Feasibility and efficacy of minimally invasive limited resection for primary duodenal gastrointestinal stromal tumors: a retrospective cohort study.

BACKGROUND: The primary duodenal gastrointestinal stromal tumor (GIST) is a rare type of gastrointestinal tract tumor. Limited resection (LR) has been increasingly performed for duodenal GIST. However, only a few studies reported minimally invasive limited resection (MI-LR) for primary duodenal GIST. METHODS: The clinical data of 33 patients with primary duodenal GIST from December 2014 to February 2024 were retrospectively analyzed including 23 who received MI-LR and 10 who received laparoscopic or robotic pancreaticoduodenectomy (LPD/RPD). RESULTS: A total of 33 patients with primary duodenal GIST were enrolled and retrospectively reviewed. Patients received MI-LR exhibited less OT (280 vs. 388.5min, P=0.004), EBL (100 vs. 450ml, P<0.001), and lower morbidity of postoperative complications (52.2% vs. 100%, P=0.013) than LPD/RPD. Patients received LPD/RPD burdened more aggressive tumors with larger size (P=0.047), higher classification (P<0.001), and more mitotic count/50 HPF(P=0.005) compared with patients received MI-LR. The oncological outcomes were similar in MI-LR group and LPD/RPD group. All the patients underwent MI-LR with no conversion, including 12 cases of LLR and 11 cases of RLR. All of the clinicopathological data of the patients were similar in both groups. The median OT was 280(210-480) min and 257(180-450) min, and the median EBL was 100(20-1000) mL and 100(20-200) mL in the LLR and the RLR group separately. The postoperative complications mainly included DGE (LLR 4 cases, 33.4% and RLR 4 cases, 36.4%), intestinal fistula (LLR 2 cases, 16.7%, and RLR 0 case), gastrointestinal hemorrhage (LLR 0 case and RLR 1 case, 9.1%), and intra-abdominal infection (LLR 3 cases, 25.0% and RLR 1 case, 9.1%). The median postoperative length of hospitalization was 19.5(7-46) days in the LLR group and 19(9-38) days in the RLR group. No anastomotic stenosis, local recurrence or distant metastasis was observed during the follow-up period in the two groups. CONCLUSIONS: Minimally invasive limited resection is an optional treatment for primary duodenal GIST with satisfactory short-term and long-term oncological outcomes.

Multiple duodenal epithelial tumors in a patient with polymerase proofreading-associated polyposis in POLE variant.

Polymerase proofreading-associated polyposis (PPAP) is a rare disease with autosomal-dominant inheritance caused by germline variants in the POLE and POLD1 genes. PPAP has been reported to increase the risk of multiple cancers, including colon, duodenal, and endometrial cancers. Herein, we report a case in which multiple duodenal tumors led to the detection of a POLE mutation. A 43-year-old woman underwent esophagogastroduodenoscopy (EGD). Multiple duodenal tumors were detected, and all lesions were treated endoscopically. The patient had a history of multiple colorectal cancers and endometrial cancer along with a family history of cancer; hence, genetic testing was performed, and POLE variant, c.1270C > G (p.Leu424Val) was detected. Hereditary colorectal cancer syndromes should be considered in patients with colorectal cancer who have multiple cancers or a family history of cancer, and multigene panel sequencing is useful in confirming the diagnosis. In addition, duodenal tumors frequently coexist in patients with PPAP-carrying POLE variants, while the endoscopic treatment for duodenal tumors becomes safe and useful with several new approaches. Therefore, surveillance EGD is necessary in such patients for the early detection and treatment of duodenal tumors.

Characteristics of synchronous and metachronous duodenal tumors and association with colorectal cancer: a supplementary analysis.

BACKGROUND: We previously reported outcomes of endoscopic resection for duodenal tumors in a large cohort. This study investigated the frequency and characteristics of synchronous and metachronous lesions, and their association with colorectal advanced adenoma (CAA) and colorectal cancer (CRC). METHODS: Patients underwent duodenal endoscopic resection during January 2008 to December 2018. Background and characteristics, incidence of synchronous and metachronous lesions, and incidence of CAA and CRC were investigated. Patients without synchronous lesions were classified as the single group, and those with synchronous lesions as the synchronous group. Patients were also classified as the metachronous and non-metachronous groups. The characteristics among the groups were compared. RESULTS: We included 2658 patients with 2881 duodenal tumors: 2472 (93.0%) patients had single, 186 (7.0%) had synchronous, and 54 (2.0%) had metachronous lesions. The 5-year cumulative incidence of metachronous lesions was 4.1%. In total, 208 (7.8%) had CAA and 127 (4.8%) patients had CRC, and colonoscopy was performed in 936 (35.2%) patients. The incidence of CAA in the synchronous groups tended to be higher compared with that in the single groups (11.8% vs 7.5%, adjusted risk ratio 1.56), and the incidence of CRC in the metachronous groups tended to be higher compared with that in the non-metachronous groups (13.0% vs 4.6%, adjusted risk ratio 2.75), but there was no difference after adjusting for colonoscopy. CONCLUSIONS: This study showed the incidence of synchronous and metachronous duodenal lesions. There was no significant difference in incidence of CAA and CRC among each group, but further studies are warranted.

Intraductal papillary mucinous neoplasm mimicking a duodenal tumor.

Intraductal papillary mucinous neoplasms of the pancreas are characterized by ductal dilatation, mucus secretion, and intraductal papillary growth. Intraductal papillary mucinous neoplasms can cause fistulation and extend to adjacent organs. However, they rarely present as large mass in the small bowel that causes bowel obstruction. Herein, we describe a case of intraductal papillary mucinous neoplasm that protruded into the duodenal lumen through the ampulla of Vater, presented as a large duodenal tumor, and developed duodenal obstruction. A 78-year-old woman was admitted to the emergency department with a 2-day history of vomiting and epigastric pain and 2 kg weight-loss in 2 months. Esophagogastroduodenoscopy showed a duodenal mass; however, the endoscope could not pass further, and visualization of the entire tumor was impossible. Computed tomography and magnetic resonance imaging revealed a heterogeneous enhancing mass measuring 8 cm in the second portion of the duodenum, which continued further as an intraductal mass of the pancreas. The main pancreatic duct was dilated, and the parenchyma was atrophied. The biopsy showed a well-differentiated adenocarcinoma and an intact overlying duodenal mucosa. Surgical option of treatment was offered to the patient; however, she refused it due to her advanced age and personal religious beliefs. Thus, we conclude that the experience and knowledge gathered through this patient regarding intraductal papillary mucinous neoplasms could provide further understanding of this disease and evolve subsequent patient care.

Novel closure method for a large mucosal defect after endoscopic resection: String clip suturing method with an anchor.

Duodenal endoscopic submucosal dissection (ESD) is a high-risk technique; however, prophylactic closure of mucosal defects reduces the risk. Unfortunately, we have encountered cases where closure is difficult, especially in large lesions. Therefore, we developed a novel closure technique, a string clip suturing method with an anchor (SCSM-A). This study aimed to elucidate the feasibility of this method. Five patients underwent this method for the closure of mucosal defects after duodenal ESD. The initial string clip was deployed at the anal end of the mucosal defects and the second clip was deployed at the other end of the mucosal defect. A third clip was deployed on the muscular layer in the middle of the mucosal defect. The free end of the string was pulled, and additional clips were deployed around the first to the third clips for complete closure. Because of grasping the muscle layer, SCSM-A can be employed for secure closure without creating a pocket. We reviewed the background and clinical course of hospitalization of patients who underwent this method. The resected specimens ranged from 52 to 103 mm in diameter. Complete closure of the mucosal defects was possible in all the cases. There were no adverse events, and no cases required additional treatment. All the patients were discharged within 7 days. The new method achieved secure closure even for large mucosal defects after duodenal ESD. This is a technique that can be applied to other organs, e.g., the colon.

Effectiveness and safety of endoscopic resection for duodenal gastrointestinal stromal tumors: A single center analysis.

BACKGROUND: Endoscopic resection for duodenal gastrointestinal stromal tumors (GISTs) is still considered a great challenge with a high risk of complications, including perforation, bleeding, tumor rupture, and residual tumor. AIM: To assess the effectiveness and safety of endoscopic resection for duodenal GISTs. METHODS: Between January 2010 and January 2022, 11 patients with duodenal GISTs were treated with endoscopic resection. Data were extracted for the incidence of complete resection, bleeding, perforation, postoperative infection, recurrence, and distant metastasis. RESULTS: The incidence of successful complete resection of duodenal GISTs was 100%. Three cases (27.3%) had suspected positive margins, and the other 8 cases (72.7%) had negative vertical and horizontal margins. Perforation occurred in all 11 patients. The success rate of perforation closure was 100%, while 1 patient (9.1%) had suspected delayed perforation. All bleeding during the procedure was managed by endoscopic methods. One case (9.1%) had delayed bleeding. Postoperative infection occurred in 6 patients (54.5%), including 1 who developed septic shock and 1 who developed a right iliac fossa abscess. All 11 patients recovered and were discharged. The mean hospital stay was 15.3 d. During the follow-up period (14-80 mo), duodenal stenosis occurred in 1 case (9.1%), and no local recurrence or distant metastasis were detected. CONCLUSION: Endoscopic resection for duodenal GISTs appears to be an effective and safe minimally invasive treatment when performed by an experienced endoscopist.

Lymph node recurrence and re-excision after primary tumor resection of a histiocytic sarcoma of duodenal origin: a case report.

BACKGROUND: Histiocytic sarcoma is a rare malignant tumor that is similar in characteristics to a mature histiocyte/macrophage and is a relatively new disease entity. In approximately one-third of cases, the site of origin is a lymph node; development from the gastrointestinal tract, spleen, soft tissue, and skin has further been reported. The tumor characteristics are not well-understood as reports on its clinical presentation and treatment are limited. We report a case of duodenal primary histiocytic sarcoma. CASE PRESENTATION: An elevated lesion in the second part of the duodenum was detected in a 70-year-old woman during routine examination using upper gastrointestinal tract endoscopy. Blood biochemistry findings were normal for tumor markers. No abnormal findings were observed in the blood count and biochemical examination. Upper gastrointestinal endoscopy revealed a 20-mm elevated lesion with a slight depression in the center, opposite to the papilla of the descending duodenum. The biopsy showed erosions of the mucosal epithelium and inflammatory cell infiltration, but no evidence of malignancy. Ultrasound-guided endoscopy revealed an ischemic tumor of submucosal origin, and bowel biopsy suggested a histiocytic sarcoma. Distant metastasis and lymph node enlargement were absent on abdominal sonography, computed tomography, and magnetic resonance imaging. Duodenal segmental resection was performed. Immunostaining of the excised lesion was positive for CD68, CD163, CD4, CD5, CD15, and CD45 and negative for CD1a, CD21, CD34, MPO, and S-100 protein. Ki-67 positivity was approximately 20%. Based on these findings, the diagnosis of histiocytic sarcoma was confirmed. Ten months after the surgery, a lymph node recurrence in the dorsum of the pancreatic uncus was observed. No evidence of recurrence was found in any other part; hence, we performed pancreaticoduodenectomy. Pathological findings of the excised lymph node confirmed the recurrence of histiocytic sarcoma in the lymph node. CONCLUSIONS: This is the first reported case of a duodenal primary histiocytic sarcoma with recurrence in the lymph node after the primary resection. The patient was treated for recurrence by lymph node excision and pancreaticoduodenectomy.

Gastroptosis due to Gastric Outlet Obstruction Secondary to Duodenal Tumor: Glenard's Disease Revisited.

Currently, gastroptosis is rarely reported, and the actual prevalence is unknown. Similarly, the possible predisposing factor and cause remain unclear. A 69-year-old had melena for 1 week, and other symptoms were left upper abdominal pain, nausea, and vomiting especially postprandially that was persistent for several months with no response to medication. The nasogastric tube produced yellowish discharge and dark-colored undigested material. The CT scan showed obstruction of the gastroduodenal junction and gastroptosis. Endoscopy revealed a gastric lumen that was extended inferiorly and an occluded pyloric ring by a bleeding mass protruding from the duodenum. We report the first case of gastroptosis caused by gastric outlet obstruction secondary to duodenal tumor.

Epithelioid angiosarcoma of the duodenum: a case report.

BACKGROUND: Angiosarcomas are rare malignant tumors that arise from the endothelium of blood vessels. They occur most commonly in the skin and soft tissue, and less commonly in the breast, liver, bone, and spleen. Gastrointestinal angiosarcomas are extremely rare. Herein, we present a case of duodenal epithelioid angiosarcoma that was treated with surgical resection. CASE PRESENTATION: A 68-year-old man presented with a 1-month history of fatigue and hypotension. He visited the outpatient clinic for a routine follow-up. Laboratory examination revealed anemia. Esophagogastroduodenoscopy revealed multiple duodenal lesions with central ulceration. A biopsy showed a sheet-like arrangement of large round and spindle-shaped tumor cells that were positive for CD31. Based on the histological and immunohistochemical staining findings, an epithelioid angiosarcoma was diagnosed. Computed tomography (CT) and positron emission tomography-CT revealed no lymph node metastasis or distant metastasis. Radical subtotal stomach-preserving pancreatoduodenectomy with lymphadenectomy was performed. After removing the specimen, reconstruction was performed using the Child procedure. Grossly, two dark-red polypoid tumors were found in the second portion of the duodenum. Histological evaluation revealed proliferation of malignant round and polygonal cells arranged in sheets and spindle-like cells arranged in bundles. Vasoformative structures were recognized as slit-like spaces containing red blood cells. Immunohistochemical staining demonstrated that the tumor cells were positive for CD31. These findings confirmed the diagnosis of epithelioid angiosarcoma in the duodenum. The patient's postoperative course was uneventful. The patient was discharged on postoperative day 19 without any complications. At a follow-up examination in the outpatient clinic at postoperative 4 months, no evidence of recurrence was detected. CONCLUSION: The present report describes a case of duodenal epithelioid angiosarcoma. Duodenal angiosarcomas may cause anemia and gastrointestinal bleeding. Because angiosarcomas sometimes show epithelioid cytomorphology, immunohistochemical analysis is useful for confirming the diagnosis.

Relationship between gene mutations and clinicopathological features in nonampullary duodenal epithelial tumors.

BACKGROUND: Molecular features of nonampullary duodenal epithelial tumors (NADETs) remain unclear. AIM: The aim of this study is to determine the association between the genetic features and clinicopathological findings of NADETs. METHODS: In total, 75 NADETs were enrolled in this study, and was performed targeted DNA sequencing of the GNAS, KRAS, TP53, and APC genes. Histological grade was classified as category 3 or category 4/5 according to the Vienna classification, and the immunophenotype was categorized as the gastric phenotype (G type), gastrointestinal phenotype (GI type), or the intestinal phenotype (I type). RESULTS: The prevalence of GNAS and KRAS mutations was significantly higher in the G type than in the GI/I type (GNAS, P = 0.027; KRAS, P = 0.005). In contrast, the frequency of TP53 mutations was significantly higher in the GI/I type than in the G type (P = 0.049). Notably, APC mutations, excluding c.4479 G>A which was synonymous mutation, were more frequently identified in category 4/5 tumors than in category 3 tumors (50% vs. 24.5%; P = 0.039). CONCLUSION: G-type NADETs harbored frequent GNAS and KRAS mutations, whereas TP53 mutations are common in NADETs with intestinal features. APC mutations were significantly associated with high-grade neoplasia and invasive carcinoma.

Cold snare polypectomy for superficial non-ampullary duodenal epithelial tumor: a prospective clinical trial (pilot study).

BACKGOUND: Cold snare polypectomy (CSP) can minimize the risk of adverse events and has become a standard treatment for small colorectal polyps. CSP might also be suitable for small superficial non-ampullary duodenal epithelial tumors (SNADETs). This study aimed to evaluate the safety of CSP for SNADETs. METHODS: The major indication criteria were as follows: (1) endoscopically diagnosed SNADET, (2) ≤ 10 mm, and (3) a single primary lesion. CSP was performed using an electrosurgical snare without electrocautery. Follow-up endoscopy and scar biopsy were performed 3 months after CSP. The primary endpoint was the delayed adverse events rate. RESULTS: In total, 21 patients were enrolled. Two and 19 lesions were located in the duodenal bulb and 2nd portion, respectively; the median lesion size was 8 mm. CSP was attempted for all lesions; three lesions could not be resected without electrocautery and were removed by conventional endoscopic mucosal resection (EMR). The rate of spurting bleeding after CSP was 0%. The median procedure time was 12 min, the median resected specimen size was 12 mm, and the rate of en bloc resection was 81% (17/21). No adverse events were observed intraoperatively, with no delayed adverse events after CSP. Histopathology revealed 15 adenomas, 4 cancers (intramucosal), and 2 non-neoplastic lesions. The horizontal margins were negative/positive/undetermined in 9, 1, and 11 cases, respectively. All vertical margins were negative. Only one recurrence was detected by follow-up endoscopy 3 months after CSP. CONCLUSIONS: CSP can be performed safely for small SNADETs. CLINICAL TRIAL REGISTRATION: This trial was registered with the University Hospital Medical Information Network Clinical Trials Registry ( http://www.umin.ac.jp/ctr/index.htm ), and the registration number is UMIN000019157.

Current Treatment Strategy for Superficial Nonampullary Duodenal Epithelial Tumors.

Endoscopic submucosal dissection (ESD) is the standard treatment method for esophageal, gastric, and colorectal cancers. However, it has not been standardized for duodenal lesions because of its high complication rates. Recently, minimally invasive and simple methods such as cold snare polypectomy and underwater endoscopic mucosal resection have been utilized more for superficial nonampullary duodenal epithelial tumors (SNADETs). Although the rate of complications associated with duodenal ESD has been gradually decreasing because of technical advancements, performing ESD for all SNADETs is unnecessary. As such, the appropriate treatment plan for SNADETs should be chosen according to the lesion type, patient condition, and endoscopist's skill.

Rare Finding of Inflammatory Fibroid Polyp of the Duodenum: A Complete Diagnostic and Pathological Workup.

Inflammatory fibroid polyps (IFP) are solitary benign tumors rarely found in the gastrointestinal (GI) tract. Additionally, duodenal polyps are diagnosed incidentally. We present a case of a 51-year-old female admitted to the department with an initial diagnosis of duodenal polyp on gastroscopy, CT, and positron-emission tomography (PET). COVID-19 pandemics was the reason for delayed treatment which allowed the lesion to progress and almost double its size in an eight-month period. We performed conventional duodenotomy and excision of the polyp. Diseases like gastrointestinal stromal tumor (GIST), inflammatory myofibroblastic tumor, and inflammatory polyp of Crohn's disease must be considered in the differential diagnosis of IFP because they could be observed in the same location.

Two case of bilateral approach in laparoscopic pancreas-sparing distal duodenectomy for duodenal neoplasms arising from the distal duodenum.

INTRODUCTION: Laparoscopic pancreas-sparing distal duodenectomy is a less invasive surgical therapy; however, the anatomical complexity of the duodenum increases the difficulty of laparoscopic procedures. We introduce our technique for laparoscopic pancreas-sparing distal duodenectomy for distal duodenal tumors. PRESENTATION OF CASES: A first patient was 47-year-old woman who had 30 mm of duodenal tumor which located in third portion of duodenum. A second patient was 66-year-old man who had 35 mm of submucosal tumor which located in the third portion of duodenum. Laparoscopic pancreas-sparing duodenectomy was performed using bilateral approach for both cases. We began by dissecting an avascular area on the right side of the transverse mesocolon to mobilize the second and third portions of the duodenum with the uncinate process of the pancreas. Next, from the left side, the jejunum and the fourth portion of the duodenum were fully mobilized orally from the surrounding tissue, connecting the dissection plane with the right-side area. The jejunum and duodenum were cut with a linear stapler. Intracorporeal reconstruction was performed in an overlapped manner. We performed this procedure in two patients. Operative time was 326 and 370 min, respectively. Patients were discharged on postoperative days 9-12 without postoperative complications. DISCUSSION: Duodenal tumors are found increasingly often because of developments in endoscopic technology and techniques; therefore, establishing safe surgical procedures for duodenal tumor excision is imperative. Our surgical approach was simple and safe procedure. CONCLUSION: Laparoscopic pancreas-sparing distal duodenectomy with a bilateral approach is a useful approach without wide mobilization of duodenum.

Endoscopic and Clinicopathological Features of Superficial Non-Ampullary Duodenal Tumor Based on the Mucin Phenotypes.

AIMS: We aimed to clarify the endoscopic/clinicopathological features of superficial non-ampullary duodenal epithelial tumors (SNADETs) based on their mucin phenotypes. METHODS: We analyzed 62 SNADET lesions and classified them based on mucin phenotypic expression. Endoscopic and clinicopathological findings were compared according to mucin phenotypes. RESULTS: Eleven lesions had the gastric phenotype (GP) and 43 lesions had the intestinal phenotype (IP). All GP lesions were located in the first portion of the duodenum, while most IP lesions (72.1%) were located in the second portion (p < 0.01). Tumor size was significantly larger in the GP than in the IP group (14.4 mm vs. 10.2 mm, p < 0.05). Reddish color (72.7% in GP vs. 37.2% in IP, p < 0.05), type 0-I (72.7% vs. 11.6%, p < 0.01), lobular/granular pattern (81.8% vs. 4.7%, p < 0.01), and category 4/5 in Vienna classification (81.8% vs. 30.2%, p < 0.01) were observed significantly more often in the GP than in the IP group. Regarding findings of magnifying endoscopy with narrow-band imaging (M-NBI), white opaque substance (22.2% in GP vs. 89.7% in IP, p < 0.01) and light blue crest (0% vs. 43.6%, p < 0.05) were significantly less frequently observed in the GP group. Oval-shaped marginal epithelium (66.7% vs. 17.9%, p < 0.01), dense pattern (55.6% vs. 2.6%, p < 0.01), and dilatation of the intervening part (100% vs. 12.8%, p < 0.01) were more frequently observed in the GP group. CONCLUSIONS: SNADETs showed distinct endoscopic/clinicopathological features according to the mucin phenotype. Tumor location, coloration, macroscopic type, and endoscopic findings including M-NBI are useful to distinguish the mucin phenotypes of SNADETs.

A Case of Duodenal Tumor Adjacent to the Diverticulum That Was Resected by the Technique of Partial Submucosal Injection Combined with Underwater Endoscopic Mucosal Resection.

A 66-year-old male was referred to our hospital for treatment of duodenal tumor. The most difficult part was that the lesion was adjacent to duodenal diverticulum. Endoscopic mucosal resection (EMR) was difficult because submucosal injection spread broadly and quickly and makes it difficult to visualize the diverticulum edge of the lesion. Simple underwater EMR (UEMR) had risk for perforation at the diverticulum part because duodenal diverticulum is spurious diverticulum that defects the proper muscle layer. Therefore, to make sufficient distance between diverticulum and the lesion, we performed partial submucosal injection into only the diverticulum side of the lesion combined with simple UEMR. The lesion was resected en bloc without any adverse events. Histopathological diagnosis was tubular adenoma with moderate atypia and surgical margin negative. Partial submucosal injection combined with simple UEMR might be useful for duodenal tumor that has any technical difficulties as in this case.

Establishment of an in-vivo porcine delayed perforation model after duodenal endoscopic submucosal dissection.

OBJECTIVES: Duodenal endoscopic submucosal dissection (ESD) for superficial non-ampullary duodenal epithelial tumors has a significant incidence rate of delayed perforation. Although several methods have been proposed to prevent delayed perforation, the most appropriate methods remain unclear. Currently, there is no appropriate animal model to validate methods for preventing duodenal delayed perforation. This study aimed to establish an in-vivo porcine delayed perforation model after duodenal submucosal dissection. METHODS: Two porcine models underwent either ESD or surgical submucosal dissection. In the surgical dissection model, an inverted duodenal mucosa was resected with electrosurgical energy. In the ESD model, a gauze was placed behind the duodenum with grasped transverse part to improve endoscopic maneuverability. The mucosal defects after dissection were treated with omental coverage without suture in both models. All models were euthanized 0-5 days after procedure. Body weight; resection size; procedure dissection time; presence of intraoperative perforation and delayed perforation; and adhesion score were assessed. RESULTS: There were no significant differences in body weight and adhesion score between the two models. Resection size was significantly larger in the surgical dissection models than in the ESD models (19 mm vs 14.3 mm, P < 0.01). Procedure time was significantly longer in the ESD models than in the surgical models (45.2 minutes vs 4.5 minutes, P < 0.01). Delayed perforation rates in the surgical dissection models and the ESD models were 0% (0/5) and 100% (5/5), respectively (P < 0.01). CONCLUSIONS: This study indicated that our in-vivo porcine duodenal ESD model is beneficial to evaluate a prevention strategy for delayed perforation.