Fratoni Stefano, Trawinska Malgorzata Monika, Capalbo Anna, Bernardini Laura, Fabbretti Maria, Martini Maurizio, Niscola Pasquale, Zhao Xiangfeng Frank
Diagnostic Surgical Pathology Department/Hematopathology Section, Saint'Eugenio Hospital, Rome, Italy.
Hematology Unit, Saint'Eugenio Hospital, piazzale dell'Umanesimo 10 (00144), Rome, Italy.
Virchows Arch. 2022 Dec;481(6):975-979. doi: 10.1007/s00428-022-03386-5. Epub 2022 Jul 26.
Systemic anaplastic lymphoma kinase-negative (ALK-) anaplastic large cell lymphoma (ALCL) comprises a genomically heterogeneous disease that is considered a distinct entity by the 2016 World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues. Other than lymph nodes, systemic ALK- ALCL may affect extranodal tissues, sites where the inflammatory background may be especially prominent. In this scenario, myxoid change is exceptional in systemic ALK- ALCL. We describe a rare case of systemic ALK- ALCL with distinctive myxoid changes, carrying specific chromosomal aberrations that affect the clinical outcome. Careful morphological, immunohistochemical, and molecular workup is mandatory because a myxoid background should not be a reason to ignore the possibility of a lymphoma. Finally, extensive correlation with staging and the detection of prognostic biomarkers such as DUSP22 and TP63 rearrangements are essential for the diagnosis and prediction of clinical outcome in ALK- ALCL.
系统性间变性淋巴瘤激酶阴性（ALK-）间变性大细胞淋巴瘤（ALCL）是一种基因组异质性疾病，在2016年世界卫生组织造血与淋巴组织肿瘤分类中被视为一个独特的实体。除淋巴结外，系统性ALK- ALCL可能累及结外组织，这些部位的炎症背景可能尤为突出。在这种情况下，黏液样改变在系统性ALK- ALCL中较为罕见。我们描述了一例罕见的具有独特黏液样改变的系统性ALK- ALCL病例，该病例携带影响临床结局的特定染色体畸变。由于黏液样背景不应成为忽视淋巴瘤可能性的理由，因此仔细的形态学、免疫组织化学和分子检查是必要的。最后，与分期进行广泛关联以及检测预后生物标志物如DUSP22和TP63重排对于ALK- ALCL的诊断和临床结局预测至关重要。
