Shimizu Yuji, Ashida Ryo, Sugiura Teiichi, Okamura Yukiyasu, Ito Takaaki, Yamamoto Yusuke, Ohgi Katsuhisa, Sasaki Keiko, Uesaka Katsuhiko
Division of Hepato-Biliary-Pancreatic Surgery, Shizuoka Cancer Center, 1007 Shimo-Nagakubo, Sunto-Nagaizumi, Shizuoka, 411-8777, Japan.
Division of Pathology, Shizuoka Cancer Center, 1007 Shimo-Nagakubo, Sunto-Nagaizumi, Shizuoka, 411-8777, Japan.
Surg Case Rep. 2020 Aug 14;6(1):210. doi: 10.1186/s40792-020-00972-0.
Intraductal tubulopapillary neoplasm (ITPN) is a rare and newly described entity defined as an intraductal, grossly visible, tubule-forming epithelial neoplasm with high-grade dysplasia and ductal differentiation without overt production of mucin. Because of its rarity, the clinical and molecular aspects of ITPN have not been fully investigated.
A 73-year-old woman presented to a local hospital with epigastric discomfort and pain. Abdominal multidetector-row computed tomography (MDCT) revealed a 2.5-cm hypovascular tumor in the pancreatic body with distal pancreatic duct dilatation and a slightly low-density area spreading over the ventral side of the pancreatic body. Endoscopic ultrasonography and fine-needle biopsy of the tumor revealed adenocarcinoma of the pancreas. She was referred to our hospital 2 months later. MDCT performed at our hospital showed no significant change in the tumor size or pancreatic duct dilatation. However, the low-density area at the ventral side of the pancreas had shrunk; therefore, this finding was considered to have been an inflammatory change. Under a preoperative diagnosis of resectable pancreatic ductal adenocarcinoma, distal pancreatectomy was performed. The final diagnosis was ITPN with associated invasive carcinoma. Macroscopically and microscopically, the main pancreatic duct (MPD) had ruptured at the distal side of the tumor, and the fistula connected the MPD and extrapancreatic scar tissue.
ITPN with rupture of the pancreatic duct is extremely rare. In the present case, a sudden increase in the pancreatic duct internal pressure or acute inflammation likely caused the rupture of the MPD.
导管内小管状乳头状肿瘤（ITPN）是一种罕见的新描述实体，定义为导管内肉眼可见的形成小管的上皮性肿瘤，具有高级别异型增生和导管分化，无明显黏液分泌。由于其罕见性，ITPN的临床和分子方面尚未得到充分研究。
一名73岁女性因上腹部不适和疼痛就诊于当地医院。腹部多排螺旋计算机断层扫描（MDCT）显示胰体部有一个2.5厘米的低血供肿瘤，伴有远端胰管扩张，以及一个略低密度区域蔓延至胰体腹侧。肿瘤的内镜超声检查和细针穿刺活检显示为胰腺癌。2个月后她被转诊至我院。我院进行的MDCT显示肿瘤大小和胰管扩张无明显变化。然而，胰腺腹侧的低密度区域缩小了；因此，这一发现被认为是炎症改变。在术前诊断为可切除的胰腺导管腺癌的情况下，进行了远端胰腺切除术。最终诊断为ITPN合并浸润性癌。大体和显微镜下，主胰管（MPD）在肿瘤远端破裂，瘘管连接MPD和胰腺外瘢痕组织。
伴有胰管破裂的ITPN极为罕见。在本病例中，胰管内压力突然升高或急性炎症可能导致了MPD破裂。
