Division of Pediatric Hematology-Oncology, Mayo Clinic, Rochester, Minnesota 55905, USA.
Pediatr Blood Cancer. 2011 Feb;56(2):307-10. doi: 10.1002/pbc.22810.
Histiocytic sarcoma (HS) is a rare malignancy of tissue histiocytes with a dismal prognosis. We report a 4-year-old male who developed HS during maintenance chemotherapy for precursor B-cell acute lymphoblastic leukemia (pre-B ALL). Both tumors showed identical clonal immunoglobulin and T-cell receptor gene re-arrangement patterns, as well as homozygous deletion of the CDKN2A gene encoding p16(INK4A). These data suggest a clonal relationship between the two neoplasms despite their distinct lineages. Since CDKN2A deletion predisposes to development of HS in experimental models, the cytogenetic features of the patient's pre-B ALL may have predisposed to this change in lineage.
组织细胞肉瘤（HS）是一种组织细胞的罕见恶性肿瘤，预后较差。我们报告了一例 4 岁男性，在接受前体 B 细胞急性淋巴细胞白血病（pre-B ALL）维持化疗期间发生 HS。两个肿瘤均表现出相同的克隆免疫球蛋白和 T 细胞受体基因重排模式，以及 CDKN2A 基因（编码 p16(INK4A)）的纯合缺失。这些数据表明尽管两种肿瘤的谱系不同，但它们之间存在克隆关系。由于 CDKN2A 缺失易导致实验模型中发生 HS，因此患者 pre-B ALL 的细胞遗传学特征可能易导致这种谱系改变。
