Martínez de Juan F, Reolid Escribano M, Martínez Lapiedra C, Maia de Alcantara F, Caballero Soto M, Calatrava Fons A, Machado I. Pancreatic adenosquamous carcinoma and intraductal papillary mucinous neoplasm in a CDKN2A germline mutation carrier. World J Gastrointest Oncol 2017; 9(9): 390-396 [PMID: 28979722 DOI: 10.4251/wjgo.v9.i9.390]
Corresponding Author of This Article
Fernando Martínez de Juan, MD, Gastroenterology and Digestive Endoscopy Department, Valancian Institute of Oncology, Calle Profesor Beltrán Báguena, 8. 46009 Valencia, Spain. fmartinezj@fivo.org
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Gastrointest Oncol. Sep 15, 2017; 9(9): 390-396 Published online Sep 15, 2017. doi: 10.4251/wjgo.v9.i9.390
Pancreatic adenosquamous carcinoma and intraductal papillary mucinous neoplasm in a CDKN2A germline mutation carrier
Fernando Martínez de Juan, María Reolid Escribano, Carmen Martínez Lapiedra, Fernanda Maia de Alcantara, María Caballero Soto, Ana Calatrava Fons, Isidro Machado
Fernando Martínez de Juan, María Reolid Escribano, Carmen Martínez Lapiedra, Fernanda Maia de Alcantara, Gastroenterology and Digestive Endoscopy Department, Valencian Institute of Oncology, 46009 Valencia, Spain
María Caballero Soto, Digestive Surgery Department, Valencian Institute of Oncology, 46009 Valencia, Spain
Ana Calatrava Fons, Isidro Machado, Pathology Department, Valencian Institute of Oncology, 46009 Valencia, Spain
Author contributions: Martínez de Juan F and Machado I wrote the paper and contributed equally to this work; Reolid Escribano M, Martínez Lapiedra C and Maia de Alcantara F collected the clinical data and endoscopy ultrasound images and made a critical revision of the paper; Calatrava Fons A collected the cytology images and made a critical revision of the paper; Caballero Soto M collected the data referred to the surgical details and made a critical revision of the paper.
Institutional review board statement: The study was reviewed and approved by the Clinical Investigation Ethical Board.
Informed consent statement: All study participants, or their legal guardian provided informed written consent prior to study enrollment.
Conflict-of-interest statement: The authors declare non conflict of interest.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Fernando Martínez de Juan, MD, Gastroenterology and Digestive Endoscopy Department, Valancian Institute of Oncology, Calle Profesor Beltrán Báguena, 8. 46009 Valencia, Spain. fmartinezj@fivo.org
Telephone: +34-96-1114005 Fax: +34-96-1114341
Received: December 12, 2016 Peer-review started: December 14, 2016 First decision: March 22, 201 Revised: April 20, 2017 Accepted: July 14, 2017 Article in press: July 17, 2017 Published online: September 15, 2017
Abstract
A 69-year-old woman from a kindred with familial atypical multiple mole melanoma and carrier of a germline mutation in CDKN2A, presented with abdominal pain caused by a solid-cystic pancreatic mass. The patient had an abdominal computed tomography three years before in which there was no evidence of pancreatic lesion. The endoscopic ultrasound guided fine needle aspiration showed adenocarcinoma with squamous component. After surgical resection the final diagnosis was adenosquamous pancreatic carcinoma (ASPC) arising in an intraductal papillar mucinous neoplasm (IPMN). Adenosquamous carcinomas are uncommon in the pancreas and have rarely been described in association with IPMNs. It has worse prognosis than the ordinary pancreatic ductal adenocarcinoma and some distinct features. We review the clinical, imaging, pathologic and molecular aspects of ASPC. Differential diagnosis with contamination, squamous metaplasia and pancreatic metastases from a distant squamous carcinoma is discussed. Besides, the case is an accelerated model of the adenoma (IPMN)-carcinoma sequence probably due to the CDKN2A germline mutation. Somatic CDKN2A mutations are common events in the early steps of sporadic pancreatic cancer, but germline mutation carriers have a significantly higher risk of pancreatic carcinoma.
Core tip: We present a rare case of adenosquamous pancreatic carcinoma (ASPC) arising in an intraductal papillar mucinous neoplasm in a CDKN2A germline mutation carrier from a kindred with familial atypical multiple mole melanoma. The case is an accelerated model of the adenoma-carcinoma sequence in pancreatic carcinogenesis. We discuss the differential diagnoses of squamous lesions in the pancreas and review the clinical and morphological features of ASPC.
