b）重塑周围微环境；和（c）病毒介导的T细胞生物学重新连接。揭示T细胞转化的分子机制将有助于阐明每个PTCL实体的独特临床和病理特征，并将导致新型抗肿瘤疗法的表征。这些疗法将常规化合物和新一代化合物与免疫调节剂结合起来，以消融肿瘤细胞和支持肿瘤的微环境。这项审查解决了PTCLs的致病机制，特别注意这种侵袭性肿瘤的临床治疗的新型治疗策略。