Acinar cell carcinoma of the pancreas with intraductal growth: Report of a case
Acinar cell carcinoma of the pancreas with intraductal growth: Report of a case
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DOI：
10.1097/00006676-200304000-00016
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发表时间：
2003-04-01
期刊：
影响因子：
2.9
通讯作者：
Ishibashi, M
中科院分区：
文献类型：
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作者：
Hashimoto, M;Matsuda, M;Ishibashi, M
accs是一种罕见的胰腺肿瘤，据报道占所有胰腺癌的1%-2%。Klimstra等人(1)和Hoorens等人(2)对ACC进行了广泛的研究，他们分别报道了28例和22例的临床病理特征。与内分泌细胞肿瘤不同，ACCs被认为起源于转化的腺泡细胞(3)。ACC的特点是由过量的脂肪酶产生引起的皮下脂肪坏死。该病例未出现皮下绒毛炎、多发性关节炎、骨内脂肪坏死和嗜酸性粒细胞增多等副肿瘤症状(1)。ACC的症状往往罕见且不特异性（1,2）。在50%的报告病例（平均肿瘤直径≈10 cm）中，肝脏和淋巴结有远处转移。由于该病的罕见性，ACC的影像学诊断存在差异(4)，诊断主要基于组织学特征，如典型的腺泡排列、酶原颗粒的超微结构显示、外分泌胰腺产物（胰蛋白酶、胰凝乳胰蛋白酶、脂肪酶、磷脂酶A2和胰石蛋白）的免疫染色，以及脂肪酶偶有分泌到血清中的情况。在一项研究中，100%的ACCs对胰蛋白酶的免疫反应性为阳性(1)。组织学上，ACC有时难以与神经内分泌肿瘤区分，因为后者通常在神经内分泌标志物（synaptophysin， chromogranin等）上呈阴性。血液中导管细胞标志物（CA19-9和CEA）的水平一般在正常范围内。在大多数报道的ACC病例中，电镜显示与酶原颗粒相对应的大分泌囊泡(5)。ACC通过手术切除治疗。虽然预后不是很好，但有报道称其优于胰腺管细胞癌（1,2）。
DISCUSSIONACCs are rare tumors of the pancreas, reported to represent 1%–2% of all pancreatic cancers. Extensive studies of ACC have been carried out by Klimstra et al.(1) and Hoorens et al.(2), who reported detailed descriptions of the clinicopathologic features of 28 and 22 cases, respectively. ACCs, unlike endocrine cell tumors, are believed to derive from transformed acinar cells (3).The peculiarity of ACC is the extreme subcutaneous fat necrosis resulting from excessive lipase production. Paraneoplastic symptoms such as subcutaneous panniculitis, polyarthritis, intraosseous fat necrosis, and eosinophilia were not present in this case (1). Symptoms of ACC tend to be rare and not specific (1, 2). In 50% of reported cases (mean tumor diameter,≈ 10 cm), there are distant metastases in the liver and lymph nodes. The imaging diagnosis of ACC varies due to the rarity of the disease (4), and diagnosis is mainly based on histologic features such as typical acinar arrangement, ultrastructual demonstration of zymogen granules, immunostaining for exocrine pancreatic products (trypsin, chymotrypsin, lipase, phospholipase A2, and pancreatic stone protein), and occasional secretion of lipase into the serum. In one study, immunoreactivity to trypsin was positive in 100% of ACCs (1). Histologically, ACC is sometimes difficult to differentiate from neuroendocrine tumors, since the latter are usually negative for neuroendocrine markers (synaptophysin, chromogranin, etc.). The blood levels of duct cell markers (CA19–9 and CEA) are generally within normal limits. In most reported cases of ACC, electron microscopy revealed large secretory vesicles corresponding to zymogen granules (5). ACC is treated by surgical resection. Although the prognosis is not very good, it is reported to be better than that of duct cell carcinoma of the pancreas (1, 2).