Page 136 - Read Online

Ambe et al. Mini-invasive Surg 2018;2:37 Mini-invasive Surgery
DOI: 10.20517/2574-1225.2018.45

Review Open Access

Surgical management of hereditary colorectal
cancer

Peter C. Ambe , Gabriela Möslein 2,3
1,3
1 Department for Visceral, Minimally Invasive and Oncologic Surgery, Marien Hospital Düsseldorf, Düsseldorf 40479,
Germany.
2 Center for Hereditary Gastrointestinal Tumors, Helios University Hospital Wuppertal, Wuppertal 42283, Germany.
3 Chair of Surgery, Witten/Herdecke University, Witten 58448, Germany.
Correspondence to: Dr. Peter C. Ambe, Department for Visceral, Minimally Invasive and Oncologic Surgery, Marien Hospital
Düsseldorf, Düsseldorf 40479, Germany. E-mail: Peter.ambe@uni-wh.de; Peter.ambe@vkkd-kliniken.de
How to cite this article: Ambe PC, Möslein G. Surgical management of hereditary colorectal cancer. Mini-invasive Surg
2018;2:37. http://dx.doi.org/10.20517/2574-1225.2018.45
Received: 22 Jun 2018 First Decision: 25 Jun 2018 Revised: 25 Sep 2018 Accepted: 9 Oct 2018 Published: 28 Oct 2018

Science Editor: Gordon N. Buchanan Copy Editor: Cui Yu Production Editor: Huan-Liang Wu

Abstract
Colorectal cancer (CRC) is one of the most common solid malignancies worldwide. Although sporadic CRC represents
the most common form, genetic alterations is increasingly being identified in a relevant portion of patients with CRC.
Familial CRC describes an increased incidence of adenomatous polyps and CRC in first - degree relatives. Hereditary CRC
is defined by the identification of deleterious mutations in known predisposing genes. Typical hereditary syndromes with
predisposition to CRC include: hereditary non-polyposis colon cancer or Lynch syndrome, familial adenomatous polyposis,
attenuated familial adenomatous polyposis, Peutz-Jeghers syndrome and MUTYH associated polyposis. Newly identified
genetic alterations with increased risk for CRC include: PPAP, NAD, MSH3 and NTHL1. The diagnosis, surveillance and
optimal surgical management of patients with hereditary predisposition to CRC warrant a good understanding of the
genetic syndrome in question. Prophylactic surgery must be segregated from symptom-related procedures depending on
the syndrome in question. The need for extended surgical procedures must be made in an individualized manner based on
gene and gender. The patient should play an active role in the surgical decision-making. Minimally invasive access should
be the preferred approach and postoperative quality of life must be seen as a primary outcome measure.

Keywords: Hereditary colorectal cancer, hereditary non-polyposis colon cancer, Lynch Syndrome, familial adenomatous
polyposis, MUTYH associated polyposis, polyposis, proctocolectomy, virtual ileostomy

INTRODUCTION
[1]
Colorectal cancer (CRC) represents one of the most common solid malignancies worldwide . Based on

© The Author(s) 2018. Open Access This article is licensed under a Creative Commons Attribution 4.0
International License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use,
sharing, adaptation, distribution and reproduction in any medium or format, for any purpose, even commercially, as long
as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license,
and indicate if changes were made.

www.misjournal.net