Case Report
Copyright ©The Author(s) 2019. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Dec 6, 2019; 7(23): 4011-4019
Published online Dec 6, 2019. doi: 10.12998/wjcc.v7.i23.4011
Robotic wedge resection of a rare gastric perivascular epithelioid cell tumor: A case report
Felice Borghi, Renato Fasoli, Roberto Priotto, Giulio Fraternali Orcioni, Mirella Fortunato, Diego Sasia, Paolo Geretto, Luca Pellegrino, Yanghee Woo, Francesca Maione, Alessandra Marano
Alessandra Marano, Francesca Maione, Luca Pellegrino, Paolo Geretto, Diego Sasia, Felice Borghi, Department of Surgery, General and Oncologic Surgery Unit, Santa Croce e Carle Hospital, Cuneo 12100, Italy
Yanghee Woo, Department of Surgery, City of Hope, Duarte, CA 91010, United States
Mirella Fortunato, Giulio Fraternali Orcioni, Department of Pathology, Santa Croce e Carle Hospital, Cuneo 12100, Italy
Roberto Priotto, Department of Radiology, Santa Croce e Carle Hospital, Cuneo 12100, Italy
Renato Fasoli, Department of Gastroenterology and Digestive Endoscopy, Santa Croce e Carle Hospital, Cuneo 12100, Italy
Author contributions: Borghi F, Pellegrino L, Geretto P and Marano A performed the surgery. Fasoli R performed the endoscopic diagnosis. Fortunato M and Fraternali Orcioni G performed the pathological and immunohistochemical analyses of the tumor tissues. Priotto R analyzed and interpreted the radiological findings. Marano A and Maione F reviewed the literature and drafted the manuscript. Woo Y, Pellegrino L and Sasia D revised the manuscript. Marano A edited the video of the surgical procedure. Borghi F critically reviewed the manuscript and gave final approval for publication. All authors have read and approved the final manuscript.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images and video.
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 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/
Corresponding author: Felice Borghi, MD, Director, Surgeon, General and Oncologic Surgery Unit, Santa Croce e Carle Hospital, Via Michele Coppino 26, Cuneo 12100, Italy. borghi.f@ospedale.cuneo.it
Telephone: +39-17-1642215 Fax:+39-17-1642214
Received: August 19, 2019
Peer-review started: August 19, 2019
First decision: October 24, 2019
Revised: October 31, 2019
Accepted: November 14, 2019
Article in press: November 14, 2019
Published online: December 6, 2019
Processing time: 108 Days and 18.3 Hours
Abstract
BACKGROUND

Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm that can arise in many different organs with a broad spectrum of biological behavior, from indolent to aggressive progression. Only ten cases of gastric PEComas have been reported in the English literature, which were treated with endoscopic, laparoscopic, or open resections. Due to its rarity, the optimal surgical management and prognosis of this tumor are still uncertain.

CASE SUMMARY

We present a case of robotic wedge resection of a 6.5 cm bleeding lesion of the gastric fundus located 3 cm below the esophago-gastric junction in a 55-year-old man. Biopsy revealed a malignant tumor with epithelioid cells focally positive for muscle markers desmin and smooth muscle actin. In addition, histology revealed that the tumor was positive for HMB-45, melan-A (MART-1), microphthalmia transcription factor and negative for pan-cytokeratin AE1/AE3, CD34, p40, DOG-1, CD117 (c-kit), S100, CD3, CD79a, caldesmon and myogenin. These markers suggested the possibility of a PEComa. The patient underwent a diagnostic laparoscopy via the da Vinci® Si™ system and robotic wedge resection. Final pathology confirmed a malignant gastric PEComa with negative margins. At his 11-mo follow-up visit, the patient remained disease-free.

CONCLUSION

Gastric PEComa can be treated with a robotic R0 resection with acceptable postoperative and short-term oncological outcomes.

Keywords: Perivascular epithelioid cell tumor; Stomach; Robotic; Surgery; Minimally invasive; Case report

Core tip: Robot-assisted surgical resection can be considered to facilitate the surgical treatment of a gastric perivascular epithelioid cell tumor (PEComa). PEComa should be included in the differential diagnosis of gastric tumors with unclear pathology. Due to its rarity, optimum management of the primary and metastatic disease is still unknown. To date, only ten cases of gastric PEComas treated with open or laparoscopic surgery are described in the literature. We report the first malignant PEComa of the fundus treated with a robotic R0 resection. The robotic approach was safe, feasible and provided acceptable short-term oncological outcomes.