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

作　　者：Alessandra Marano Francesca Maione Yanghee Woo Luca Pellegrino Paolo Geretto Diego Sasia Mirella Fortunato Giulio Fraternali Orcioni Roberto Priotto Renato Fasoli Felice Borghi 

机构地区：[1]Department of Surgery,General and Oncologic Surgery Unit,Santa Croce e Carle Hospital,Cuneo 12100,Italy [2]Department of Surgery,City of Hope,Duarte,CA 91010,United States [3]Department of Pathology,Santa Croce e Carle Hospital,Cuneo 12100,Italy [4]Department of Radiology,Santa Croce e Carle Hospital,Cuneo 12100,Italy [5]Department of Gastroenterology and Digestive Endoscopy,Santa Croce e Carle Hospital,Cuneo 12100,Italy

出　　处：《World Journal of Clinical Cases》2019年第23期4011-4019,共9页世界临床病例杂志

摘　　要：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, CD79 a, 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.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^TM 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.

关 键 词：PERIVASCULAR EPITHELIOID cell tumor STOMACH ROBOTIC Surgery MINIMALLY INVASIVE Case report 

分 类 号：R73[医药卫生—肿瘤]