Primary central nervous system ALK-positive anaplastic large cell lymphoma with CD56 abnormally expression in a Chinese child: Challenge in diagnostic practice

Primary central nervous system ALK-positive anaplastic large cell lymphoma with CD56 abnormally expression in a Chinese child: Challenge in diagnostic practice
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中国儿童原发性中枢神经系统 ALK 阳性间变性大细胞淋巴瘤伴 CD56 异常表达:诊断实践中的挑战

DOI:
10.1177/2058738420941756
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发表时间:
2020-07-01
影响因子:
3.5
通讯作者:
Zhang, Wenyan
Zhang, Wenyan
中科院分区:
医学4区
文献类型:
--
作者:
Liu, Qian;Chen, Xian;Zhang, Wenyan

文献摘要

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间变性淋巴瘤激酶(ALK)阳性间变性大细胞淋巴瘤(ALK + ALCL)最常见于青年,具有广泛的形态谱。然而,累及中枢神经系统(CNS)绝对是罕见的。我们报告的病例是一名12岁的中国男性,他表现为头痛和呕吐几天。肿瘤成分为类似星空生长模式的小细胞,CD30、ALK1和CD56免疫组化染色阳性。利用ALK分离探针荧光原位杂交(FISH)检测到与ALK相关的单克隆t细胞受体(TCR γ)基因重排和易位,支持ALK + ALCL的诊断。本病例显示ALK + ALCL发生在CD56异常表达的罕见部位。认识这个实体是重要的,以区分它与其他颅内淋巴瘤。
Anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALK + ALCL) is most frequent in youth and possesses a broad morphologic spectrum. However, involvement in central nervous system (CNS) is definitely rare. The case we presented was a 12-year-old Chinese male who presented with headache and emesis for a couple of days. The neoplastic component was smaller cells resembling starry-sky growth pattern and immunohistochemical stained positively for CD30, ALK1, and CD56. Monoclonal T-cell receptor (TCR gamma) gene rearrangement and gene translocation involving ALK identified by fluorescence in situ hybridization (FISH) using ALK break apart probe supported the diagnosis of ALK + ALCL. This case showed ALK + ALCL occur in a rare site with an abnormal CD56 expression. Awareness of this entity is important to distinguish it from other intracranial lymphoma.