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Gupta S, Ahuja S, Kalwaniya DS. A rare unifocal gastric gastrointestinal stromal tumor in a young NF1 patient: A case report. Int J Surg Case Rep 2025; 129:111126. [PMID: 40054407 PMCID: PMC11930091 DOI: 10.1016/j.ijscr.2025.111126] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/20/2025] [Revised: 03/01/2025] [Accepted: 03/03/2025] [Indexed: 03/26/2025] Open
Abstract
INTRODUCTION AND IMPORTANCE Gastrointestinal stromal tumors (GISTs) are the most prevalent mesenchymal neoplasms of the gastrointestinal tract, predominantly associated with mutations in the KIT and PDGFRA genes. Neurofibromatosis type 1 (NF1) increases the risk of developing GISTs, particularly in younger individuals, and often presents with atypical features. CASE PRESENTATION A 16-year-old female with a known diagnosis of NF1 presented with severe anemia, hematemesis, and melena. Physical examination revealed multiple café-au-lait spots and cutaneous nodular masses. A contrast-enhanced CT scan showed a large mass arising from the stomach's greater curvature, consistent with GIST. The patient underwent a sleeve gastrectomy, and histopathological evaluation confirmed high-grade spindle cell GIST. Immunohistochemistry was positive for CD117, CD34, and DOG-1. Postoperative PET-CT showed no residual disease. CLINICAL DISCUSSION GIST in NF1 patients typically presents with a multifocal pattern, but our patient exhibited a rare unifocal GIST in the stomach. The diagnosis was complicated by atypical presentation and required multimodal imaging and histopathological confirmation. NF1-associated GISTs generally lack KIT and PDGFRA mutations, rendering targeted therapies like imatinib ineffective, emphasizing surgical management as the primary treatment. CONCLUSION This case highlights the unique clinical and pathological characteristics of NF1-associated GISTs, stressing the need for vigilant assessment in NF1 patients with gastrointestinal symptoms.
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Affiliation(s)
- Sumedha Gupta
- Department of Obstetrics and Gynaecology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
| | - Sana Ahuja
- Department of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India.
| | - Dheer Singh Kalwaniya
- Department of Surgery, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India
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Wang SS, Ji MY, Huang X, Li YX, Yu SJ, Zhao Y, Shen L. Double-nylon purse-string suture in closing postoperative wounds following endoscopic resection of large (≥ 3 cm) gastric submucosal tumors. World J Gastroenterol 2024; 30:1143-1153. [PMID: 38577185 PMCID: PMC10989488 DOI: 10.3748/wjg.v30.i9.1143] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/01/2023] [Revised: 01/04/2024] [Accepted: 02/02/2024] [Indexed: 03/06/2024] Open
Abstract
BACKGROUND Endoscopic full-thickness resection (EFTR) of gastric submucosal tumors (SMTs) is safe and effective; however, postoperative wound management is equally important. Literature on suturing following EFTR for large (≥ 3 cm) SMTs is scarce and limited. AIM To evaluate the efficacy and clinical value of double-nylon purse-string suture in closing postoperative wounds following EFTR of large (≥ 3 cm) SMTs. METHODS We retrospectively analyzed the data of 85 patients with gastric SMTs in the fundus of the stomach or in the lesser curvature of the gastric body whose wounds were treated with double-nylon purse-string sutures after successful tumor resection at the Endoscopy Center of Renmin Hospital of Wuhan University. The operative, postoperative, and follow-up conditions of the patients were evaluated. RESULTS All tumors were completely resected using EFTR. 36 (42.35%) patients had tumors located in the fundus of the stomach, and 49 (57.65%) had tumors located in the body of the stomach. All patients underwent suturing with double-nylon sutures after EFTR without laparoscopic assistance or further surgical treatment. Postoperative fever and stomach pain were reported in 13 (15.29%) and 14 (16.47%) patients, respectively. No serious adverse events occurred during the intraoperative or postoperative periods. A postoperative review of all patients revealed no residual or recurrent lesions. CONCLUSION Double-nylon purse-string sutures can be used to successfully close wounds that cannot be completely closed with a single nylon suture, especially for large (≥ 3 cm) EFTR wounds in SMTs.
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Affiliation(s)
- Shan-Shan Wang
- Department of Gastroenterology, Renmin Hospital of Wuhan University, Wuhan 430000, Hubei province, China
| | - Meng-Yao Ji
- Department of Gastroenterology, Renmin Hospital of Wuhan University, Wuhan 430000, Hubei province, China
| | - Xu Huang
- Department of Gastroenterology, Renmin Hospital of Wuhan University, Wuhan 430000, Hubei province, China
| | - Yan-Xia Li
- Department of Gastroenterology, Renmin Hospital of Wuhan University, Wuhan 430000, Hubei province, China
| | - Shi-Jie Yu
- Department of Gastroenterology, Renmin Hospital of Wuhan University, Wuhan 430000, Hubei province, China
| | - Yu Zhao
- Department of Gastroenterology, Renmin Hospital of Wuhan University, Wuhan 430000, Hubei province, China
| | - Lei Shen
- Department of Gastroenterology, Renmin Hospital of Wuhan University, Wuhan 430000, Hubei province, China
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Li X, He J, Zhu Z, Xiao Y, Jiang Y, Cheng Q, Hu Y. The value of single-channel endoscopic traction and kiss suture technique in closing wounds caused by endoscopic resection of gastrointestinal muscularis propria tumors. Surg Endosc 2023; 37:7709-7716. [PMID: 37563343 DOI: 10.1007/s00464-023-10277-6] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/30/2023] [Accepted: 07/02/2023] [Indexed: 08/12/2023]
Abstract
BACKGROUND To investigate the value of single forceps endoscopic traction stapling suture technique (SFETSST) in closing wounds caused by endoscopic resection of gastrointestinal muscularis propria tumor (GMPT). METHODS Consecutive patients who underwent submucosal tumor excavation (ESE) and endoscopic full-thickness resection (EFR) for GMPT in the Second Affiliated Hospital of Xiamen Medical College from January 2015 to January 2022 were retrospectively collected. They were divided into the SFETSST group and the standard group (patients who receive single forceps traction-free endoscopic suture technique). The healing effects were compared between the two groups. RESULTS Seventy-seven patients were included in our study with 50 patients included in SFETSST group. The baseline characteristics had no significant difference between the two groups. The technical success rate of wound suture in SFETSST cluster was significantly upper than that within standard cluster (100% vs. 88.89%, P = 0.04). The wound suture time in SFETSST cluster was significantly lower than that within standard cluster (33.19 ± 10.64 min, P < 0.001). Moreover, the incidence rates of intra-operative and postoperative complications in SFETSST cluster were lower than standard cluster (0 vs. 7.41%, P = 0.051 and 0 vs. 11.11%, P = 0.016). Interestingly, the SFETSST cluster had lower cost of consumables (2485.40 ± 591.78 vs. 4098.52 ± 1903.06 Yuan, P = 0.01) and shorter hospital stay (4.96 ± 0.90 vs. 7.19 ± 2.45, P < 0.001) than standard cluster. CONCLUSION Our study showed that to fully closure the full-thickness defects of digestive tract, SFETSST was effective, safe, and economical, which was worth popularizing.
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Affiliation(s)
- Xiaohua Li
- Department of Gastroenterology, The Second Affiliated Hospital of Xiamen Medical College, No. 566 Jimei Shengguang Road, Xiamen, 361021, Fujian, China.
| | - Jie He
- Department of Gastroenterology, The Second Affiliated Hospital of Xiamen Medical College, No. 566 Jimei Shengguang Road, Xiamen, 361021, Fujian, China
| | - Zhongmei Zhu
- Department of Gastroenterology, The Second Affiliated Hospital of Xiamen Medical College, No. 566 Jimei Shengguang Road, Xiamen, 361021, Fujian, China
| | - Yaxuan Xiao
- Department of Gastroenterology, The Second Affiliated Hospital of Xiamen Medical College, No. 566 Jimei Shengguang Road, Xiamen, 361021, Fujian, China
| | - Yuecui Jiang
- Department of Gastroenterology, The Second Affiliated Hospital of Xiamen Medical College, No. 566 Jimei Shengguang Road, Xiamen, 361021, Fujian, China
| | - Qinghong Cheng
- Department of Gastroenterology, The Second Affiliated Hospital of Xiamen Medical College, No. 566 Jimei Shengguang Road, Xiamen, 361021, Fujian, China
| | - Youzhen Hu
- Department of Gastroenterology, The Second Affiliated Hospital of Xiamen Medical College, No. 566 Jimei Shengguang Road, Xiamen, 361021, Fujian, China
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Yao MQ, Jiang YP, Yi BH, Yang Y, Sun DZ, Fan JX. Neurofibromatosis type 1 with multiple gastrointestinal stromal tumors: A case report. World J Clin Cases 2023; 11:2336-2342. [PMID: 37122520 PMCID: PMC10131015 DOI: 10.12998/wjcc.v11.i10.2336] [Citation(s) in RCA: 5] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/21/2022] [Revised: 02/01/2023] [Accepted: 03/14/2023] [Indexed: 03/30/2023] Open
Abstract
BACKGROUND Neurofibromatosis type 1 (NF1) is characterized by café-au-lait patches on the skin and the presence of neurofibromas. Gastrointestinal stromal tumor (GIST) is the most common non-neurological tumor in NF1 patients. In NF1-associated GIST, KIT and PDGFRA mutations are frequently absent and imatinib is ineffective. Surgical resection is first-line treatment.
CASE SUMMARY A 56-year-old woman with NF1 was hospitalized because of an incidental pelvic mass. Physical examination was notable for multiple café-au-lait patches and numerous subcutaneous soft nodular masses of the skin of the head, face, trunk, and limbs. Her abdomen was soft and nontender. No masses were palpated. Digital rectal examination was unremarkable. Abdominal computed tomography was suspicious for GIST or solitary fibrous tumor. Laparoscopy was performed, which identified eight well-demarcated masses in the jejunum. All were resected and pathologically diagnosed as GISTs. The patient was discharged on day 7 after surgery without complications. No tumor recurrence was evident at the 6-mo follow-up.
CONCLUSION Laparoscopy is effective for both diagnosis and treatment of NF1-associated GIST.
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Affiliation(s)
- Min-Quan Yao
- Department of Gastrointestinal Surgery, Tongxiang First People’s Hospital, Jiaxing 314500, Zhejiang Province, China
| | - Yu-Peng Jiang
- Department of Gastrointestinal Surgery, Tongxiang First People’s Hospital, Jiaxing 314500, Zhejiang Province, China
| | - Bing-Hong Yi
- Department of Gastrointestinal Surgery, Tongxiang First People’s Hospital, Jiaxing 314500, Zhejiang Province, China
| | - Yong Yang
- Department of Gastrointestinal Surgery, Tongxiang First People’s Hospital, Jiaxing 314500, Zhejiang Province, China
| | - Da-Zhuang Sun
- Department of Gastrointestinal Surgery, Tongxiang First People’s Hospital, Jiaxing 314500, Zhejiang Province, China
| | - Jin-Xing Fan
- Endoscopy Center, Tongxiang First People’s Hospital, Jiaxing 314500, Zhejiang Province, China
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Pal P, Ramchandani M, Inavolu P, Reddy DN, Tandan M. Endoscopic Full Thickness Resection: A Systematic Review. JOURNAL OF DIGESTIVE ENDOSCOPY 2022. [DOI: 10.1055/s-0042-1755304] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023] Open
Abstract
Abstract
Background Endoscopic full thickness resection (EFTR) is an emerging therapeutic option for resecting subepithelial lesions (SELs) and epithelial neoplasms. We aimed to systematically review the techniques, applications, outcomes, and complications of EFTR.
Methods A systematic literature search was performed using PubMed. All relevant original research articles involving EFTR were included for the review along with case report/series describing novel/rare techniques from 2001 to February 2022.
Results After screening 7,739 citations, finally 141 references were included. Non-exposed EFTR has lower probability of peritoneal contamination or tumor seeding compared with exposed EFTR. Among exposed EFTR, tunneled variety is associated with lower risk of peritoneal seeding or contamination compared with non-tunneled approach. Closure techniques involve though the scope (TTS) clips, loop and clips, over the scope clips (OTSC), full thickness resection device (FTRD), and endoscopic suturing/plicating/stapling devices. The indications of EFTR range from esophagus to rectum and include SELs arising from muscularis propria (MP), non-lifting adenoma, recurrent adenoma, and even early gastric cancer (EGC) or superficial colorectal carcinoma. Other indications include difficult locations (involving appendicular orifice or diverticulum) and full thickness biopsy for motility disorders. The main limitation of FTRD is feasibility in smaller lesions (<20–25 mm), which can be circumvented by hybrid EFTR techniques. Oncologic resection with lymphadencetomy for superficial GI malignancy can be accomplished by hybrid natural orifice transluminal endoscopic surgery (NOTES) combining EFTR and NOTES. Bleeding, perforation, appendicitis, enterocolonic fistula, FTRD malfunction, peritoneal tumor seeding, and contamination are among various adverse events. Post OTSC artifacts need to be differentiated from recurrent/residual lesions to avoid re-FTRD/surgery.
Conclusion EFTR is safe and effective therapeutic option for SELs, recurrent and non-lifting adenomas, tumors in difficult locations and selected cases of superficial GI carcinoma.
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Affiliation(s)
- Partha Pal
- Department of Interventional Endoscopy, Asian Institute of Gastroenterology, Hyderabad, Telangana, India
| | - Mohan Ramchandani
- Department of Interventional Endoscopy, Asian Institute of Gastroenterology, Hyderabad, Telangana, India
| | - Pradev Inavolu
- Department of Interventional Endoscopy, Asian Institute of Gastroenterology, Hyderabad, Telangana, India
| | - Duvvuru Nageshwar Reddy
- Department of Interventional Endoscopy, Asian Institute of Gastroenterology, Hyderabad, Telangana, India
| | - Manu Tandan
- Department of Interventional Endoscopy, Asian Institute of Gastroenterology, Hyderabad, Telangana, India
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Granata A, Martino A, Ligresti D, Zito FP, Amata M, Lombardi G, Traina M. Closure techniques in exposed endoscopic full-thickness resection: Overview and future perspectives in the endoscopic suturing era. World J Gastrointest Surg 2021; 13:645-654. [PMID: 34354798 PMCID: PMC8316845 DOI: 10.4240/wjgs.v13.i7.645] [Citation(s) in RCA: 10] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/06/2021] [Revised: 03/29/2021] [Accepted: 06/16/2021] [Indexed: 02/06/2023] Open
Abstract
Exposed endoscopic full-thickness resection (EFTR) without laparoscopic assistance is a minimally invasive natural orifice transluminal endoscopic surgery technique that is emerging as a promising effective and safe alternative to surgery for the treatment of muscularis propria-originating gastric submucosal tumors. To date, various techniques have been used for the closure of the transmural post-EFTR defect, mainly consisting in clip- and endoloop-assisted closure methods. However, the recent advent of dedicated tools capable of providing full-thickness defect suture could further improve the efficacy and safety of the exposed EFTR procedure. The aim of our review was to evaluate the efficacy and safety of the different closure methods adopted in gastric-exposed EFTR without laparoscopic assistance, also considering the recent advent of flexible endoscopic suturing.
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Affiliation(s)
- Antonino Granata
- Digestive Endoscopy Service, Department of Diagnostic and Therapeutic Services, IRCCS–ISMETT, Palermo 90127, Italy
| | - Alberto Martino
- Department of Gastroenterology and Digestive Endoscopy, AORN “Antonio Cardarelli”, Napoli 80131, Italy
| | - Dario Ligresti
- Digestive Endoscopy Service, Department of Diagnostic and Therapeutic Services, IRCCS–ISMETT, Palermo 90127, Italy
| | - Francesco Paolo Zito
- Department of Gastroenterology and Digestive Endoscopy, AORN “Antonio Cardarelli”, Napoli 80131, Italy
| | - Michele Amata
- Digestive Endoscopy Service, Department of Diagnostic and Therapeutic Services, IRCCS–ISMETT, Palermo 90127, Italy
| | - Giovanni Lombardi
- Department of Gastroenterology and Digestive Endoscopy, AORN “Antonio Cardarelli”, Napoli 80131, Italy
| | - Mario Traina
- Digestive Endoscopy Service, Department of Diagnostic and Therapeutic Services, IRCCS–ISMETT, Palermo 90127, Italy
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Granata A, Martino A, Amata M, Ligresti D, Traina M. Gastrointestinal exposed endoscopic full-thickness resection in the era of endoscopic suturing: a retrospective single-center case series. Wideochir Inne Tech Maloinwazyjne 2021; 16:321-328. [PMID: 34136027 PMCID: PMC8193753 DOI: 10.5114/wiitm.2021.104496] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/29/2020] [Accepted: 10/26/2020] [Indexed: 12/24/2022] Open
Abstract
INTRODUCTION Exposed endoscopic full thickness resection (EFTR) is a minimally invasive technique that has shown promising efficacy and safety in the removal of both gastrointestinal (GI) submucosal tumors (SMTs) arising from the muscularis propria (MP) and select epithelial tumors (ETs) unsuitable for conventional resection techniques. Given the chance of realizing endosurgical full-thickness suturing, the Endoscopic Suturing System (ESS) can be used to close wall defects in this setting. However, data concerning its use in EFTR are still limited. AIM This study was conducted to evaluate the safety and efficacy of exposed EFTR with defect closure using the ESS for the removal of both GI SMTs and select ETs unsuitable for conventional resection techniques. MATERIAL AND METHODS This was a retrospective, single-center, observational cohort study of patients who underwent GI exposed EFTR. RESULTS Seven patients (M : F 6 : 1) with a mean age of 56 ±14.5 years were identified. The indications were MP-originating SMTs of the stomach (n = 2) and duodenum (n = 2), and from submucosa of the rectum (n = 1), and 2 ETs of the rectum. Exposed EFTR and defect closure were successfully performed in 6/7 patients. One case was converted to laparoscopic gastric wedge resection due to technical unfeasibility. We performed an R0 resection in all cases, with the exception of 1 case of rectal EFTR. No macroscopic recurrence was detected at 6-month endoscopic follow-up. CONCLUSIONS GI exposed EFTR with defect closure by the ESS appears to be feasible, effective, and safe in referral centers. Further studies are necessary to clarify the role of the ESS for post-EFTR wall defect closure.
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Affiliation(s)
- Antonino Granata
- Endoscopy Service, Department of Diagnostic and Therapeutic Services, IRCCS – ISMETT, Palermo, Italy
| | - Alberto Martino
- Department of Gastroenterology and Digestive Endoscopy, AORN “Antonio Cardarelli”, Napoli, Italy
| | - Michele Amata
- Endoscopy Service, Department of Diagnostic and Therapeutic Services, IRCCS – ISMETT, Palermo, Italy
| | - Dario Ligresti
- Endoscopy Service, Department of Diagnostic and Therapeutic Services, IRCCS – ISMETT, Palermo, Italy
| | - Mario Traina
- Endoscopy Service, Department of Diagnostic and Therapeutic Services, IRCCS – ISMETT, Palermo, Italy
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Granata A, Martino A, Amata M, Ligresti D, Tuzzolino F, Traina M. Efficacy and safety of gastric exposed endoscopic full-thickness resection without laparoscopic assistance: a systematic review. Endosc Int Open 2020; 8:E1173-E1182. [PMID: 32904958 PMCID: PMC7458762 DOI: 10.1055/a-1198-4357] [Citation(s) in RCA: 16] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/05/2020] [Accepted: 05/25/2020] [Indexed: 02/06/2023] Open
Abstract
Background and study aims Exposed endoscopic full-thickness resection (Eo-EFTR) without laparoscopic assistance is a minimally invasive natural orifice transluminal endoscopic surgery (NOTES) technique that has shown promising efficacy and safety in resection of gastric submucosal tumors (G-SMTs) arising from muscularis propria (MP). However, data on the efficacy and safety of gastric Eo-EFTR mostly come from relatively small retrospective studies and concern regarding its use still exists. The aim of our systematic review was to assess the efficacy and safety of gastric Eo-EFTR without laparoscopic assistance. Methods A detailed MEDLINE and EMBASE search was performed for papers published from January 1998 to November 2019 and reporting on gastric Eo-EFTR without laparoscopic assistance. The search strategy used the terms "endoscopic full thickness resection" and "gastric" or "stomach". The primary outcomes were complete resection and surgical conversion rates. The secondary outcomes were overall major adverse events, delayed bleeding, delayed perforation, peritonitis, abdominal abscess and/or abdominal infection and successful Eo-EFTR. Results Fifteen Asian studies were included in our final review, providing data on 750 Eo-EFTR-treated G-SMTs. The per-lesion rate of complete resection and surgical conversion were 98.8 %\0.8 %, respectively. The per-lesion rate of major adverse events, delayed bleeding, delayed perforation and peritonitis, abdominal abscess and/or abdominal infection was 1.6 %\0.5 %\0.1 %\0.9 %, respectively. The per-lesion rate of successful Eo-EFTR (i. e. complete tumor resection and effective endoscopic defect closure) was 98.3 %. Conclusions Eo-EFTR without laparoscopic assistance appears to be highly effective and safe NOTES for removing deep G-SMTs, particularly those arising from MP layer.
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Affiliation(s)
- Antonino Granata
- Digestive Endoscopy Service, Department of Diagnostic and Therapeutic Services, IRCCS – ISMETT Palermo, Italy
| | - Alberto Martino
- Digestive Endoscopy Service, Department of Diagnostic and Therapeutic Services, IRCCS – ISMETT Palermo, Italy
| | - Michele Amata
- Digestive Endoscopy Service, Department of Diagnostic and Therapeutic Services, IRCCS – ISMETT Palermo, Italy
| | - Dario Ligresti
- Digestive Endoscopy Service, Department of Diagnostic and Therapeutic Services, IRCCS – ISMETT Palermo, Italy
| | | | - Mario Traina
- Digestive Endoscopy Service, Department of Diagnostic and Therapeutic Services, IRCCS – ISMETT Palermo, Italy
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Chiu PWY. Future of full thickness resection – Devices, indications, robotics, what is missing. TECHNIQUES IN GASTROINTESTINAL ENDOSCOPY 2019. [DOI: 10.1016/j.tgie.2019.03.010] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
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