A Rare Case of Intraductal Tubulopapillary Neoplasm of the Pancreas Rupturing and Causing Acute Peritonitis

Abstract

An intraductal tubulopapillary neoplasm (ITPN) is a very rare pancreatic tumor. Here we report an extremely rare case of an ITPN rupturing and causing acute peritonitis. A 50-year-old woman presented with left flank pain and vomiting. A computed tomography (CT) scan revealed gigantic multilocular cysts in the pancreatic tail and massive fluid collection in the abdominal cavity. The serum, urine, and abdominal fluid amylase levels were highly elevated, so she was conservatively treated with intraperitoneal drainage and antibiotics for a diagnosis of ruptured pancreatic cysts. After this patient recovered, a CT scan revealed a 2-cm low-density mass located in the body of the pancreas. This was diagnosed as a pancreatic ductal adenocarcinoma of the pancreatic body with an intraductal papillary mucinous neoplasm, and a distal pancreatectomy was performed. The tumor was composed of cuboidal high-grade dysplastic cells proliferating in a tubulopapillary growth pattern without mucin production. An immunohistochemical examination revealed that the tumor cells were positive for MUC1 and CK7, but negative for MUC5AC. These features led to the final diagnosis of ITPN. In this case, the solid ITPN growth obstructed the lumen of the main pancreatic duct, and the intraductal pressure of the distal side rose gradually. Then, pancreatic cysts formed and burst into the abdominal cavity when the intraductal pressure was at its maximum. However, an ITPN consists of high-grade atypical cells derived from the pancreatic ductal epithelium in principle, so the rupture may be an independent risk factor for peritonitis carcinomatosa in the future.

Intraductal tubulopapillary neoplasms with rupture of the distal main pancreatic duct: a case report

“…However, the rupture of the MPD was extremely rare. A PubMed search using the key words "ITPN" and "rupture" revealed only one case, in which an ITPN with the formation of gigantic pancreatic cysts ruptured and caused acute peritonitis [4]. The authors considered that solid ITPN formation in the MPD had obstructed the flow of pancreatic juice and increased the internal pressure of the distal pancreatic duct, leading to the development of gigantic pancreatic cysts and MPD rupture [4].…”

“…An ITPN usually appears as a solid mass obstructing the main pancreatic duct (MPD), thereby causing distal ductal dilatation [3]. However, ITPN-associated rupture of the MPD is extremely rare, and only one case has been reported to date [4].…”

Total Pancreatectomy with Splenectomy for Multifocal Intraductal Tubulopapillary Neoplasm (ITPN) of the Pancreas Associated with Invasive Component: Report of a Rare Case

“…In published case series, patients with ITPN presented with a variety of symptoms: abdominal pain or discomfort, nausea and vomiting, jaundice, anemia, severe diarrhea or steator-rhea, appetite or weight loss, fever, excessive thirst, exacerbation of diabetes mellitus and acute abdomen due to rupture of ITPN. About one-third of patients were asymptomatic and ITPN was an incidental finding [ 2 , 5 – 8 , 10 , 19 ]. ITPN has also been associated with episodes of recurrent acute pancreatitis and should be considered in the differential diagnosis of idiopathic acute pancreatitis [ 20 ].…”

Intraductal tubular papillary neoplasm (ITPN), a novel entity of pancreatic epithelial neoplasms and precursor of cancer: A case report and review of the literature

“…Reported clinical symptoms included abdominal pain, jaundice, exacerbation of diabetes mellitus, and loss of appetite and weight [9]. Umemura et al described a rare case of an ITPN rupturing and causing acute peritonitis [10]. As far as it is known, there is no sex predilection and ITPNs are not associated with any specific clinical syndrome [6[9].…”