Folia Medica 63(6): 970-976, doi: 10.3897/folmed.63.e63071
Intraductal Papillary Mucinous Neoplasm of the Pancreas: Need for a Tailored Approach to a Rare Entity
expand article infoMarina Konaktchieva, Dimitar Penchev§, Georgi Popivanov|, Lilia Vladova, Roberto Cirocchi#, Marin Penkov¤, Petko Karagyozov«, Ventsislav Mutafchiyski»
‡ Department of Gastroenterology, Hepatology and Transplantology, Sofia, Bulgaria§ Clinic of Endoscopic, Endocrine surgery and Coloproctology, Military Medical Academy, Sofia, “Sv. G. Sofiiski” Str. 3, Sofia, Bulgaria| Military Medical Academy, Sofia, Bulgaria¶ Department of Tumor morphology, University Hospital for Active Treatment of Oncologic diseases, Sofia, Bulgaria# Department of Surgical Science, University of Perugia,, Perugia, Italy¤ Department of Imaging diagnostic, UHAT "Sv. Ivan Rilski", Sofia, Bulgaria« Department of Interventional Gastroenterology, Acibadem City Clinic Tokuda Hospital,, Sofia, Bulgaria» Department of Surgery, Military Medical Academy, Sofia, Sofia, Bulgaria
Open Access

Intraductal papillary mucinous neoplasm (IPMN) of the pancreas is a relatively new entity that has gained increased attention because of its unique features – presence of different subtypes with different malignant potential, biological behavior, and prognosis, higher rates of recurrences and concomitant or metachronous pancreatic duct cancer. It is rare with an incidence of 4 to 5 cases per 100 000. The relative lack of experience significantly hampers decision making for surgery (pancreatic head resection, distal pancreatectomy or enucleation) or follow-up.

Herein we present two cases managed by diametrically different tactic according to the risk stratification – distal pancreatectomy with splenectomy and observation, respectively. An up-to-date literature review on the key points in diagnostics, indications for surgery, the extent of surgery, follow-up, and prognosis is provided.

The tailored approach based on risk stratification is the cornerstone of management. Absolute indications for surgery are the lesions with high-risk stigmata, whereas the worrisome features should be evaluated by endoscopic ultrasound and fine-needle aspiration. Main duct and mixed type are usually referred to surgery, whereas the management of a branch type is more conservative due to the lower rate of invasive cancer. Strict postoperative follow-up is mandatory even in negative resection margins due to a high risk for recurrences and metachronous lesions.

Despite the guidelines, the intraductal papillary mucinous neoplasm remains a major challenge for clinicians and surgeons in the balance the risk/benefit of observation versus resection. Risk stratification plays a key role in decision-making. Future trials need to determine the optimal period of surveillance and the most reliable predictive factors for concomitant pancreatic duct cancer.

follow-up, imaging diagnostic, intra-ductal papillary mucinous neoplasm, pancreas, surgery, tailored approach