Department of Hematopathology, The University of Texas M. D. Anderson Cancer Center, Houston, TX 77030, USA.
Ann Diagn Pathol. 2009 Dec;13(6):413-27. doi: 10.1016/j.anndiagpath.2009.09.002.
We saw in consultation a biopsy specimen from a 6-year old girl with anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma (ALCL). The tumor arose in soft tissue of the neck, and diagnostic tissue was obtained by core needle biopsy. Histologically, the neoplasm was cellular without pattern. Immunohistochemical workup with a large panel of antibodies at another institution showed immunoreactivity for NB84 and neuron specific enolase (dim). Antibodies specific for CD3, CD20, and CD45/LCA were negative; CD30 or ALK were not assessed. Electron microscopy showed cytoplasmic structures thought to be neurosecretory granules. The neoplasm was interpreted initially as a neuroblastoma. At the time of our review, we considered the possibility of ALCL. Immunohistochemical analysis for CD30 showed bright, uniform expression and ALK was positive in a nuclear and cytoplasmic pattern, confirming the diagnosis of ALK+ ALCL. The purpose of this review is to discuss ALK+ ALCL and many of the other entities included under the rubric of small round blue cell tumor, with a focus on tumors that occur in children.
我们会诊了一位 6 岁女孩的活检标本，该女孩患有间变性淋巴瘤激酶 (ALK) 阳性间变性大细胞淋巴瘤 (ALCL)。肿瘤发生于颈部软组织，通过芯针活检获得诊断组织。组织学上，肿瘤细胞无明显形态。在另一机构进行的大量抗体免疫组化检测显示 NB84 和神经元特异性烯醇化酶（弱表达）阳性。CD3、CD20 和 CD45/LCA 抗体阴性；未评估 CD30 或 ALK。电子显微镜显示细胞质内存在被认为是神经分泌颗粒的结构。最初，该肿瘤被诊断为神经母细胞瘤。在我们进行复查时，我们考虑了 ALCL 的可能性。CD30 的免疫组化分析显示明亮、均匀的表达，ALK 呈核浆阳性模式，从而确诊为 ALK+ ALCL。本次复习的目的是讨论 ALK+ ALCL 和许多其他归入小圆蓝细胞肿瘤范畴的实体瘤，重点关注发生在儿童中的肿瘤。
