Ohmoto Takuji, Yoshitani Nobuyuki, Nishitsuji Kazuchika, Takayama Tetsuji, Yanagisawa Yuto, Takeya Motohiro, Sakashita Naomi
Undergraduate student, The University of Tokushima School of Medicine, Tokushima, Japan; Department of Human Pathology, Institute of Health Biosciences, The University of Tokushima Graduate School, Tokushima, Japan.
Pathol Int. 2015 May;65(5):264-70. doi: 10.1111/pin.12283. Epub 2015 Mar 9.
Carcinoma with rhabdoid features is a rare malignant tumor with a poor prognosis whose molecular mechanism for aggressive behavior is unclear. We describe an undifferentiated pancreatic carcinoma with rhabdoid features that demonstrated extensive invasion and metastasis. Examination of a 63-year-old man with back pain disclosed a retroperitoneal tumor with multiple metastases. Lymph node biopsy revealed an undifferentiated carcinoma of unknown origin. Intensive chemotherapy was ineffective; the patient died 3 months after initial symptoms. Autopsy showed that the tumor displaced the retroperitoneal space: it diffusely invaded and destroyed the pancreas and duodenum. Histology demonstrated tumor cells with eccentric vesicular nuclei, large nucleoli, juxtanuclear eosinophilic inclusions, and poor cell adhesion. Immunohistochemistry showed that tumor cells expressed cytokeratin and vimentin, and electron microscopy confirmed a perinuclear mass of intermediate fibrils and lipid droplets, which indicated an undifferentiated carcinoma with rhabdoid features. Tumor tissue contained hyaluronan; tumor cells strongly expressed CD44, matrix metalloproteinase-9, hypoxia-inducible factor-1α, hyaluronan synthase 2, and acyl-CoA:cholesterol acyltransferase 1 and had a high Ki-67(+) ratio. Since hyaluronan is a ligand for CD44, formation of CD44-hyaluronan complex on the cell surface activates CD44 and this activation may explain why the tumor manifested aggressive invasion and metastasis throughout the clinical course.
具有横纹肌样特征的癌是一种罕见的恶性肿瘤，预后较差，其侵袭性行为的分子机制尚不清楚。我们描述了一例具有横纹肌样特征的未分化胰腺癌，该肿瘤表现出广泛的侵袭和转移。一名63岁背痛男性的检查发现腹膜后肿瘤伴多处转移。淋巴结活检显示为来源不明的未分化癌。强化化疗无效；患者在出现初始症状3个月后死亡。尸检显示肿瘤占据腹膜后间隙：弥漫性侵袭并破坏胰腺和十二指肠。组织学显示肿瘤细胞具有偏心泡状核、大核仁、核旁嗜酸性包涵体以及细胞黏附性差。免疫组化显示肿瘤细胞表达细胞角蛋白和波形蛋白，电子显微镜证实核周有中间丝和脂滴团块，提示为具有横纹肌样特征的未分化癌。肿瘤组织含有透明质酸；肿瘤细胞强烈表达CD44、基质金属蛋白酶-9、缺氧诱导因子-1α、透明质酸合酶2和酰基辅酶A：胆固醇酰基转移酶1，且Ki-67(+)比例较高。由于透明质酸是CD44的配体，细胞表面CD44-透明质酸复合物的形成激活了CD44，这种激活可能解释了肿瘤在整个临床过程中表现出侵袭性侵袭和转移的原因。
