Published online Dec 6, 2019. doi: 10.12998/wjcc.v7.i23.4011
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
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.
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.
Gastric PEComa can be treated with a robotic R0 resection with acceptable postoperative and short-term oncological outcomes.
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.