Primary rare anaplastic large cell lymphoma, ALK positive in small intestine: case report and review of the literature
Primary rare anaplastic large cell lymphoma, ALK positive in small intestine: case report and review of the literature
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DOI：
10.1186/s13000-016-0539-6
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发表时间：
2016-09-09
影响因子：
2.6
通讯作者：
Peng, Tingsheng
中科院分区：
文献类型：
--
作者：
Cao, Qinghua;Liu, Fang;Peng, Tingsheng
背景资料：小肠原发性间变性大细胞淋巴瘤，ALK阳性在临床上罕见，临床、放射学和病理学信息通常不可用。我们报告一位32岁男性空回肠交界处ALK阳性间变性大细胞淋巴瘤的病例，并着重讨论此类型淋巴瘤的临床病理特徴及鉴别诊断。计算机断层扫描（CT）显示空肠和回肠交界处有一个8.5 x 7.4 x 4 cm的肿块。诊断是在通过肠切除术切除的组织的病理学检查后作出的。大体上，肿块主要位于肠壁，呈灰白色，边界模糊。显微镜下，几乎所有层的肠壁被多形性肿瘤细胞浸润，具有弥漫性和粘附性生长模式。肿瘤细胞主要为中型至大型细胞，细胞质中度嗜碱性。大多数细胞核深染，核仁明显。“标志”细胞很容易被检测到。免疫组化显示肿瘤细胞CD 30、ALK、CD 5、TIA-1、Granzyme B、EMA阳性，CD 2、CD 3、CD 7、CD 4、CD 8、CD 20、CD 79 a阴性。EB病毒编码RNA（EBER）基因组也为阴性。最终诊断为原发性小肠ALK阳性间变性大细胞淋巴瘤。结论：原发性小肠ALK阳性间变性大细胞淋巴瘤是一种少见的恶性肿瘤，在临床上是一种少见的恶性肿瘤。正确诊断应结合临床、CT及病理特点，并与其他小肠淋巴瘤或实体瘤相鉴别。
Background: Primary anaplastic large cell lymphoma, ALK positive in small intestine is clinically rare and the clinical, radiological and pathological information are generally not available. Here, we report a case of 32-year-old male with ALK positive anaplastic large cell lymphoma at the junction of jejunum and ileum, and highlight the clinicopathological features and the differential diagnosis of this type lymphoma.Case presentation: The patient presented with right middle abdominal mass for 1 month with sporadic pain. Computed tomography (CT) showed a mass measured 8.5 x 7.4 x 4 cm at the junction of jejunum and ileum. The diagnosis was made after pathological examination of the excised tissue by enterectomy. Grossly, the mass was located predominately in intestinal wall with grayish appearance and blurry boundary. Microscopically, almost all layers of the intestinal wall were infiltrated by pleomorphic tumor cells with diffuse and cohesive growth pattern. The neoplastic cells were mainly medium to large size with moderate basophilic cytoplasm. Most of them had hyperchromatic nuclei and prominent nucleoli. "Hallmark" cells were easily detected. Immunohistochemically, tumor cells are characterized by CD30, ALK, CD5, TIA-1, Granzyme B, EMA positive staining, and CD2, CD3, CD7, CD4, CD8, CD20, CD79a negative staining. The Epstein-Barr virus encoded RNAs (EBERs) genome was also negative. A diagnosis as primary small intestinal ALK positive anaplastic large cell lymphoma was finally made. The patient received CHOP chemotherapy and is alive till now without recurrence 5 months after enterectomy.Conclusions: Primary small intestinal ALK positive anaplastic large cell lymphoma is rare. The accurate diagnosis should be based on combined consideration of clinical characteristics, CT image and pathological features, and should be distinguished from other lymphomas or solid tumors in small intestine.