PMID- 31450729 OWN - NLM STAT- MEDLINE DCOM- 20200224 LR - 20200224 IS - 1648-9144 (Electronic) IS - 1010-660X (Print) IS - 1010-660X (Linking) VI - 55 IP - 9 DP - 2019 Aug 23 TI - Pancreatic Polypeptide-Secreting Tumour of the Proximal Pancreas (PPoma)-Ultra Rare Pancreatic Tumour: Clinically Malign, Histologically Benign. LID - 10.3390/medicina55090523 [doi] LID - 523 AB - Background and objectives: Here we report a rare case of a pancreatic polypeptide-secreting tumour (PPoma) discovered by accident during an autopsy. These PPomas occur in less than 2% of all pancreatic neoplasms and are almost exclusively silent, i.e., they are non-functional. Symptoms arising from PPoma are due to its compression of surrounding tissue. Materials and methods: The autopsy was performed on a 68-year-old male diagnosed with multiple endocrine neoplasm type 1 (MEN1) due to the patient's sudden death. Results: A solitary, densely fibrotic, pink-brown tumour, 18 mm in size tumorous mass, was localised in the head of the pancreas. Microscopically, the tumour had a glandular structure with a tubuloacinar arrangement of the cells. Immunohistochemically, we detected strong PP (pancreatic polypeptide) intracytoplasmic activity and negative glucagon activity. The PPoma was located in the head of the pancreas, likely resulting in the obstruction of the main pancreatic and common bile duct. Conclusions: To the best of our knowledge, this is the first report suggesting the association of PPomas with MEN1. Also, the PPoma could be the cause of acute hemorrhagic pancreatitis due to its location. FAU - Ilic, Ivan AU - Ilic I AD - Medical Faculty of Nis, University of Nis, 18000 Nis, Serbia. FAU - Katic, Vuka AU - Katic V AD - Policlinic Human, 18000 Nis, Serbia. FAU - Randjelovic, Pavle AU - Randjelovic P AD - Medical Faculty of Nis, University of Nis, 18000 Nis, Serbia. FAU - Stojanovic, Nikola AU - Stojanovic N AUID- ORCID: 0000-0002-0124-3501 AD - Medical Faculty of Nis, University of Nis, 18000 Nis, Serbia. nikola.st90@yahoo.com. FAU - Antovic, Aleksandra AU - Antovic A AD - Medical Faculty of Nis, University of Nis, 18000 Nis, Serbia. FAU - Ilic, Ratko AU - Ilic R AD - Medical Faculty of Nis, University of Nis, 18000 Nis, Serbia. LA - eng GR - 172061/Ministarstvo Prosvete, Nauke i Tehnoloskog Razvoja/ GR - 175091/Ministarstvo Prosvete, Nauke i Tehnoloskog Razvoja/ PT - Case Reports DEP - 20190823 PL - Switzerland TA - Medicina (Kaunas) JT - Medicina (Kaunas, Lithuania) JID - 9425208 RN - 59763-91-6 (Pancreatic Polypeptide) SB - IM MH - Aged MH - Humans MH - Male MH - Multiple Endocrine Neoplasia Type 1 MH - Pancreatic Neoplasms/*diagnosis/genetics/*pathology MH - Pancreatic Polypeptide/*analysis PMC - PMC6780158 OTO - NOTNLM OT - PPoma OT - neuroendocrine tumours OT - pancreatic neoplasms OT - pancreatic polypeptide COIS- The authors declare no conflict of interest. EDAT- 2019/08/28 06:00 MHDA- 2020/02/25 06:00 PMCR- 2019/08/23 CRDT- 2019/08/28 06:00 PHST- 2019/06/30 00:00 [received] PHST- 2019/08/13 00:00 [revised] PHST- 2019/08/20 00:00 [accepted] PHST- 2019/08/28 06:00 [entrez] PHST- 2019/08/28 06:00 [pubmed] PHST- 2020/02/25 06:00 [medline] PHST- 2019/08/23 00:00 [pmc-release] AID - medicina55090523 [pii] AID - medicina-55-00523 [pii] AID - 10.3390/medicina55090523 [doi] PST - epublish SO - Medicina (Kaunas). 2019 Aug 23;55(9):523. doi: 10.3390/medicina55090523.