Fritz Stefan, Küper-Steffen Regina, Feilhauer Katharina, Sommer Christof M, Richter Götz M, Bosse Alexander, Hennig René, Köninger Jörg
Department of General, Visceral, Thoracic and Transplantation Surgery, Department of Pathology, Clinics for Diagnostic and Interventional Radiology, Katharinenhospital Klinikum Stuttgart, Germany.
Department of General, Visceral, Thoracic and Transplantation Surgery, Department of Pathology, Clinics for Diagnostic and Interventional Radiology, Katharinenhospital Klinikum Stuttgart, Germany.
Int J Surg Case Rep. 2019;55:187-191. doi: 10.1016/j.ijscr.2019.01.036. Epub 2019 Feb 5.
Intraductal tubular papillary neoplasm (ITPN) displays a very rare subtype of epithelial neoplasms of the pancreas. ITPN is characterized by intraductal tubulopapillary growth and cellular dysplasia. In contrast to intraductal papillary neoplasm (IPMN) no overt epithelial mucin production is observed. To date, little is known about ITPN and particularly about pancreatic cancer arising in this tumor entity.
A 68-year-old male presented at our hospital with a distal bile duct occlusion suspicious for adenocarcinoma of the pancreatic head. Preoperative staging revealed no signs of distant metastasis. The patient was surgically explored and pylorus preserving duodenopancreatectomy was performed for a solid pancreatic head tumor. Final histopathology surprisingly revealed an ITPN with an associated invasive carcinoma pT3, pN0 (0/12), R0, G2.
Patients with ITPN frequently present with jaundice suspicious for a bile duct stenosis or a malignant tumor of the pancreatic head. Although, it is possible to diagnose ITPN by endoscopic retrograde cholangiopancreaticography, many tumors are found not before histopathological examination. Differential diagnosis includes ductal adenocarcinoma of the pancreas, neuroendocrine tumors, IPMN, distal bile duct tumors, and solid pseudopapillary neoplasms. Using immunohistochemistry, other entities of pancreatic tumors can be ruled out. In case of R0 resection oncological prognosis is described to be more favorable when compared to regular ductal adenocarcinoma.
ITPN displays a rare entity of pancreatic neoplasms. As shown in the present case report, there is a relevant potential of malignant transformation and therefore radical surgical resection and oncologic follow-up is warranted.
导管内管状乳头状肿瘤(ITPN)是胰腺上皮性肿瘤中一种非常罕见的亚型。ITPN的特征是导管内呈小管状乳头状生长及细胞发育异常。与导管内乳头状瘤(IPMN)不同,未观察到明显的上皮黏液产生。迄今为止,对ITPN了解甚少,尤其是对起源于该肿瘤实体的胰腺癌更是知之甚少。
一名68岁男性因怀疑胰头腺癌导致远端胆管梗阻入住我院。术前分期未发现远处转移迹象。对该患者进行了手术探查,并针对胰腺实性肿块行保留幽门的十二指肠胰头切除术。最终组织病理学检查结果令人惊讶,发现为ITPN伴浸润性癌,pT3,pN0(0/12),R0,G2。
ITPN患者常表现为黄疸,怀疑是胆管狭窄或胰头恶性肿瘤。虽然通过内镜逆行胰胆管造影有可能诊断ITPN,但许多肿瘤直到组织病理学检查时才被发现。鉴别诊断包括胰腺导管腺癌、神经内分泌肿瘤、IPMN、远端胆管肿瘤和实性假乳头状肿瘤。通过免疫组织化学可排除其他胰腺肿瘤实体。在R0切除的情况下,与普通导管腺癌相比,其肿瘤学预后被认为更有利。
ITPN是一种罕见的胰腺肿瘤实体。如本病例报告所示,其存在恶性转化的相关可能性,因此有必要进行根治性手术切除及肿瘤学随访。