Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2018 Sep 21:3:71.
doi: 10.21037/tgh.2018.09.04. eCollection 2018.

Surgical treatment of gastrointestinal stromal tumors of the duodenum: a literature review

Georgi Popivanov  1 , Mihail Tabakov  2 , George Mantese  3 , Roberto Cirocchi  3 , Irene Piccinini  3 , Vito D'Andrea  4 , Piero Covarelli  3 , Carlo Boselli  3 , Francesco Barberini  3 , Renata Tabola  5 , Ursi Pietro  4 , Davide Cavaliere  6
Affiliations

Surgical treatment of gastrointestinal stromal tumors of the duodenum: a literature review

Georgi Popivanov et al. Transl Gastroenterol Hepatol. .

Abstract

Background: Gastrointestinal stromal tumors (GIST) are the most frequent mesenchymal tumours in the digestive tract. The duodenal GIST (dGIST) is the rarest subtype, representing only 4-5% of all GIST, but up to 21% of the resected ones. The diagnostic and therapeutic management of dGIST may be difficult due to the rarity of this tumor, its anatomical location, and the clinical behavior that often mimic a variety of conditions; moreover, there is lack of consent for their treatment. This study has evaluated the scientific literature to provide consensus on the diagnosis of dGIST and to outline possible options for surgical treatment.

Methods: An extensive research has been carried out on the electronic databases MEDLINE, Scopus, EMBASE and Cochrane to identify all clinical trials that report an event or case series of dGIST.

Results: Eighty-six studies that met the inclusion criteria were identified with five hundred forty-nine patients with dGIST: twenty-seven patients were treated with pancreatoduodenectomy and ninety-six with only local resection (segmental/wedge resections); in four hundred twenty-six patients it is not possible identify the type of treatment performed (pancreatoduodenectomy or segmental/wedge resections).

Conclusions: dGISTs are a very rare subset of GISTs. They may be asymptomatic or may involve symptoms of upper GI bleeding and abdominal pain at presentation. Because of the misleading clinical presentation the differential diagnosis may be difficult. Tumours smaller than 2 cm have a low biological aggressiveness and can be followed annually by endoscopic ultrasound. The biggest ones should undergo radical surgical resection (R0). In dGIST there is no uniformly adopted surgical strategy because of the low incidence, lack of experience, and the complex anatomy of the duodenum. Therefore, individually tailored surgical approach is recommended. R0 resection with 1-2 cm clear margin is required. Lymph node dissection is not recommended due to the low incidence of lymphatic metastases. Tumor rupture should be avoided.

Keywords: Duodenal gastrointestinal stromal tumor (dGIST); surgery.

PubMed Disclaimer

Conflict of interest statement

Conflicts of Interest: The authors have no conflicts of interest to declare.

Figures

Figure 1
Figure 1
Study selection process.
See this image and copyright information in PMC

References

    1. Yang WL, Yu JR, Wu YJ, et al. Duodenal gastrointestinal stromal tumor: clinical, pathologic, immunohistochemical characteristics, and surgical prognosis. J Surg Oncol 2009;100:606-10. 10.1002/jso.21378 - DOI - PubMed
    1. Cirocchi R, Patelli S, Castellani E, et al. Right hemicolectomy plus pancreaticoduodenectomy vs partial duodenectomy in treatment of locally advanced right colon cancer invading pancreas and/or only duodenum. Surg Oncol 2014;23:92-8. 10.1016/j.suronc.2014.03.003 - DOI - PubMed
    1. Hirota S, Isozaki K, Moriyama Y, et al. Gain-of-function mutations of c-kit in human gastrointestinal stromal tumors. Science 1998;279:577-80. 10.1126/science.279.5350.577 - DOI - PubMed
    1. Biasco G, Velo D, Angriman I, et al. Gastrointestinal stromal tumors: report of an audit and review of the literature. Eur J Cancer Prev 2009;18:106-16. 10.1097/CEJ.0b013e32830c8da8 - DOI - PubMed
    1. Miettinen M, Lasota J. Gastrointestinal stromal tumors (GISTs): definition, occurrence, pathology, differential diagnosis and molecular genetics. Pol J Pathol 2003;54:3-24. - PubMed