Department of Medical Biotechnologies, Section of Pathology, University of Siena, Siena, Italy.
Diagn Pathol. 2014 Jun 20;9:124. doi: 10.1186/1746-1596-9-124.
T-cell lymphoblastic lymphoma comprises approximately 85-90% of all lymphoblastic lymphomas. It often arises as a mediastinal mass, and with bone marrow involvement. Presentation at other sites without nodal or mediastinal localization is uncommon.
We describe clinical, histologic, immunohistochemical, and molecular features of two cases of primary T-cell lymphoblastic lymphoma arising respectively in uterine corpus and testis. The tumors were composed by medium to large cells, exhibiting a diffuse pattern of growth but sometimes forming indian files or pseudo-rosettes. The neoplastic cells strongly expressed TdT and T-cell markers in both uterine corpus and testis. However, the testis case also showed aberrant expression of B-cell markers, thus molecular biology was necessary to achieve a final diagnosis. T-cell receptor gene rearrangement analysis identified a T-cell origin.
To the best of our knowledge, only one doubtful previous case of primary uterine T-cell lymphoblastic lymphoma and no previous cases of primary testicular T-cell lymphoblastic lymphoma have been reported. Due to the morphology of neoplastic cells, a challenging differential diagnosis with all the tumors belonging to the so-called small round blue cell tumor category is mandatory. In ambiguous lineage cases, molecular biology may represent an adequate tool to confirm diagnosis.
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T 细胞淋巴母细胞淋巴瘤约占所有淋巴母细胞淋巴瘤的 85-90%。它通常表现为纵隔肿块，并伴有骨髓侵犯。在无淋巴结或纵隔定位的其他部位表现少见。
我们描述了分别发生在子宫体和睾丸的两例原发性 T 细胞淋巴母细胞淋巴瘤的临床、组织学、免疫组织化学和分子特征。肿瘤由中到大细胞组成，呈弥漫性生长模式，但有时形成印第安文件或假玫瑰结。肿瘤细胞在子宫体和睾丸中均强烈表达 TdT 和 T 细胞标志物。然而，睾丸病例也表现出 B 细胞标志物的异常表达，因此需要进行分子生物学检查以做出最终诊断。T 细胞受体基因重排分析确定了 T 细胞起源。
据我们所知，仅报告过一例可疑的原发性子宫 T 细胞淋巴母细胞淋巴瘤和一例原发性睾丸 T 细胞淋巴母细胞淋巴瘤。由于肿瘤细胞的形态，与所有属于所谓小圆蓝细胞肿瘤类别的肿瘤进行具有挑战性的鉴别诊断是必要的。在不确定谱系的情况下，分子生物学可能是确认诊断的合适工具。
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