Department of Pathology and Laboratory Medicine, Vancouver General Hospital, Canada.
Ann Hepatol. 2012 Sep-Oct;11(5):715-20.
Primary hepatic neuroendocrine tumours are rare tumours effecting relatively young patients. As metastatic neuroendocrine tumours to the liver are much more common, extensive investigations are crucial to exclude a primary tumour elsewhere. We report a case of a 27 year old woman who presented with fatigue, increased abdominal girth and feeling of early satiety and bloating. Extensive work up failed to show tumour at another primary site. Hepatic artery embolization showed no effect, so the patient underwent total hepatectomy and live-donor liver transplant. Grossly the tumour measured 27 cm. Microscopic examination showed bland, monomorphic cells growing in tubuloglandular and trabecular growth patterns. Cells were positive for neuroendocrine (synaptophysin, chromogranin, CD56) and epithelial markers (MOC31, CK7, CK19). Cytoplasmic dense neurosecretory vesicles were seen on ultrastructural examination. Based on the Ki-67 rate, mitotic count, lack of marked nuclear atypia and absence of necrosis, a diagnosis of primary neuroendocrine grade 2 was conferred.
原发性肝神经内分泌肿瘤是一种罕见的肿瘤，主要影响相对年轻的患者。由于转移性神经内分泌肿瘤到肝脏更为常见，因此广泛的检查对于排除其他部位的原发性肿瘤至关重要。我们报告了一例 27 岁女性患者，其主要表现为疲劳、腹部增大、早饱和腹胀。广泛的检查未能发现其他原发性肿瘤部位的肿瘤。肝动脉栓塞治疗没有效果，因此患者接受了全肝切除术和活体供肝移植。大体上肿瘤大小为 27 厘米。显微镜下观察到良性、形态单一的细胞呈管状和小梁状生长方式生长。细胞对神经内分泌标志物（突触素、嗜铬粒素、CD56）和上皮标志物（MOC31、CK7、CK19）呈阳性。电镜下可见细胞质内致密的神经分泌小泡。根据 Ki-67 率、有丝分裂计数、核异型性不明显和无坏死，诊断为原发性神经内分泌肿瘤 2 级。
