Hematopathology Service, Department of Pathology and Laboratory Medicine, Memorial Sloan Kettering Cancer Center.
Molecular Diagnostic Laboratory, Department of Hematopathology, MD Anderson Cancer Center, Houston, TX.
Haematologica. 2023 Dec 1;108(12):3261-3277. doi: 10.3324/haematol.2023.282718.
The extranodal mature T-cell and NK-cell lymphomas and lymphoproliferative disorders represent a unique group of rare neoplasms with both overlapping and distinct clinicopathological, biological, and genomic features. Their predilection for specific sites, such as the gastrointestinal tract, aerodigestive tract, liver, spleen, and skin/soft tissues, underlies their classification. Recent genomic advances have furthered our understanding of the biology and pathogenesis of these diseases, which is critical for accurate diagnosis, prognostic assessment, and therapeutic decision-making. Here we review clinical, pathological, genomic, and biological features of the following extranodal mature T-cell and NK-cell lymphomas and lymphoproliferative disorders: primary intestinal T-cell and NK-cell neoplasms, hepatosplenic T-cell lymphoma, extranodal NK/T-cell lymphoma, nasal type, and subcutaneous panniculitis-like T-cell lymphoma.
结外成熟 T 细胞和 NK 细胞淋巴瘤和淋巴增生性疾病是一组独特的罕见肿瘤，具有重叠和独特的临床病理、生物学和基因组特征。它们对特定部位的偏好，如胃肠道、呼吸道、肝脏、脾脏和皮肤/软组织，构成了它们的分类基础。最近的基因组学进展进一步加深了我们对这些疾病的生物学和发病机制的理解，这对于准确诊断、预后评估和治疗决策至关重要。在这里，我们回顾了以下结外成熟 T 细胞和 NK 细胞淋巴瘤和淋巴增生性疾病的临床、病理、基因组和生物学特征：原发性肠道 T 细胞和 NK 细胞肿瘤、肝脾 T 细胞淋巴瘤、结外 NK/T 细胞淋巴瘤、鼻型和皮下脂膜炎样 T 细胞淋巴瘤。
