Fujita Hiroaki, Ishido Keinosuke, Kimura Norihisa, Wakiya Taiichi, Nagase Hayato, Yoshizawa Tadashi, Haga Toshihiro, Goto Shintaro, Kijima Hiroshi, Hakamada Kenichi
Department of Gastroenterological Surgery, Hirosaki University Graduate School of Medicine, Hirosaki, Japan.
Department of Pathology and Bioscience, Hirosaki University Graduate School of Medicine, Hirosaki, Japan.
Surg Case Rep. 2021 Jan 15;7(1):25. doi: 10.1186/s40792-020-01045-y.
Intra-ampullary papillary-tubular neoplasm (IAPN) has been classified as a Vater papillary tumor. The prognosis of IAPN is generally relatively good. Here, we describe a patient with a mucinous adenocarcinoma cluster in the Vater papilla of IAPN origin.
The patient was a 66-year-old man who was admitted to our hospital after a diagnosis of pancreatic head carcinoma based on a pancreatic duct dilatation found on abdominal ultrasound. CT showed a 40 mm lesion in the pancreatic head and expansion of the main pancreatic duct to a maximum diameter of 9 mm on the caudal side of the lesion. The extrahepatic bile duct had also expanded to a maximum diameter of 8 mm. PET/CT showed fluorodeoxyglucose (FDG) accumulation of SUV 6.02 that corresponded to the tumor in the pancreatic head, though it did not suggest distant metastasis. The patient was diagnosed with pancreatic head carcinoma T3 N0 M0 Stage IIA and underwent a pancreaticoduodenectomy. Pathology indicated that the tumor in the pancreatic head was a benign inflammatory lesion. On the other hand, the papillotubular tumor pervading the lumen in the duodenal papillary common channel met the criteria for IAPN, and a mucinous adenocarcinoma cluster found in the surrounding stroma suggested malignant transformation of IAPN. No metastasis to lymph nodes was demonstrated. With regard to the mucus phenotype of each lesion, the IAPN was MUC2 and MUC5AC positive, while the mucinous adenocarcinoma was MUC2-positive and MUC5AC-negative. In addition, CD10 was negative in both lesions, suggesting that mucus transformation from the gastric type to the intestinal type was a key element. A blood test 10 months after surgery showed increased CA19-9 (105 U/mL) and CEA (7.1 ng/mL). Abdominal CT showed multiple cystoid nodes in the liver, which were diagnosed as multiple liver metastases of mucinous adenocarcinoma transformed from the IAPN.
We reported a case with IAPN that developed in the Vater papilla, which took an extremely malignant course. IAPN generally has a good prognosis, but it is important to understand that a malignant course may occur.
壶腹内乳头状管状肿瘤（IAPN）已被归类为 Vater 乳头肿瘤。IAPN 的预后通常相对较好。在此，我们描述一例起源于 IAPN 的 Vater 乳头处伴有黏液腺癌灶的患者。
患者为一名 66 岁男性，因腹部超声发现胰管扩张而被诊断为胰头癌后入住我院。CT 显示胰头有一个 40mm 的病灶，主胰管在病灶尾侧扩张至最大直径 9mm。肝外胆管也扩张至最大直径 8mm。PET/CT 显示胰头肿瘤处氟脱氧葡萄糖（FDG）摄取，SUV 值为 6.02，虽未提示远处转移。患者被诊断为胰头癌 T3 N0 M0 ⅡA 期，并接受了胰十二指肠切除术。病理显示胰头肿瘤为良性炎性病变。另一方面，弥漫于十二指肠乳头共同通道管腔的乳头管状肿瘤符合 IAPN 标准，且在周围间质中发现的黏液腺癌灶提示 IAPN 发生了恶变。未发现淋巴结转移。关于每个病灶的黏液表型，IAPN 为 MUC2 和 MUC5AC 阳性，而黏液腺癌为 MUC2 阳性、MUC5AC 阴性。此外，两个病灶的 CD10 均为阴性，提示从胃型黏液向肠型黏液的转变是一个关键因素。术后 10 个月的血液检查显示 CA19 - 9（105U/mL）和 CEA（7.1ng/mL）升高。腹部 CT 显示肝脏有多个囊状结节，诊断为 IAPN 转变而来的黏液腺癌多发肝转移。
我们报告了一例发生在 Vater 乳头的 IAPN 病例，其病程极为凶险。IAPN 一般预后良好，但了解其可能发生恶性病程很重要。
