Intrapancreatic recurrence of intraductal tubulopapillary neoplasm (ITPN) 16 years after the initial surgery for noninvasive ITPN: a case report

Abstract

BackgroundIntraductal tubulopapillary neoplasm (ITPN) is a rare pancreatic intraductal neoplasm. It is characterized by a tubulopapillary growth pattern, entirely high-grade atypical cells, minimal cytoplasmic mucin, and no obvious luminal mucin secretion. Most of its biological nature remains unclear.Case presentationWe herein report a case of intrapancreatic recurrence of ITPN in the remnant pancreas of a patient who underwent pancreatoduodenectomy 16 years previously for a noninvasive intraductal pancreatic head tumor. We reexamined the primary tumor and compared it with the most recently resected specimen. Histologically, the primary tumor showed a tubulopapillary growth of high-grade atypical cells with scanty cytoplasmic mucin, which was similar to the recently resected specimen except for the invasive area. Immunohistochemically, the neoplastic cells in both specimens showed focal staining of MUC1 and positivity for MUC6 but negativity for MUC2, MUC5AC, CDX2, and trypsin. Molecular analysis revealed no KRAS/GNAS/BRAF/PIK3CA mutations in either of the specimens.ConclusionsThese findings of the original tumor and recently resected tumor were compatible with the features of ITPN. Thus, recurrence is possible even for a primary noninvasive ITPN, and long-term surveillance is recommended.

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

“…Intraductal tubulopapillary neoplasms (ITPNs) of the pancreas account for less than 1% of all pancreatic exocrine tumors and approximately 3% of all pancreatic intraductal neoplasms [ 1 , 6 , 7 ]. Intraductal tumors of the pancreas are classified as intraductal pancreatic mucinous neoplasms (IPMNs) and ITPNs according to the 4 th World Health Organization (WHO) tumor classification in 2010 [ 4 – 9 ].…”

“…Recurrence of ITPN may require total pancreatectomy. Additionally, recurrence has occurred even in cases where the initial ITPN was not associated with invasive carcinoma [ 1 , 9 , 28 ]. Three possible mechanisms have been proposed to elucidate this phenomenon: a) residual cancer cells in the remnant pancreas, b) intraductal or intrapancreatic lymphovascular spread, and c) metachronous, multicentric development [ 9 , 28 ].…”

“…Two of them were alive and disease-free 15 and 19 months after surgery [ 2 , 23 ], whereas the third was diagnosed with hepatic metastases in the fourth postoperative month [ 18 ]. There were also 4 reported cases of patients from Japan with pancreatic ITPN who received postoperatively S-1 (tegafur-gimeracil-oteracil potassium) [ 9 , 20 , 30 , 31 ]. S-1 plays a key role in the treatment of pancreatic cancer in Japan besides gemcitabine [ 32 ].…”

“…S-1 plays a key role in the treatment of pancreatic cancer in Japan besides gemcitabine [ 32 ]. Three of these cases were diagnosed with recurrence of the primary disease at 15 months [ 30 ], 34 months [ 31 ] and 16 years after pancreatoduodenectomy [ 9 ]. The fourth was alive and disease-free in the first postoperative year [ 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

“…Even in case of ITPN associated carcinoma, patients revealed an excellent overall 5-year survival of 81.5%, which is more favorable when compared to ductal adenocarcinoma of the pancreas or even IPMN associated pancreatic cancer [8,9]. However, there are patients who showed local recurrent disease [16], or even died of ITPN due to multiple liver metastases [6]. The latter may indicate that oncological resection is warranted before a systemic spread of the tumor occurs.…”