Deparment of Pathology, University of Virginia, Charlottesville, USA.
Am J Surg Pathol. 2010 Apr;34(4):510-8. doi: 10.1097/PAS.0b013e3181cfcac7.
BACKGROUND: Pancreatic acinar cell carcinomas (ACCs) are clinically and pathologically distinct from pancreatic ductal adenocarcinomas (PDAs). Whereas endocrine differentiation has been well shown in ACCs, significant ductal components are rare. This paper reviews the clinicopathologic features of a series of ACCs with prominent ductal differentiation. DESIGN: Cases of pancreatic ACCs with significant ductal differentiation were identified in the surgical pathology databases of 2 academic centers. Patient clinical information, gross and histologic features, and histochemical and immunohistochemical (IHC) results were recorded. Cases were tested for KRAS2 mutations. RESULTS: Eleven cases were identified (10 men and 1 woman; age range 52 to 79 y). Four patients presented with jaundice. At last follow-up, 7 patients died of disease and 2 others had recurrences. Tumors measured between 2 and 5.5 cm and were ill-defined, nodular, and multilobulated. Ten were located in the head of the pancreas. All but 2 exhibited extrapancreatic invasion. All cases showed significant evidence of both acinar and ductal differentiation, estimated to be at least 25% of the neoplastic cells, and 3 cases in addition had endocrine differentiation in more than 25% of cells. Five cases were predominately acinar with intracellular and sometimes extracellular mucin ("mucinous acinar cell carcinoma" pattern). Six cases seemed more mixed with areas recapitulating typical PDAs whereas the other portions of the tumors seemed akin to typical acinar cell carcinomas ("combined acinar and ductal" pattern). IHC positive staining results were as: trypsin (92%), chymotrypsin (92%), monoclonal carcinoembryonic antigen (100%), CK19 (100%), B72.3 (73%), CA19.9 (73%), CD56 (18%), synaptophysin (36%), and chromogranin (36%). One case showed p53 over-expression aznd none showed DPC4/Smad4 loss. Two cases had KRAS2 mutations. CONCLUSION: Despite the early embryologic divergence of acinar and ductal cell lineages, rare pancreatic tumors have both acinar and ductal differentiation, usually predominantly the former. The clinical course is highly aggressive.
背景：胰腺腺泡细胞癌 (ACCs) 在临床和病理上与胰腺导管腺癌 (PDAs) 不同。虽然在 ACCs 中已经很好地显示出内分泌分化，但很少有明显的导管成分。本文回顾了一系列具有明显导管分化的胰腺 ACC 的临床病理特征。
设计：在 2 个学术中心的外科病理学数据库中确定了具有显著导管分化的胰腺 ACC 病例。记录了患者的临床信息、大体和组织学特征以及组织化学和免疫组织化学 (IHC) 结果。对 KRAS2 突变进行了检测。
结果：共确定了 11 例病例（10 名男性和 1 名女性；年龄 52 至 79 岁）。4 例患者出现黄疸。在最后一次随访时，7 例患者死于疾病，另外 2 例患者出现复发。肿瘤大小为 2 至 5.5 厘米，边界不清，呈结节状和多叶状。10 例位于胰头部。除 2 例外，所有病例均有胰腺外侵犯。所有病例均显示出明显的腺泡和导管分化证据，估计至少有 25%的肿瘤细胞，另外 3 例的细胞中有超过 25%的内分泌分化。5 例主要为腺泡细胞，伴有细胞内和有时细胞外粘蛋白（“粘蛋白腺泡细胞癌”模式）。6 例似乎更混合，有典型 PDAs 的区域，而肿瘤的其他部分则类似于典型的腺泡细胞癌（“腺泡和导管混合”模式）。免疫组化阳性染色结果为：胰蛋白酶（92%）、糜蛋白酶（92%）、单克隆癌胚抗原（100%）、CK19（100%）、B72.3（73%）、CA19.9（73%）、CD56（18%）、突触素（36%）和嗜铬粒蛋白 A（36%）。1 例显示 p53 过表达，无 1 例显示 DPC4/Smad4 缺失。2 例有 KRAS2 突变。
结论：尽管腺泡和导管细胞谱系在早期胚胎发育上存在分歧，但很少有胰腺肿瘤具有腺泡和导管分化，通常主要是前者。临床病程具有高度侵袭性。
Dig Dis Sci. 2002-10
J Korean Med Sci. 1996-4
Cold Spring Harb Mol Case Stud. 2020-8
Mol Clin Oncol. 2019-5
J Gastrointest Surg. 2019-1-31
Zotero 插件