关键词: Enteroblastic differentiation Hepatoid Neuroendocrine carcinoma Pancreatic ductal adenocarcinoma Rb

来  源:   DOI:10.1016/j.ijscr.2024.109854   PDF(Pubmed)

Abstract:
BACKGROUND: Herein we report a case of an extremely rare pancreatic adenocarcinoma with enteroblastic differentiation (AED), an underrecognized histological subtype. Moreover, the tumor was mixed with a neuroendocrine carcinoma (NEC), which is also a rare malignancy in the pancreas.
METHODS: The patient was an elderly male who was incidentally diagnosed with a 35 mm-sized pancreatic head tumor and underwent pancreatoduodenectomy. Histopathologically, the tumor was composed of four different types: conventional ductal adenocarcinoma, AED, NEC, and squamous cell carcinoma. Interestingly, p53 overexpression and loss of Rb expression, which are characteristic findings of NEC, were observed in all components. He had been received adjuvant chemotherapy after the surgery, however, he died of bath-related cardiac arrest 14 months after surgery.
CONCLUSIONS: In the stomach, AED, a carcinoma resembling fetal gut epithelium, is a rare but established subtype and is considered a related entity of hepatoid carcinoma (HAC). However, gastric AED and HAC differ to some extent. In contrast to the stomach, extragastric AED, including pancreatic AED, is extremely rare, and its biological features are unclear. A mixed tumor with NEC is a complex phenomenon, but it is occasionally reported in extragastric AED. The histogenesis of mixed AED-NEC can be resolved by determining p53 and Rb status.
CONCLUSIONS: Owing to their rare and novel nature, extragastric AED is under-recognized or confused with HAC. Further studies and the establishment of an extragastric AED classification are required.
摘要:
背景:在此,我们报告了一例极为罕见的胰腺腺癌与肠母细胞分化(AED),一种未被认识的组织学亚型。此外,肿瘤与神经内分泌癌(NEC)混合,这也是胰腺中一种罕见的恶性肿瘤。
方法:患者是一名老年男性,偶然诊断为35毫米大小的胰头肿瘤,并接受了胰十二指肠切除术。组织病理学,肿瘤由四种不同类型组成:传统导管腺癌,AED,NEC,和鳞状细胞癌。有趣的是,p53过表达和Rb表达的丧失,这是NEC的特征发现,在所有组件中都观察到。手术后他接受了辅助化疗,然而,他在手术后14个月死于与沐浴相关的心脏骤停。
结论:在胃中,AED,类似胎儿肠道上皮的癌,是一种罕见但确定的亚型,被认为是肝样癌(HAC)的相关实体。然而,胃AED和HAC在一定程度上不同。与胃相反,胃外AED,包括胰腺AED,非常罕见,其生物学特征尚不清楚。混合瘤与NEC是一个复杂的现象,但偶尔会在胃外AED中报告。混合AED-NEC的组织发生可以通过确定p53和Rb状态来解决。
结论:由于其稀有和新颖的性质,胃外AED与HAC的认识不足或混淆。需要进一步的研究和建立胃外AED分类。
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