Cancer in Patients With Familial Adenomatous Polyposis: A Nationwide Danish Cohort Study With Matched Controls.

BACKGROUND & AIMS: Familial adenomatous polyposis (FAP) is a hereditary disorder that predisposes patients to colorectal cancer (CRC). Prophylactic colectomy has greatly reduced the risk of CRC. However, new associations between FAP and the risk of other cancers have subsequently emerged. In this study, we assessed the risk of specific primary and secondary cancers among patients with FAP compared with matched controls. METHODS: All known patients with FAP up until April 2021 were identified in the nationwide Danish Polyposis Register and paired with 4 unique controls matched by birth year, sex, and postal code. The risk of overall cancers, specific cancer types, and risk of a second primary cancer was assessed and compared with controls. RESULTS: The analysis included 565 patients with FAP and 1890 controls. The overall risk of cancer was significantly higher for patients with FAP than for controls (hazard ratio [HR], 4.12; 95% confidence interval [CI], 3.28-5.17; P < .001). The increased risk was mainly due to CRC (HR, 4.61; 95% CI, 2.58-8.22; P < .001), pancreatic cancer (HR, 6.45; 95% CI, 2.02-20.64; P = .002), and duodenal/small-bowel cancer (HR, 14.49; 95% CI, 1.76-119.47; P = .013), whereas no significant difference was observed for gastric cancer (HR, 3.29; 95% CI, 0.53-20.23; P = .20). Furthermore, the risk of a second primary cancer was significantly higher for patients with FAP (HR, 1.89; 95% CI, 1.02-3.50; P = .042). Between 1980 and 2020, the risk of cancer among patients with FAP decreased by ∼50%. CONCLUSIONS: Despite an absolute reduction in the risk of developing cancer among patients with FAP, the risk remained significantly higher than for the background population due to colorectal, pancreatic, and duodenal/small-bowel cancers.

Unusual presentation of jejunal adenocarcinoma and ovarian metastasis.

It is rare to find a small bowel tumour presenting as intestinal obstruction. This type of cancer is an extremely unusual condition often misdiagnosed until late stages. We report the case of a patient with persistent vomiting secondary to an obstructing jejunal adenocarcinoma not related to intestinal bowel disease. After resection and chemotherapy treatment a huge mass was detected in the left ovary. The anatomopathological findings confirmed a metastatic cancer consequent to the jejunal adenocarcinoma previously resected. This case illustrates a successful outcome of a jejunal adenocarcinoma with very poor prognosis after a extremely unusual ovarian metastasis. It is highly important to suspect other causes than intestinal bowel disease when doing a differential diagnosis in a young patient presenting with clinical symptoms of intestinal obstruction.

Intensive endoscopic resection strategy for multiple duodenal polyposis associated with familial adenomatous polyposis.

BACKGROUND AND AIM: Multiple duodenal polyposis associated with familial adenomatous polyposis (FAP) is a high risk of duodenal cancer development. We evaluated the feasibility of intensive endoscopic resection that is a comprehensive treatment strategy combining multiple kinds of endoscopic treatments. METHODS: This is a retrospective observational study. From January 2012 to July 2022, a total of 28 consecutive patients in FAP who underwent endoscopic resection for multiple duodenal polyposis more than twice were included. Various endoscopic treatments, such as cold polypectomy (CP), endoscopic mucosal resection (EMR), underwater EMR (UEMR), endoscopic submucosal dissection (ESD), and endoscopic papillectomy (EP), were applied depending on lesions size and location. We evaluated individual information from patients' medical records, including patient characteristics, lesion characteristics, details of endoscopic treatment, pathologic findings, and Spigelman index (SI). We compared the differences in the number of treatments and observation periods with and without SI decrease. RESULTS: A total of 1040 lesions were removed by 138 sessions of endoscopic resections. The median follow-up period was 3.2 years. At the beginning of the endoscopic intervention, median SI was 9 (6-11) and the proportion of Spigelman stage (SS) IV was 61%. Repeated endoscopic treatments finally reduced SI in 26 patients (93%), and the proportion of SS IV significantly decreased to 13% with every endoscopic treatment. The mean SI change was -4.2 points per year (95% confidence interval: -0.6 to -5.9). There were no patients required surgical duodenectomy during the follow-up period. CONCLUSION: Intensive resection has a potential of downstaging duodenal lesions associated with FAP.

Duodenal angiolipoma: a rare tumor causing recurrent upper gastrointestinal bleeding.

Duodenal angiolipoma is a rare adipocytic tumor, with non-specific symptoms precluding an early diagnosis. We present a case of a 67-year-old female admitted due to upper gastrointestinal bleeding. The upper endoscopy and endoscopic ultrasound evaluation showed a subepithelial lesion in the third portion of the duodenum. Endoscopic excision was performed using a standard polypectomy technique after endoloop placement. Histopathology was compatible with duodenal angiolipoma. The authors highlight duodenal angiolipoma as a rare adipocytic tumor potentially causing gastrointestinal bleeding, which can be safely treated with endoscopic excision.

Biliary obstruction secondary to duodenal follicular lymphoma with papillary involvement.

Duodenal-type follicular lymphoma is a clinical variant of follicular lymphoma located in the intestine, presenting as multiple small polyps. The estimated frequency is 1/7000 people and extraduodenal involvement is rare. It predominantly affects middle-aged women, although some cases of young patients have been published in the literature. They are usually located in the second periampullary portion of the duodenum. It is diagnosed incidentally or after performing an imaging test due to non-specific digestive symptoms (abdominalgia, diarrhea or hyporexia). The prognosis is usually favorable without specific treatment5.

[A Case Report of SOX Therapy for an Elderly Patient with Hemorrhagic Primary Duodenal Carcinoma].

BACKGROUND: Although pancreatoduodenectomy is recommended as a radical surgery for duodenal carcinoma, it has been reported that pancreatoduodenectomy in elderly patients has a high risk of surgical complications. CASE PRESENTATION: A man in his 80's was diagnosed with advanced duodenal carcinoma, presenting with anemia(Hb 5.4 g/dL). Computed tomography scanning showed wall thickening in the descending leg of the duodenum, pancreatic invasion was suspected, and clinical diagnosis was Stage ⅡB(cT4N0M0). Although radical surgery was possible, the patient refused surgery considering the risks of surgical complications. The gastroduodenal bypass surgery was performed to control bleeding, and the patient was treated with S-1 plus oxaliplatin(SOX; S-1 100 mg/body, days 1-14; oxaliplatin 100 mg/m2, day 1 q21 days). After 6 courses of the SOX regimen, the wall thickening of duodenum disappeared, and SOX was switched to S-1 monotherapy (S-1 100 mg/body, days 1-28, q42 days)according to Grade 2 thrombocytopenia and decreased performance status. After 11 courses of S-1, upper gastrointestinal endoscopy showed that the tumor had disappeared, the biopsy of duodenum showed no evidence of malignancy, and chemotherapy was terminated. The patient has been followed up for 7 months without recurrence. CONCLUSIONS: SOX for elderly patient showed efficacy against hemorrhagic duodenal carcinoma.

Primary duodenal papilla lymphoma producing obstructive jaundice: a case report.

BACKGROUND: Obstructive jaundice caused by primary duodenal lymphoma is a rare disease. CASE PRESENTATION: We reported a 59-year-old man who underwent endoscopic ultrasonography for obstructive jaundice and found a duodenal papilla tumor. Light microscopy revealed a non-Hodgkin's lymphoma. Immunohistochemical staining showed that the tumor was aggressive B-cell lymphoma. We carried out molecular targeted therapy combined with CHOP regimen chemotherapy. CONCLUSION: Surgery plays an important role in resolving obstructive jaundice when accurate histological diagnosis cannot be made. After diagnosis, chemotherapy should play a central role in treatment.

Duodenal Ampulla Neuroendocrine Tumor with GISTs of the Proximal Jejunum: A Case Report.

Neuroendocrine tumors (NEN) are a type of heterogenous, slow-growing tumors, that only in about half of the cases can be found in the gastrointestinal tract. Half of these is in the small intestine. The ampullary NENs are rare, accounting for less than 1% of gastroenteropancreatic NENs. Gastrointestinal stromal tumors (GIST) are a more common type of tumors of the gastrointestinal tract that consist of pacemaker cells. The occurrence of both tumors simultaneously is rare, but in patients with neurofibromatosis type 1, the co-existence of NEN and GIST is more often. Here we report a case of simultaneous occurrence of a well-differentiated NEN and a GIST in a patient without neurofibromatosis. Also, we provide a short review of the current knowledge and treatment strategies regarding these tumors.

Anemia induced by hemangioma at the duodenal papilla, resected by endoscopic mucosal resection.

A 7-year-old ever healthy girl presented to our hospital with anemia for half-a-year, and her lowest hemoglobin level was 75g/L. She denied any hematemesis or melena, however her fecal occult blood test was positive. Computed tomography revealed an oval lesion in the descending duodenum.

Hemostatic powder TC-325 treatment of malignancy-related upper gastrointestinal bleeds: International registry outcomes.

BACKGROUND AND AIM: Upper gastrointestinal tumors account for 5% of upper gastrointestinal bleeds. These patients are challenging to treat due to the diffuse nature of the neoplastic bleeding lesions, high rebleeding rates, and significant transfusion requirements. TC-325 (Cook Medical, North Carolina, USA) is a hemostatic powder for gastrointestinal bleeding. The aim of this study was to examine the outcomes of upper gastrointestinal bleeds secondary to tumors treated with Hemospray therapy. METHODS: Data were prospectively collected on the use of Hemospray from 17 centers. Hemospray was used during emergency endoscopy for upper gastrointestinal bleeds secondary to tumors at the discretion of the endoscopist as a monotherapy, dual therapy with standard hemostatic techniques, or rescue therapy. RESULTS: One hundred and five patients with upper gastrointestinal bleeds secondary to tumors were recruited. The median Blatchford score at baseline was 10 (interquartile range [IQR], 7-12). The median Rockall score was 8 (IQR, 7-9). Immediate hemostasis was achieved in 102/105 (97%) patients, 15% of patients had a 30-day rebleed, 20% of patients died within 30 days (all-cause mortality). There was a significant improvement in transfusion requirements following treatment (P < 0.001) when comparing the number of units transfused 3 weeks before and after treatment. The mean reduction was one unit per patient. CONCLUSIONS: Hemospray achieved high rates of immediate hemostasis, with comparable rebleed rates following treatment of tumor-related upper gastrointestinal bleeds. Hemospray helped in improving transfusion requirements in these patients. This allows for patient stabilization and bridges towards definitive surgery or radiotherapy to treat the underlying tumor.

Clinical outcomes of tumor bleeding in duodenal gastrointestinal stromal tumors: a 20-year single-center experience.

BACKGROUND: Duodenal gastrointestinal stromal tumors (GISTs) are rare, and reports on duodenal GIST bleeding are few. We analyzed the risk factors and clinical outcomes of hemorrhagic duodenal GISTs and compared them with those of gastric GISTs. METHODS: Primary duodenal GISTs surgically diagnosed between January 1998 and December 2017 were retrospectively reviewed. Furthermore, patients with duodenal GIST were compared with those with primary gastric GIST histopathologically diagnosed between January 1998 and May 2015 using previously published data. RESULTS: Of the 170 total patients with duodenal GISTs, 48 (28.2%) exhibited tumor bleeding. Endoscopic intervention, embolization, and non-interventional conservative treatment were performed for initial hemostasis in 17, 1, and 30 patients, respectively. The 5-year survival rate was 81.9% in the bleeding group and 89.4% in the non-bleeding group (P = 0.495). Multivariate analysis showed that p53 positivity was a significant risk factor for duodenal GIST bleeding (hazard ratio [HR] 2.781, P = 0.012), and age ≥ 60 years (HR 3.163, P = 0.027), a large maximum diameter (comparing four groups: < 2, 2-5, 5-10, and ≥ 10 cm), and mitotic count ≥ 5/high-power field (HPF) (HR 3.265, P = 0.032) were risk factors for overall survival. The incidence of bleeding was significantly higher in duodenal GISTs than in gastric GISTs (28.2% vs. 6.6%, P < 0.001), and the re-bleeding rate after endoscopic hemostasis was also higher in duodenal GISTs than in gastric GISTs (41.2% vs. 13.3%, P = 0.118). CONCLUSION: In patients with duodenal GIST with old age, large tumor diameter, and mitotic count ≥ 5/HPF, a treatment plan should be established in consideration of the poor prognosis, although tumor bleeding does not adversely affect the prognosis. Duodenal GISTs have a higher incidence of tumor bleeding and re-bleeding rate after endoscopic hemostasis than gastric GISTs.

[Management of obstructive jaundice in patients with neoplasms of the major duodenal papilla]. / Taktika lecheniya mekhanicheskoi zheltukhi u patsientov s novoobrazovaniyami bol'shogo sosochka dvenadtsatiperstnoi kishki.

OBJECTIVE: To develop the indications and assess an effectiveness of treatment of patients with ampullary tumors followed by mechanical jaundice. MATERIAL AND METHODS: There were 26 patients with major duodenal papilla neoplasms for the period 2015-2020 at the Sklifosovsky Research Institute for Emergency Care. RESULTS: Twenty patients underwent transpapillary interventions: papillosphincterotomy followed by lithoextraction and bilio-duodenal stenting in 4 (15.3%) patients, bilio-duodenal stenting in 12 (46.1%) patients, nasobiliary drainage in 2 (7.6%) patients, pancreaticoduodenal stenting in 2 (7.6%) patients. Percutaneous transhepatic microcholecystostomy was performed in 6 (23.4%) patients. In all cases, laboratory values decreased in 5-7 days after drainage. Eight (30.7%) patients refused further surgical treatment and were discharged in satisfactory condition. Another 8 (44.5%) patients underwent endoscopic submucosal papillectomy. There were no postoperative complications. Patients were discharged after 5-7 days. Four (22.2%) patients underwent tumor resection via laparotomy. One of these patients required redo laparotomy in postoperative period due to acute perforated duodenal ulcer. Six (33.3%) patients underwent palliative bilio-duodenal stenting. CONCLUSION: Invasion of muscle layer or distal parts of the common bile duct, as well as abnormal vascularization are indications for open surgery or palliative endoscopic treatment. Tumor location within mucous and muscle layers without invasion of distal third of the common bile duct and no abnormal vascularization of tumor justify endoscopic papillectomy. Endoscopic approach can be considered as a final minimally invasive method with minimal risk of postoperative complications in case of benign ampullary tumor.

Survival outcomes after surgical management of sporadic or familial adenomatous polyposis associated duodenal cancer.

BACKGROUND: Duodenal cancer is the second most common cause of cancer death in familial adenomatous polyposis (FAP) patients. In this study, we compare oncologic outcomes between sporadic and FAP-associated duodenal cancer. METHODS: In this retrospective study, all patients who underwent surgeries between 2000 and 2014 for either sporadic or FAP duodenal cancer were identified. The patients were grouped based on diagnoses and perioperative and survival outcomes were compared. RESULTS: A total of 56 patients with duodenal cancer (43 sporadic, 13 FAP) who underwent surgery were identified. Pancreatoduodenectomy (PD) was the most common procedure performed. The overall median survival was 7.5 years (1 year: 92%; 5 years: 58.1%). FAP patients had earlier tumor, node, and metastasis stage, less margin involvement, less perineural, and angiolymphatic invasion but had a comparable survival to sporadic patients. The median survival for FAP duodenal cancer was 7.4 vs 9.6 years for sporadic (P = .97) with similar utilization of adjuvant chemotherapy. Although not statistically significant, PD had an improved median survival compared to segmental duodenal resection (SDR) (9.6 years for PD vs 3.6 years for SDR, P = .17). Non-periampullary location and presence of positive lymph nodes were significant predictors of mortality on multivariate analysis. CONCLUSIONS: FAP duodenal cancer has no survival advantage compared to sporadic duodenal cancer despite an improved stage of resection with extraampullary lesions having a worse survival.

Pancreaticoduodenectomy for a primary duodenal capicua transcriptional repressor (CIC) -rearranged sarcoma with severe bleeding: a case report.

BACKGROUND: Capicua transcriptional repressor (CIC) -rearranged sarcoma is characterized by small round cells, histologically similar to Ewing sarcoma. However, CIC-rearranged sarcoma has different clinical, histological, and immunohistochemical features from Ewing sarcoma. It is important to differentiate between these tumors. CASE PRESENTATION: The patient is a 44-year-old man with a duodenal tumor diagnosed in another hospital who presented with a history of melena. Laboratory studies showed anemia with a serum hemoglobin of 6.0 g/dL. He was hospitalized and gastrointestinal bleeding was controlled successfully with endoscopy. However, he suffered from appetite loss and vomiting and progression of anemia a few weeks after presentation. Upper gastrointestinal endoscopy showed a circumferential soft tumor in the second portion of the duodenum and the endoscope could not pass distally. Computed tomography scan showed a greater than 10 cm tumor in the duodenum, with compression of the inferior vena cava and infiltrating the ascending colon. A definitive pathologic diagnosis could not be established despite four biopsies from the tumor edge. Due to gastrointestinal obstruction and progression of anemia, a pylorus-preserving pancreaticoduodenectomy with partial resection of the inferior vena cava and right hemicolectomy was performed as a complete tumor resection. The tumor was diagnosed as a CIC-rearranged sarcoma, but 2 months postoperatively local recurrence and distant metastases to the liver and lung were found. The patient died 3 months after surgery. CONCLUSIONS: Although the only definitive treatment for CIC-rearranged sarcoma is surgical resection, the CIC-rearranged sarcoma is highly malignant with a poor prognosis even after radical resection. More research is needed to establish optimal treatment strategies.

Hepatic arterial system anomalies encountered during pancreaticoduodenectomy - our experience.

We present our experience of incidence and management of aberrant hepatic arterial anatomy encountered during pancreaticoduodenectomy (PD). Patients undergoing PD between December 2014 and November 2016 at the Shaukat Khanum Memorial Cancer Hospital, Lahore were included in this short report. Preoperative imaging and operative findings of these patients were reviewed to evaluate the hepatic arterial anatomy and classified according to Hiatt classification. Sixty-four PD were performed with aberrant arterial anatomy identified in 24 (37.5%) of the cases. Most common anomaly was replaced right hepatic artery (rRHA) arising from the superior mesenteric artery seen in seven (11%) of the patients. Aberrant vessels were recognised and preserved in 23 cases. In one patient, the rRHA was coursing through the pancreatic parenchyma needing resection and reconstruction with uneventful postoperative recovery. Hepatic arterial anomalies are common and it is possible to preserve these vessels with careful surgical dissection using artery first technique.

Duodenal Schwannoma as a Rare Association With Membranous Nephropathy: A Case Report.

Membranous nephropathy (MN) associated with malignancies is a well-known entity. However, its association with benign neoplasm is not broadly recognized. A 69-year-old man with recurrent nephrotic syndrome presented with pedal edema and proteinuria of 5 months' duration. Laboratory results showed hypoalbuminemia and hyperlipidemia. Proteinuria was estimated to be protein excretion of 3.5g/d. Studies were negative for viral hepatitis, syphilis, human immunodeficiency virus, autoimmune diseases, and paraproteinemia. Kidney biopsy disclosed MN with negative phospholipase A2 receptor (PLA2R) staining, favoring a secondary form of MN. Computed tomography detected a 7.6-cm duodenal schwannoma. Elective surgical resection was performed. Pathologic study showed that THSD7A (thrombospondin type 1 domain-containing 7A) was positive in both glomeruli and schwannoma. Commonly, secondary MN is related to underlying conditions, including lupus, hepatitis, and neoplasm, and can be medication induced. The risk for developing a concomitant neoplasm among patients with PLA2R-negative MN is up to 12 times higher than in the general population. Most of these neoplasms are malignancies, and the presence of autoantibodies directed at similar tissue targets is hypothesized as the potential mechanism. In our case, THSD7A may be the autoantibody that has linked the schwannoma and the development of MN. Although benign tumors rarely produce renal manifestations, effective treatment may lead to resolution of nephrotic syndrome.

Single-stage EUS-guided choledochoduodenostomy using a lumen-apposing metal stent for malignant distal biliary obstruction.

BACKGROUND AND AIMS: EUS-guided choledochoduodenostomy (EUS-CD) using a lumen-apposing metal stent (LAMS) has recently been reported as an alternative treatment approach for patients with malignant obstructive jaundice and failed ERCP. We analyzed the safety and technical and clinical efficacy of EUS-CD using LAMSs in patients with malignant obstructive jaundice. METHODS: This was a retrospective study of consecutive patients with inoperable malignant distal bile duct obstruction who underwent EUS-CD using an electrocautery-enhanced (EC)-LAMS over a 3-year period (2015-2018). The main outcome measures were technical and clinical success (defined as a decline in serum bilirubin level by 50% at 2-week follow-up). Secondary outcomes were occurrence of adverse events, procedure time, and stent patency. RESULTS: Forty-six patients (47.8% women; median age, 73.1 ± 12.6 years) underwent direct EUS-CD using the biliary EC-LAMS. The procedure was technically successful in 43 patients (93.5%). The rate of clinical success was 97.7%. Adverse events occurred in 5 (11.6%) patients and included the following: 1 fatal bleeding 17 days after stent placement, 3 episodes of stent occlusion (food impaction), and 1 spontaneous migration (all 4 requiring reintervention). The mean follow-up was 114.37 days (95% confidence interval, 73.2-155.4). CONCLUSIONS: EUS-CD using the EC-LAMS is effective. The rate of adverse events including one fatal event is not negligible and should be carefully considered before using the stent in this clinical setting. Prospective studies are required to validate our preliminary findings to fully assess the long-term efficacy and safety of the stent.

Duodenal descending part-jejunum intussusception and upper gastrointestinal bleeding caused by duodenal fibrolipoma: a case report.

BACKGROUND: Duodenal fibrolipoma and duodenum-jejunum intussusception are both rare occasions in clinical practice. The diagnosis of duodenal fibrolipoma mainly depends on endoscopy examination, supplemented by CT and MRI. As the tumor grows, some severe symptoms need surgical intervention. As the development of endoscopic techniques, the operation plan should be made individually. CASE PRESENTATION: A 47-year-old female with the complaint of upper abdominal pain and melena was reported. Abdominal examination revealed upper abdomen lightly tender and blood test showed severe anemia. Image and endoscopy examination exhibited "a giant mass" in the descending (D2) part of duodenum, dragged by the tumor into the distal intestinal canal and causing intussusception. Intermittent blood transfusion treatment, enteral and parenteral nutrition were adopted to adjust her general state. Two weeks later, the mass was resected together with the basement intestinal wall via the jejunum incision and then the intussuscepted D2 part was restored. The paraffin pathological diagnosis correlated with the preoperative judgment of fibrolipoma and the patient was discharged healthy on POD 14. CONCLUSIONS: Duodenal fibrolipoma is a rare disease, infrequently causing intussusception and severe upper GIB. Duodenoscopy and endoscopic ultrasound contribute to making an appropriate diagnosis, and for patients with severe symptoms needed surgical intervention, operation plan should be individualized depending on the size and location of the lesion.

Endoscopic balloon dilation in duodenal stenosis secondary to intestinal lymphoma in a heart transplant patient.

Intestinal lymphoma can appear as a result of a state of immunosuppression, secondary to the transplantation of solid organs. Treatment with chemotherapy can result in various complications such as intestinal stenosis due to fibrotic scar tissue, which is a complication barely described in the literature. We present the case of a 12-year-old male with a heart transplant, who was diagnosed with intestinal lymphoma. After chemotherapy treatment, he developed 1-2 daily vomiting episodes as a result of intestinal stenosis due to fibrosis without tumor infiltration. Endoscopic balloon dilation was used and the symptoms were resolved without the need for surgical treatment.