Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Fukuoka, 812-8582, Japan.
Department of Surgery and Oncology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Virchows Arch. 2018 Aug;473(2):247-252. doi: 10.1007/s00428-018-2398-x. Epub 2018 Jun 24.
Imaging of a 53-year-old Japanese man revealed two tumors in the liver and a tumor in the head of the pancreas with a swelling lymph node. A needle biopsy for the liver tumors was performed, revealing a neuroendocrine tumor. Enucleation, lymphadenectomy, and partial hepatectomy were performed. The microscopic examination identified many tumor cells with intracytoplasmic inclusions arranged in a nested, cord, or tubular fashion. The intracytoplasmic inclusions displayed densely eosinophilic globules and displaced the nuclei toward the periphery, which constitutes "rhabdoid" features. The tumor cells were positive for synaptophysin and weakly positive for NCAM, but negative for chromogranin A. Epithelial markers (AE1/AE3 and CAM5.2) accentuated intracytoplasmic globules. Pancreatic neuroendocrine tumors with rhabdoid features are very rare. Generally, rhabdoid features are aggressive and dedifferentiated characteristics of various types of tumor. Pancreatic neuroendocrine tumors containing rhabdoid cells tend to display extrapancreatic spread at the time of presentation, although some of these tumors with rhabdoid features are not always associated with aggressive behavior.
一位 53 岁的日本男性的影像学检查显示肝脏有两个肿瘤和胰腺头部有一个肿瘤，还有一个淋巴结肿大。对肝脏肿瘤进行了针吸活检，结果显示为神经内分泌肿瘤。进行了肿瘤剜除术、淋巴结切除术和部分肝切除术。显微镜检查发现许多肿瘤细胞的胞质内有排列呈巢状、索状或管状的包涵体。胞质内包涵体显示密集的嗜酸性小球，将细胞核推向边缘，构成“横纹肌样”特征。肿瘤细胞对突触素呈阳性反应，对 NCAM 呈弱阳性反应，但对嗜铬粒素 A 呈阴性反应。上皮标记物（AE1/AE3 和 CAM5.2）突出了胞质内的小球。具有横纹肌样特征的胰腺神经内分泌肿瘤非常罕见。一般来说，横纹肌样特征是各种类型肿瘤的侵袭性和去分化特征。具有横纹肌样特征的胰腺神经内分泌肿瘤在发病时往往有胰腺外扩散的趋势，尽管其中一些具有横纹肌样特征的肿瘤并不总是与侵袭性行为相关。
