Case Report
Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Surg. Mar 27, 2021; 13(3): 315-322
Published online Mar 27, 2021. doi: 10.4240/wjgs.v13.i3.315
Giant hepatic extra-gastrointestinal stromal tumor treated with cytoreductive surgery and adjuvant systemic therapy: A case report and review of literature
Michel Ribeiro Fernandes, Caroline Lorenzoni Almeida Ghezzi, Tomaz JM Grezzana-Filho, Flávia Heinz Feier, Ian Leipnitz, Aljamir Duarte Chedid, Carlos Thadeu Schmidt Cerski, Marcio Fernandes Chedid, Cléber Rosito Pinto Kruel
Michel Ribeiro Fernandes, Tomaz JM Grezzana-Filho, Flávia Heinz Feier, Ian Leipnitz, Aljamir Duarte Chedid, Marcio Fernandes Chedid, Cléber Rosito Pinto Kruel, Department of Gastrointestinal Surgery and Transplantation, Hospital de Clínicas de Porto Alegre, Porto Alegre 90035-007, RS, Brazil
Caroline Lorenzoni Almeida Ghezzi, Division of Radiology, Hospital de Clínicas de Porto Alegre, Porto Alegre 90035-903, RS, Brazil
Carlos Thadeu Schmidt Cerski, Department of Pathology, Hospital de Clínicas de Porto Alegre, Porto Alegre 90035-007, RS, Brazil
Author contributions: Grezzana-Filho TJM, Feier FH and Leipnitz I were the patient’s surgeons and contributed to manuscript drafting; Fernandes MR reviewed the literature and contributed to manuscript drafting; Ghezzi CLA analyzed and interpreted the imaging findings; Chedid AD and Cerski CTS pathology report, analyzed and provide the pathology pictures, Chedid MF and Kruel CRP were responsible for the revision of the manuscript for important intellectual content; all authors issued final approval for the version to be submitted.
Supported by Fundo de Incentivo à Pesquisa (FIPE)/Hospital de Clínicas de Porto Alegre and Universidade Federal do Rio Grande do Sul.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (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/
Corresponding author: Michel Ribeiro Fernandes, MD, Doctor, Department of Gastrointestinal Surgery and Transplantation, Hospital de Clínicas de Porto Alegre, Rua Ramiro Barcelos 2350, 6th Floor, Room 600, Porto Alegre 90035-007, RS, Brazil. michelfernandes@hcpa.edu.br
Received: October 3, 2020
Peer-review started: October 3, 2020
First decision: December 4, 2020
Revised: December 17, 2020
Accepted: March 7, 2021
Article in press: March 7, 2021
Published online: March 27, 2021
Processing time: 165 Days and 18.2 Hours
Abstract
BACKGROUND

Primary extra-gastrointestinal stromal tumors (E-GIST) of the liver are rare. The clinical presentation may range from asymptomatic to bleeding or manifestations of mass effect. Oncologic surgery followed by adjuvant therapy with imatinib is the standard of care. However, under specific circumstances, a cytoreductive approach may represent a therapeutic option. We describe herein the case of an 84-year-old woman who presented with a tender, protruding epigastric mass. Abdominal computed tomography scan revealed a large, heterogeneous mass located across segments III, IV, V, and VIII of the liver. The initial approach was transarterial embolization of the tumor, which elicited no appreciable response. Considering the large size and central location of the tumor and the advanced age of the patient, non-anatomic complete resection was indicated. Due to substantial intraoperative bleeding and hemodynamic instability, only a near-complete resection could be achieved. Histopathology and immunohistochemical staining confirmed the diagnosis of primary E-GIST of the liver. Considering the risk/benefit ratio for therapeutic options, debulking surgery may represent a strategy to control pain and prolong survival.

CASE SUMMARY

Here, we present a case report of a patient diagnosed with E-GIST primary of the liver, which was indicated a cytoreductive surgery and adjuvant therapy with imatinib.

CONCLUSION

E-GIST primary of the liver is a rare conditional, the treatment is with systemic therapy and total resection surgery. However, a cytoreductive surgery will be necessary when a complete resection is no possible.

Keywords: Extra-gastrointestinal stromal tumor; Primary gastrointestinal stromal tumor of the liver; Cytoreductive surgery; Debulking surgery; Case report

Core Tip: Extra-gastrointestinal stromal tumor (E-GIST) of the liver is a rare condition, but the clinical presentation and treatment is similar to GIST of digestive tract. We present herein a case of giant hepatic E-GIST in an oldest person already reported and treated by cytoreductive surgery. This case highlights because it contributes to discussion of treatment approach, such as, the management of large hepatic masses, especially in GIST, and patient who would not tolerate major surgical resections.