Kodama Takayuki, Tani Akiho, Yamane Hisoka, Itoh Tomoo
Division of Pathology, Department of Pathology, Kobe University Graduate School of Medicine, Kobe 650-0017, Japan; Department of Diagnostic Pathology, Kobe University Hospital, Kobe 650-0017, Japan.
Department of Diagnostic Pathology, Kobe University Hospital, Kobe 650-0017, Japan.
Int J Surg Case Rep. 2024 Jul;120:109854. doi: 10.1016/j.ijscr.2024.109854. Epub 2024 Jun 6.
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.
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.
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.
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.
在此，我们报告一例极为罕见的具有肠母细胞分化的胰腺腺癌（AED），这是一种未被充分认识的组织学亚型。此外，该肿瘤还混合了神经内分泌癌（NEC），这在胰腺中也是一种罕见的恶性肿瘤。
患者为老年男性，偶然发现胰腺头部有一个35毫米大小的肿瘤，随后接受了胰十二指肠切除术。组织病理学检查显示，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，在所有成分中均观察到p53过表达和Rb表达缺失，这是NEC的特征性表现。术后他接受了辅助化疗，然而，术后14个月因沐浴相关的心搏骤停死亡。
在胃中，AED是一种类似于胎儿肠道上皮的癌，是一种罕见但已确定的亚型，被认为是肝样癌（HAC）的相关实体。然而，胃AED和HAC在一定程度上有所不同。与胃不同，包括胰腺AED在内的胃外AED极为罕见，其生物学特征尚不清楚。伴有NEC的混合性肿瘤是一种复杂现象，但偶尔在胃外AED中报道。通过确定p53和Rb状态可以解决混合性AED-NEC的组织发生问题。
由于胃外AED罕见且新颖，其未被充分认识或与HAC混淆。需要进一步研究并建立胃外AED分类。
