Pancreas sparing duodenectomy in the treatment of primary duodenal neoplasms and other situations with duodenal involvement

Background:There are no clearly defined indications for pancreas-preserving duodenectomy.The present study aimed to analyze postoperative morbidity and the outcomes of patients undergoing pancreaspreserving duodenectomy.Methods:Patients undergoing pancreas-preserving duodenectomy from April 2008 to May 2020 were included.We divided the series according to indication:scenario 1,primary duodenal tumors;scenario 2,tumors of another origin with duodenal involvement;and scenario 3,emergency duodenectomy.Results:We included 35 patients.Total duodenectomy was performed in 1 patient of adenomatous duodenal polyposis,limited duodenectomy in 7,and third+fourth duodenal portion resection in 27.The indications for scenario 1 were gastrointestinal stromal tumor(n=13),adenocarcinoma(n=4),neuroendocrine tumor(n=3),duodenal adenoma(n=1),and adenomatous duodenal polyposis(n=1);scenario 2:retroperitoneal desmoid tumor(n=2),recurrence of liposarcoma(n=2),retroperitoneal paraganglioma(n=1),neuroendocrine tumor in pancreatic uncinate process(n=1),and duodenal infiltration due to metastatic adenopathies of a germinal tumor with digestive hemorrhage(n=1);and scenario 3:aortoenteric fistula(n=3),duodenal trauma(n=1),erosive duodenitis(n=1),and biliopancreatic limb ischemia(n=1).Severe complications(Clavien-Dindo≥IIIb)developed in 14%(5/35),and postoperative mortality was 3%(1/35).Conclusions:Pancreas-preserving duodenectomy is useful in the management of primary duodenal tumors,and is a technical option for some tumors with duodenal infiltration or in emergency interventions.

Treatment for superficial non-ampullary duodenal epithelial tumors

Because of the low prevalence of non-ampullary duodenal epithelial tumors(NADETs),standardized clinical management of sporadic superficial NADETs,including diagnosis,treatment,and follow-up,has not yet been established.Retrospective studies have revealed certain endoscopic findings suggestive of malignancy.Duodenal adenoma with high-grade dysplasia and mucosal cancer are candidates for local resection by endoscopic or minimally invasive surgery.The use of endoscopic treatment including endoscopic mucosal resection(EMR)and endoscopic submucosal dissection(ESD),for the treatment for superficial NADETs is increasing.EMR requires multiple sessions to achieve complete remission and repetitive endoscopy is needed after resection.ESD provides an excellent complete resection rate,however it remains a challenging method,considering the high risk of intraoperative or delayed perforation.Minimally invasive surgery such as wedge resection and pancreas-sparing duodenectomy are beneficial for superficial NADETs that are technically difficult to remove by endoscopic treatment.Pancreaticoduodenectomy remains a standard surgical procedure for treatment of duodenal cancer with submucosal invasion,which presents a risk of lymph node metastasis.Endoscopic or surgical treatment outcomes of superficial NADETs without submucosal invasion are satisfactory.Establishing an endoscopic diagnostic tool to differentiate superficial NADETs between adenoma and cancer as well as between mucosal and submucosal cancer is required to select the most appropriate treatment.

Hepatopancreatoduodenectomy for metastatic duodenal gastrointestinal stromal tumor

BACKGROUND: Duodenal gastrointestinal stromal tumors, which are rare, comprise 3%-5% of all gastrointestinal stromai tumors. We present a case of a metastatic duodenal gastrointestinal stromal tumor that was successfully treated by simultaneous tight hemihepatectomy and pancreaticoduodenectomy. METHODS: A 50-year-old woman was admitted to our department for the treatment of a possible metastatic duodenal gastrointestinal stromal tumor (GIST). At laparotomy a large duodenal tumor was found displacing the head of the pancreas. A 3 cm in diameter lesion in the posterior aspect of segment Ⅷ of the liver was also noted. Simultaneous right hepatectomy and pancreaticoduodenectomy were performed. RESULTS: Histological examination revealed a high grade metastatic duodenal GIST strongly positive for c-kit, CD34, and vimentin. The patient had no additional therapy. A follow-up of 21 months showed that the patient is very well and there is no evidence of recurrent diseases. CONCLUSIONS: Malignant stromai tumors of the duodenum are rarely encountered. They are usually slow growing, and may be amenable to curative surgery, even after occurrence of metastases. Resection of localized liver metastasis is still advocated when feasible, since imatinib does not provide a complete or long-term response. Combined surgical resection is an efficacious treatment for patients with metastatic duodenal gastrointestinal stromal tumor.

Spontaneous expulsion of a duodenal lipoma after endoscopic biopsy: A case report

BACKGROUND Gastrointestinal(GI)lipomas are benign submucosal tumors of mature adipocytes that arise mainly in the colon and stomach,sometimes in the ileum and jejunum,and rarely in the duodenum.Patients with symptomatic lipomas require endoscopic or surgical treatment.Spontaneous expulsion of lipomas after biopsy is a rare condition that has limited case reports.CASE SUMMARY A 56-year-old man presented to our hospital with intermittent postprandial epigastric fullness.Esophagogastroduodenoscopy(EGD)revealed a 10-mm soft yellowish submucosal lesion with the“pillow sign,”located in the second portion of duodenum.Endoscopic ultrasonography(EUS)using a 12-MHz catheter probe showed a hyperechoic,homogenous,and round solid lesion(OLYMPUS EUS EUME2,UM-DP12-25R,12-MHz radial miniprobe,Olympus Corporation,Tokyo,Japan).Deep biopsy was performed using the bite-on-bite technique with forceps.Histological examination was compatible with submucosal lipoma.The lesion spontaneously expelled 12 d after the biopsy.Follow-up EUS performed after 2 mo confirmed this condition.CONCLUSION Deep biopsy could lead to spontaneous GI lipoma expulsion.This might be the first step in lipoma diagnosis and treatment.

Perforation associated with endoscopic submucosal dissection for duodenal neoplasm without a papillary portion

AIM To investigate predictors of perforation after endoscopic resection(ER) for duodenal neoplasms without a papillary portion.METHODS This was a single-center, retrospective, cohort study conducted between April 2003 and September 2014. A total of 54 patients(59 lesions) underwent endoscopic mucosal resection(EMR)(n = 36) and endoscopic submucosal dissection(ESD)(n = 23). Clinical features, outcomes, and predictors of perforation were investigated.RESULTS Cases of perforation occurred in eight(13%) patients(95%CI: 4.7%-22.6%). Three ESD cases required sur-gical management because they could not be repaired by clipping. Delayed perforation occurred in two ESD cases, which required surgical management, although both patients underwent prophylactic clipping. All patients with perforation who required surgery had no postoperative complications and were discharged at anaverage of 13.2 d after ER. Perforation after ER showed a significant association with a tumor size greater than20 mm(P = 0.014) and ESD(P = 0.047).CONCLUSION ESD for duodenal neoplasms exceeding 20 mm may be associated with perforation. ESD alone is not recom-mended for tumor treatment, and LECS should be con-sidered as an alternative.

Review of lymphoma in the duodenum: An update of diagnosis and management

The presentation,subtype,and macroscopic images of lymphoma vary depending on the site of the tumor within the gastrointestinal tract.We searched PubMed for publications between January 1,2012 and October 10,2022,and retrieved 130 articles relating to duodenal lymphoma.A further 22 articles were added based on the manual screening of relevant articles,yielding 152 articles for full-text review.The most predominant primary duodenal lymphoma was follicular lymphoma.In this review,we provide an update of the diagnosis and management of representative lymphoma subtypes occurring in the duodenum:Follicular lymphoma,diffuse large B-cell lymphoma,extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue,mantle cell lymphoma,and Tcell lymphomas.

Segmental duodenectomy for gastrointestinal stromal tumor of the duodenum

AIM: To evaluate the results of segmental duodenectomy (SD) and pancreaticoduodenectomy (PD) for duodenal gastrointestinal stromal tumor (GIST) and help clinicians with surgical management. METHODS: All patients who underwent surgery for non-metastatic GIST of the duodenum in a single institution since 2000 were prospectively followed up. Seven patients (median age 51 years, range: 41-73 years) were enrolled: five underwent SD and two underwent PD. RESULTS: All the patients had a complete resection (R0), with no postoperative morbidity and mortality. Among the SD group, GIST was classified as low risk in two patients, intermediate risk in two, and high risk in one, according to the Fletcher scale, (vs two high risk patients in the PD group). With a median followup of 41 (18-85) mo, disease-free survival (DFS) rateswere 100% after SD and 0% after PD (P < 0.05). The median DFS was 13 mo in the PD group. CONCLUSION: Whenever associated with clear surgical margins, SD is a reliable and curative option for most duodenal GISTs, and is compatible with longterm DFS.

Malignant gangliocytic paraganglioma of the duodenum with distant metastases and a lethal course

Gangliocytic paraganglioma (GP) is rare and has been regarded as benign in general with a good prognosis. We present a patient with duodenal GP showing a malignant and lethal clinical course. A 47-year-old male patient was found to have a duodenal tumor and enlarged regional lymph nodes. The patient initially underwent a pancreaticoduodenectomy to resect the tumor and involved lymph nodes completely. Histological and immunohistochemical analyses showed findings typical of GP. However, the distant metastatic lesions in the liver and pelvic cavity were rapidly observed after surgery. The patient underwent chemotherapy and radiotherapy, as well as a second surgery to partly remove the metastatic mass in the pelvic cavity. The histological examination revealed no significant difference in histological features between the primary duodenal tumor and the metastatic pelvic mass. However, the patient finally died of the tumor due to the recurrence of the residual pelvic lesion and increased liver mass. To our knowledge, this is the first report of lethal GP with multifocal metastases. Our case confirms that GP should be regarded as a malignant potential tumor with behavior code of &#x0201c;1&#x0201d;, rather than a benign tumor of &#x0201c;0&#x0201d;.

Wireless capsule endoscopy in detecting small-intestinal polyps in familial adenomatous polyposis

AIM:To detect the prevalence of small bowel polyps by wireless capsule endoscopy(WCE)in patients with familial adenomatous polyposis(FAP).METHODS:We examined prospectively 14 patients with FAP to assess the location,size and number of small-intestinal polyps.Patients'age,sex,years of observation after surgery,type of surgery,duodenal polyps and colorectal cancer at surgery were analyzed.RESULTS:During WCE,polyps were detected in 9/14(64.3%)patients.Duodenal adenomatous polyps were found in nine(64.3%)patients,and jejunal and ileal polyps in seven(50%)and eight(57.1%),respectively.The Spigelman stage of duodenal polyposis was associated with the presence of jejunal and ileal polyps.Identification of the ampulla of Vater was not achieved with WCE.Importantly,the findings of WCE had no immediate impact on the further clinical management of FAP patients.No procedure-related complications were observed in the patients.CONCLUSION:WCE is a promising noninvasive new method for the detection of small-intestinal polyps.Further investigation is required to determine which phenotype of FAP is needed for surveillance with WCE.

Pancreas-preserving segmental duodenectomy for gastrointestinal stromal tumor of the duodenum and splenectomy for splenic angiosarcoma

BACKGROUND: Gastrointestinal stromal tumors are the most common mesenchymal tumors of the gastrointestinal tract and occur rarely in the duodenum. Splenic angiosarcoma is an aggressive neoplasm with an extremely poor prognosis. METHODS: We report a case of a 70-year-old man hospitalized for abdominal pain in the upper quadrants, dyspepsia and nausea, previously treated for Hodgkin lymphoma 30 years ago. Abdominal CT showed a solid nodular lesion in the third portion of the duodenum, the presence of retropancreatic, aortic and caval lymph nodes, and four nodular splenic masses. 111 In-octreotide scintigraphy revealed pathological tissue accumulation in the duodenal region, and in the retropancreatic, retroduodenal, aortic and caval lymph nodes, suggesting a nonfunctioning neuroendocrine peripancreatic tumor. RESULTS: At exploratory laparotomy, an exophytic soft tumor was found originating from the third portion of the duodenum. Pancreas-preserving duodenectomy with duodenojejunostomy, splenectomy and lymphnodectomy of retropancreatic aortic and caval lymph nodes were performed. Pathological evaluation and immunohistochemical studies showed the presence of a duodenal gastrointestinal stromal tumor with low mitotic activity and a well-differentiated angiosarcoma localized to the spleen and invading lymph nodes.CONCLUSIONS: We speculated that the angiosarcoma and duodenal gastrointestinal stromal tumors of this patient were due to the treatment of Hodgkin lymphoma with radiotherapy 30 years ago. Pancreas-preserving segmental duodenectomy can be used to treat non-malignant neoplasms of the duodenum and avoid extensive surgery. Splenectomy is the treatment of choice for localized angiosarcomas but a strict follow-up is mandatory because of the possibility of recurrence.

Two synchronous somatostatinomas of the duodenum and pancreatic head in one patient

Somatostatinomas are extremely rare neuroendocrine tumors of the gastrointestinal tract,f irst described in the pancreas in 1977 and in the duodenum in 1979.They may be functional and cause somatostatinoma or inhibi-tory syndrome,but more frequently are non-functioning pancreatic endocrine tumors that produce somatostatin alone.They are usually single,malignant,large lesions,frequently associated with metastases,and generally with poor prognosis.We present the unique case of a 57-year-old woman with two synchronous non-function-ing somatostatinomas,one solid duodenal lesion and one cystic lesion within the head of the pancreas,that were successfully resected with a pylorus-preserving Whipple's procedure.No secondaries were found in the liver,or in any of the removed regional lymph nodes.The patient had an uneventful recovery,and remains well and symptom-free at 18 mo postoperatively.This is an extremely rare case of a patient with two synchro-nous somatostatinomas of the duodenum and the pancreas.The condition is discussed with reference to the literature.

Extended local resection for treatment of periampullary carcinoma of vater

BACKGROUND: The trauma caused by pancreatoduode- nectomy for periampullary carcinoma of vater is often se- vere and extensive. The purpose of this study was to eva- luate the effect of extended local resection in the treatment of periampullary carcinoma of vater. METHODS: The extra-hepaticobiliary tract, the confluence of the pancreatic and biliary duct, vater ampulla and duo- denal papilla were resected en bloc in 8 patients with peri- ampullary carcinoma from 1995 to 1998. RESULTS: One patient died perioperatively. Duodenal ob- struction developed postoperatively in one of 7 survived pa- tients and was relieved after reoperation. All the 7 patients were followed up for more than 6 months without recur- rence. CONCLUSION: Extended local resection fulfils the task of radical treatment of periampullary malignancy.

EXPRESSION OF p53 PROTEIN IN CANCERS OF SMALL INTESTINE AND ITS RELATIONSHIP TO CLINICAL COURSE AND PROGNOSIS

In order to study the relationship of p53 gene mutation with the occurrence and prognosis of cancer of small intestine, expression of p53 protein was immunohistochemically examined. The results showed that p53 protein expression was high in 75% of small intestine cancer, and positive in 21.1% of the tissues close to cancer. In 7 cases of small intestinal adenoma, only one was immunoreactive. Sixteen samples of normal tissue of the intestine didn't show expression of p53protein. The study also found that the degree of p53protein expression was significantly correlated with that of tumor cell differentiation, invasion, metastasis and prognosis.

Gastrinoma and Zollinger Ellison syndrome:A roadmap for the management between new and old therapies

Zollinger-Ellison syndrome(ZES)associated with pancreatic or duodenal gastrinoma is characterized by gastric acid hypersecretion,which typically leads to gastroesophageal reflux disease,recurrent peptic ulcers,and chronic diarrhea.As symptoms of ZES are nonspecific and overlap with other gastrointestinal disorders,the diagnosis is often delayed with an average time between the onset of symptoms and final diagnosis longer than 5 years.The critical step for the diagnosis of ZES is represented by the initial clinical suspicion.Hypergastrinemia is the hallmark of ZES;however,hypergastrinemia might recognize several causes,which should be ruled out in order to make a final diagnosis.Gastrin levels>1000 pg/mL and a gastric pH below 2 are considered to be diagnostic for gastrinoma;some specific tests,including esophageal pH-recording and secretin test,might be useful in selected cases,although they are not widely available.Endoscopic ultrasound is very useful for the diagnosis and the local staging of the primary tumor in patients with ZES,particularly in the setting of multiple endocrine neoplasia type 1.Some controversies about the management of these tumors also exist.For the localized stage,the combination of proton pump inhibitory therapy,which usually resolves symptoms,and surgery,whenever feasible,with curative intent represents the hallmark of gastrinoma treatment.The high expression of somatostatin receptors in gastrinomas makes them highly responsive to somatostatin analogs,supporting their use as anti-proliferative agents in patients not amenable to surgical cure.Other medical options for advanced disease are super-imposable to other neuroendocrine neoplasms,and studies specifically focused on gastrinomas only are scant and often limited to case reports or small retrospective series.The multidisciplinary approach remains the cornerstone for the proper management of this composite disease.Herein,we reviewed available literature about gastrinoma-associated ZES with a specific focus on differential diagnosis,providing potential diagnostic and therapeutic algorithms.

Diagnostic role of 18F-fluorodeoxyglucose positron emission tomography for follicular lymphoma with gastrointestinal involvement

AIM:To investigate the capacity for 18F-fluorodeoxyglucose(18F-FDG) positron emission tomography(PET) to evaluate patients with gastrointestinal lesions of follicular lymphoma.METHODS:This retrospective case series consisted of 41 patients with follicular lymphoma and gastrointestinal involvement who underwent 18F-FDG-PET and endoscopic evaluations at ten different institutions between November 1996 and October 2011.Data for endoscopic,radiological,and biological examinations performed were retrospectively reviewed from clinical records.A semi-quantitative analysis of 18F-FDG uptake was performed for each involved area by calculating the maximum standardized uptake value(SUVmax).Based on the positivity of 18F-FDG uptake in the gastrointestinal lesions analyzed,patients were subdivided into two groups.To identify potential predictive factors for 18F-FDG positivity,these two groups were compared with respect to gender,age at diagnosis of lymphoma,histopathological grade,pattern of follicular dendritic cells,mitotic rate,clinical stage,soluble interleukin-2 receptor levels detected by 18F-FDG-PET,lactate dehydrogenase(LDH) levels,hemoglobin levels,bone marrow involvement,detectability of gastrointestinal lesions by computed tomography(CT) scanning,and follicular lymphoma international prognostic index(FLIPI) risk.RESULTS:Involvement of follicular lymphoma in the stomach,duodenum,jejunum,ileum,cecum,colon,and rectum was identified in 1,34,6,3,2,3,and 6 patients,respectively.No patient had esophageal involvement.In total,19/41(46.3%) patients exhibited true-positive 18F-FDG uptake in the lesions present in their gastrointestinal tract.In contrast,false-negative 18F-FDG uptake was detected in 24 patients(58.5%),while false-positive 18F-FDG uptake was detected in 5 patients(12.2%).In the former case,2/19 patients had both 18F-FDG-positive lesions and 18F-FDGnegative lesions in the gastrointestinal tract.In patients with 18F-FDG avidity,the SUVmax value of the involved gastrointestinal tract ranged from 2.6 to 17.4(median:4.7).For the 18F-FDG-negative(n = 22) and-positive(n = 19) groups,there were no differences in the male to female ratios(10/12 vs 4/15,P = 0.186),patient age(63.6 ± 2.4 years vs 60.1 ± 2.6 years,P = 0.323),presence of histopathological grade 1 vs 2(20/2 and 17/2,P = 1.000),follicular dendritic cell pattern(duodenal/nodal:13/5 vs 10/3,P = 1.000),mitotic rate(low/partly high,14/1 vs 10/3,P = 0.311),clinical stage according to the Ann Arbor system(stages ⅠE and ⅡE/other,15/7 vs 15/4,P = 0.499),clinical stage according to the Lugano system(stages Ⅰ and Ⅱ-1/other,14/8 vs 14/5,P = 0.489),soluble interleukin-2 receptor levels(495 ± 78 vs 402 ± 83,P = 0.884),LDH levels(188 ± 7 vs 183 ± 8,P = 0.749),hemoglobin levels(13.5 ± 0.3 vs 12.8 ± 0.4,P = 0.197),bone marrow involvement(positive/negative,1/8 vs 1/10,P = 1.000),detectability by CT scanning(positive/negative,1/16 vs 4/13,P = 0.335),and FLIPI risk(low risk/other,16/6 vs 13/6,P = 0.763),respectively in each case.CONCLUSION:These findings indicate that it is not feasible to predict 18F-FDG-avidity.Therefore,18FFDG-PET scans represent a complementary modality for the detection of gastrointestinal involvements in follicular lymphoma patients,and surveillance of the entire gastrointestinal tract by endoscopic examinations is required.