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Case Reports
. 2019 Dec 6;7(23):4011-4019.
doi: 10.12998/wjcc.v7.i23.4011.

Robotic wedge resection of a rare gastric perivascular epithelioid cell tumor: A case report

Alessandra Marano  1 , Francesca Maione  1 , Yanghee Woo  2 , Luca Pellegrino  1 , Paolo Geretto  1 , Diego Sasia  1 , Mirella Fortunato  3 , Giulio Fraternali Orcioni  3 , Roberto Priotto  4 , Renato Fasoli  5 , Felice Borghi  6
Affiliations
Case Reports

Robotic wedge resection of a rare gastric perivascular epithelioid cell tumor: A case report

Alessandra Marano et al. World J Clin Cases. .

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

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Conflict of interest statement

Conflict-of-interest statement: The authors declare that they have no conflict of interest.

Figures

Figure 1
Figure 1
Upper endoscopy and computed tomography gastrography findings at surgery. A: Upper endoscopy showed an ulcerative lesion of the gastric fundus with spontaneous bleeding; B: Computed tomography gastrography showed a relatively well-defined mass with ulceration of the gastric fundus, 3 cm below the esophago-gastric junction, with heterogeneous enhancement, measuring approximately 60 mm.
Figure 2
Figure 2
Overhead view of the operative technique. A: operative room configuration; B: da Vinci® Si™ port layout. R: Robotic trocar; SUL: Spine-umbilical line; MCL: Midclavicular line.
Figure 3
Figure 3
Gross examination and histopathology. A: Polypoid lesion with central ulcer (arrow), located in the gastric wall protruding into mucosal and serous surfaces; B: Cut surface showing grayish-white; C: Microscopic sections (hematoxylin-eosin staining) of the gastric neoplasm showing both epithelioid and spindle cell components; D: 3.3’-diaminobenzidine (DAB) immunostaining for HMB-45 demonstrates positivity for epithelioid and spindle cells (arrows); E: The spindle and epithelioid cells are consistently immunopositive for smooth muscle actin following DAB immunostaining; F: Focal expression of MART-1 in the tumor cells (arrows) following DAB immunostaining. Magnification: C: 4 ×; D: 10 ×; E: 4 ×; F: 10 ×.
See this image and copyright information in PMC

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