Clear cell ''sugar'' tumor of the pancreas - A novel member of the family of lesions characterized by the presence of perivascular epithelioid cells
Clear cell ''sugar'' tumor of the pancreas - A novel member of the family of lesions characterized by the presence of perivascular epithelioid cells
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DOI：
10.1097/00000478-199606000-00010
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发表时间：
1996-06-01
影响因子：
5.6
通讯作者：
Bonetti, F
中科院分区：
文献类型：
--
作者：
Zamboni, G;Pea, M;Bonetti, F
我们报告一个独特的，以前未报告的胰腺肿瘤发生在一个60岁的妇女谁是术前诊断为透明细胞癌细胞穿刺。切除的肿瘤由一群大的上皮样细胞组成，细胞质透明或嗜酸性，颗粒状，富含糖原，核多形性，无有丝分裂活性。尽管出现上皮样外观，但肿瘤细胞的上皮（CAM 5.2、KL 1、AE 1-AE 3）、内分泌（神经元特异性烯醇化酶[NSE]、嗜铬粒蛋白A）和腺泡（脂肪酶、淀粉酶）标志物呈阴性，肌动蛋白和黑素生成相关标志物HMB 45呈阳性。超微结构上，肿瘤细胞显示膜结合颗粒，没有证据表明，无论是上皮细胞或黑色素细胞分化。这些形态表型特征在胰腺肿瘤中从未报道过，与肺透明细胞“糖”瘤重叠。在一个以血管周围上皮样细胞为特征的病变家族中也观察到相同的形态表型特征，该家族还包括淋巴管肌瘤病和血管平滑肌脂肪瘤。目前的情况下，可能被认为是一个新的成员，这个家庭的病变。我们建议将这种新的实体命名为胰腺透明细胞“糖"瘤。
We report a unique, previously unreported pancreatic tumor occurring in a 60-year-old woman who was preoperatively diagnosed on cytoaspiration as having clear cell carcinoma. The resected tumor consisted of a population of large epithelioid cells with clear or eosinophilic, granular cytoplasm, rich in glycogen, with nuclear pleomorphism and no mitotic activity. In spite of the epithelioid appearance, the tumor cells were negative for epithelial (CAM 5.2, KL1, AE1-AE3), endocrine (neuron-specific enolase [NSE], chromogranin A), and acinar (lipase, amylase) markers and positive for actin and melanogenesis-related marker HMB 45. Ultrastructurally, the neoplastic cells showed membrane-bound granules; no evidence of either epithelial or melanocytic differentiation was present. These morphophenotypic features have never been reported in a pancreatic tumor and overlap those of clear cell ''sugar'' tumor of the lung. The same morphophenotypic features are observed in a family of lesions characterized by the presence of the perivascular epithelioid cell that also includes lymphangiomyomatosis and angiomyolipoma. The present case may be considered a novel member of this family of lesions. We propose this new entity be named clear cell ''sugar'' tumor of the pancreas.