Chuang Shih-Sung, Lin Chin-Nan, Li Chin-Yang
Department of Pathology, Chi-Mei Medical Center, Yung-kang City, Tainan, Taiwan.
Pathol Res Pract. 2002;198(5):369-73. doi: 10.1078/0344-0338-00268.
Primary bony lymphomas are rare, and nearly all are high-grade B-cell lymphomas. Natural killer (NK)/T-cell lymphomas are highly aggressive lymphomas of NK- or T-cell lineage with predominant extranodal presentation and are divided into nasal and nasal-type (extra-nasal). We report a primary bony peripheral T-cell lymphoma mimicking NK/T-cell lymphoma, nasal type. A 22-year-old Taiwanese male presented with a frontal skull bone mass noted for 3 weeks, and received craniectomy with tumor removal. His tumor showed extensive coagulative necrosis with angioinvasion by large lymphoma cells expressing CD2, CD8, CD16, CD43, CD45, CD45RO, CD56, T-cell intracellular antigen-1, and granzyme B, but not CD3, CD4, CD20, CD57, CD68, and betaF1. In situ hybridization for Epstein-Barr virus-encoded mRNA was negative. Polymerase chain reaction study of formalin-fixed tissue showed clonal rearrangement of the T-cell receptor-gamma chain gene. The diagnosis was peripheral T-cell lymphoma, unspecified subtype. The initial stage was I(EA). His lymphoma was refractory to chemotherapy, and bony metastases developed in the right iliac bone 2 months later. He died of disease after 6 months without autopsy. We emphasize the importance of detailed immunohistochemical and gene rearrangement studies for the classification of malignant lymphomas via a very rare primary bony lymphoma of peripheral T-cell subtype.
原发性骨淋巴瘤罕见，几乎均为高级别B细胞淋巴瘤。自然杀伤（NK）/T细胞淋巴瘤是NK或T细胞谱系的高度侵袭性淋巴瘤，主要表现为结外受累，分为鼻型和鼻外型（鼻外）。我们报告1例酷似鼻型NK/T细胞淋巴瘤的原发性骨外周T细胞淋巴瘤。一名22岁台湾男性因额颅骨肿块就诊，病程3周，接受颅骨切除术并切除肿瘤。其肿瘤显示广泛的凝固性坏死，大的淋巴瘤细胞有血管浸润，这些细胞表达CD2、CD8、CD16、CD43、CD45、CD45RO、CD56、T细胞细胞内抗原-1和颗粒酶B，但不表达CD3、CD4、CD20、CD57、CD68和βF1。原位杂交检测爱泼斯坦-巴尔病毒编码的mRNA为阴性。福尔马林固定组织的聚合酶链反应研究显示T细胞受体γ链基因的克隆重排。诊断为外周T细胞淋巴瘤，未特指亚型。初始分期为I（EA）。其淋巴瘤对化疗耐药，2个月后右侧髂骨出现骨转移。6个月后患者死于疾病，未行尸检。我们通过1例非常罕见的外周T细胞亚型原发性骨淋巴瘤强调详细的免疫组化和基因重排研究对恶性淋巴瘤分类的重要性。
