A Case of Pancreatic Undifferentiated Carcinoma Mimicking Proximal-Type Epithelioid Sarcoma
A Case of Pancreatic Undifferentiated Carcinoma Mimicking Proximal-Type Epithelioid Sarcoma
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模仿近端型上皮样肉瘤的胰腺未分化癌一例
DOI：
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发表时间：
2016
期刊：
影响因子：
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通讯作者：
Y. Oda
中科院分区：
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作者：
Nobuyuki Ohike;Reika Suzuki;T. Isobe;Tomoko Norose;A. Shiokawa;T. Kunimura;Kenichi Kohashi;Y. Oda
我们在此报告一例胰腺未分化/间变性癌，病理上类似近端类型上皮样肉瘤。患者是一名35岁的女性，主诉上腹痛和背部疼痛，并出现焦油状大便和体重减轻。腹部影像显示直径大于6厘米的出血胰腺肿块和多发性肝脏肿块。肝脏活检显示有恶性细胞，并接受了TS-1化疗，然而，患者通过弥漫性腹膜播散积累了大量腹水，并在7个月后死亡。尸检显示一个巨大的白色膨胀性浸润性肿块，伴有严重出血，大小为18x13厘米，主要位于胰腺头部。显微镜下，肿瘤呈髓样生长，由多形性梭形到上皮样细胞组成，这些细胞松散地粘连在一起，并含有横纹肌样形态。腺体成分，提示导管腺癌，即使在广泛的取样中也找不到。免疫组织化学染色显示细胞角蛋白(AE1/AE3)、上皮膜抗原、波形蛋白和CD34呈弥漫性阳性，而特异性分化标记物阴性。此外，还观察到SMARCB1/INI-1蛋白表达的缺失，尽管其变化在脱氧核糖核酸水平上尚未得到证实。未检测到KRAS突变。该肿瘤与Agaimy A等人最近提出的胰腺未分化横纹肌样癌的单形性间变性亚型相似，被认为是胰腺未分化/间变性癌。然而，其组织学、免疫组织学和分子生物学特征与PES完全相同，因此建议临床治疗和护理近侧型上皮样肉瘤，而不是将未分化/间变癌作为导管腺癌的一个亚型。
We herein report a case of pancreatic undifferentiated/anaplastic carcinoma pathologically mimicking proximal-type epithelioid sarcoma. The patient was a 35-year-old female who complained of epigastralgia and back pain and presented with tarry stools and weight loss. A growing, hemorrhagic pancreatic mass more than 6 cm in diameter and multiple liver masses were revealed on abdomen images. A liver biopsy showed malignant cells and chemotherapy using TS-1 was performed, however, the patient accumulated a large amount of ascites by diffuse peritoneal dissemination and died after seven months. An autopsy demonstrated the manifestation of a large whitish, expansive-infiltrative mass with severe hemorrhage, measuring 18 x 13 cm, seated primarily in the head of the pancreas. Microscopically, the tumor showed a medullary growth consisted of pleomorphic spindle to epithelioid cells, which were loosely cohesive and included rhabdoid morphology. The glandular component, suggestive of ductal adenocarcinoma, could not be found even with extensive sampling. Immunohistochemical studies showed a diffuse positivity of cytokeratin (AE1/AE3), epithelial membrane antigen, vimentin, and CD34 and a negativity of specific differentiation markers. In addition, a loss of SMARCB1/INI-1 protein expression was observed, although its alterations were not confirmed at the deoxyribonucleic acid level. No KRAS mutations were detected. The tumor was considered as pancreatic undifferentiated/anaplastic carcinoma from the similarity to "monomorphic anaplastic subtype of pancreatic undifferentiated rhabdoid carcinoma" recently proposed by Agaimy A et al. However, its histological, immunohistological and molecular characters were completely identical to those of PES, thus the clinical treatment and care for proximal-type epithelioid sarcoma may be recommended rather than those for undifferentiated/anaplastic carcinoma as a subtype of ductal adenocarcnoma.