Watanabe Kazuo, Ogura Go, Suzuki Toshimitsu
Pathology Division, Fukushima Medical University School of Medicine Hospital, Fukushima, Japan.
Pathol Int. 2003 Jun;53(6):396-400. doi: 10.1046/j.1440-1827.2003.01487.x.
We describe a distinctive neuroendocrine carcinoma (NEC) that proliferated intra-epithelially. The tumor was 35 mm in diameter and arose from the right superior turbinate of a 46-year-old woman. Histologically, the tumor exhibited papillary growth and the tumor cells were localized in the thickened mucosal epithelium. The tumor cells had round to oval and vesicular or hyperchromatic nuclei, and cohered without any specific structure such as a fibrillary background, rosette or glandular structure. No stromal invasion by the tumor was observed. Immunohistochemically, the tumor cells were positive for neuron-specific enolase focally. In addition, many tumor cells expressed cytokeratin (AE1/AE3 and CAM 5.2), mostly with characteristic perinuclear dot-like patterns. Electron microscopy revealed focal but well-eveloped cytoplasmic processes containing arrays of microtubules and a few dense core granules. The tumor was considered to be a poorly differentiated neuroendocrine carcinomas (NEC) that exhibited exceptional intra-epithelial proliferation. The tumor completely disappeared after the stereotactic radiosurgery and has not recurred for 40 months. It might be difficult to distinguish a poorly differentiated NEC in the sinonasal region from other neuroectodermal tumors, including olfactory neuroblastoma, but the differential diagnosis is important because each tumor has different clinicopathological characteristics.
我们描述了一种独特的上皮内增殖性神经内分泌癌(NEC)。该肿瘤直径为35毫米,起源于一名46岁女性的右上鼻甲。组织学上,肿瘤呈乳头状生长,肿瘤细胞位于增厚的黏膜上皮内。肿瘤细胞具有圆形至椭圆形以及泡状或深染的细胞核,相互黏附,无任何特异性结构,如纤维性背景、菊形团或腺管结构。未观察到肿瘤的间质浸润。免疫组织化学检查显示,肿瘤细胞局灶性神经元特异性烯醇化酶呈阳性。此外,许多肿瘤细胞表达细胞角蛋白(AE1/AE3和CAM 5.2),大多呈特征性的核周点状模式。电子显微镜检查发现局灶性但发育良好的细胞质突起,内含微管阵列和一些致密核心颗粒。该肿瘤被认为是一种低分化神经内分泌癌(NEC),表现出异常的上皮内增殖。立体定向放射治疗后肿瘤完全消失,40个月未复发。在鼻窦区域,低分化NEC可能难以与其他神经外胚层肿瘤,包括嗅神经母细胞瘤相鉴别,但鉴别诊断很重要,因为每种肿瘤具有不同的临床病理特征。