TY - JOUR
T1 - Pseudomyxoma Peritonei after a Total Pancreatectomy for Intraductal Papillary Mucinous Neoplasm with Colloid Carcinoma in Lynch Syndrome
AU - Hackeng, Wenzel M.
AU - De Guerre, Livia E.V.M.
AU - Kuypers, Karel C.
AU - Snoek, Alexander M.
AU - Morsink, Folkert H.
AU - Offerhaus, G. Johan A.
AU - Brosens, Lodewijk A.A.
N1 - Publisher Copyright:
© Wolters Kluwer Health, Inc. All rights reserved.
PY - 2019/1/1
Y1 - 2019/1/1
N2 - We report a case of pseudomyxoma peritonei (PMP) arising in a 62-year-old male patient with Lynch syndrome (LS). The patient's medical history included an adenocarcinoma of the colon for which a right hemicolectomy was performed and a pancreatectomy due to an intraductal papillary mucinous neoplasm (IPMN) with invasive colloid carcinoma. It was considered that the PMP could be a metastasis of the earlier colonic or pancreatic carcinoma. The pancreatic carcinoma, colon carcinoma, and PMP tissues were examined, and immunohistochemical and molecular analyses were performed to determine the PMP origin. Histopathologic examination revealed morphological similarities with the pancreatic colloid carcinoma, and further immunohistochemical and molecular analyses, including a shared GNAS mutation, confirmed the pancreatic origin of the PMP. In conclusion, this is a unique case of a patient with LS presenting with PMP originating from an IPMN with invasive colloid carcinoma, several years after pancreatectomy. The present case has important diagnostic implications. The IPMN should be considered as a rare extracolonic manifestation of LS, and pancreatic carcinoma origin should be considered in patients presenting with PMP. This case report highlights the added value of molecular diagnostics in daily pathology practice.
AB - We report a case of pseudomyxoma peritonei (PMP) arising in a 62-year-old male patient with Lynch syndrome (LS). The patient's medical history included an adenocarcinoma of the colon for which a right hemicolectomy was performed and a pancreatectomy due to an intraductal papillary mucinous neoplasm (IPMN) with invasive colloid carcinoma. It was considered that the PMP could be a metastasis of the earlier colonic or pancreatic carcinoma. The pancreatic carcinoma, colon carcinoma, and PMP tissues were examined, and immunohistochemical and molecular analyses were performed to determine the PMP origin. Histopathologic examination revealed morphological similarities with the pancreatic colloid carcinoma, and further immunohistochemical and molecular analyses, including a shared GNAS mutation, confirmed the pancreatic origin of the PMP. In conclusion, this is a unique case of a patient with LS presenting with PMP originating from an IPMN with invasive colloid carcinoma, several years after pancreatectomy. The present case has important diagnostic implications. The IPMN should be considered as a rare extracolonic manifestation of LS, and pancreatic carcinoma origin should be considered in patients presenting with PMP. This case report highlights the added value of molecular diagnostics in daily pathology practice.
KW - colloid carcinoma
KW - IPMN
KW - Lynch syndrome
KW - pseudomyxoma peritonei
UR - http://www.scopus.com/inward/record.url?scp=85058612574&partnerID=8YFLogxK
U2 - 10.1097/MPA.0000000000001201
DO - 10.1097/MPA.0000000000001201
M3 - Article
C2 - 30531244
AN - SCOPUS:85058612574
SN - 0885-3177
VL - 48
SP - 135
EP - 138
JO - Pancreas
JF - Pancreas
IS - 1
ER -