Poorly cohesive duodenal carcinoma mixed with signet ring cell carcinoma with systemic metastasis: a case report and literature review.

Poorly cohesive duodenal carcinoma mixed with signet ring cell carcinoma is very rare, and no cases have been reported. When distant metastasis occurs, it is very easy to be misdiagnosed. We report the first case of a 52-year-old man with poorly cohesive carcinoma of the duodenum mixed with signet ring cell carcinoma with systemic metastasis. The process of its diagnosis and differential diagnosis is highlighted.

Indocyanine green-fluorescent imaging for a detection of accessory pancreatic duct in pancreas-preserving duodenectomy.

Pancreas-preserving duodenectomy is indicated for select patients with a duodenal tumor in the second portion. In this procedure, identification and closure of the accessory pancreatic duct is important to prevent postoperative pancreatic fistula. A 63-y-old man was diagnosed with duodenal mucosal carcinoma in the second portion, with invasion of the major ampullary. We performed pancreas-preserving duodenectomy. Intraoperatively, indocyanine green-fluorescent imaging identified the accessory pancreatic duct clearly and it was successfully closed. Postoperative pancreatic fistula did not occur. Indocyanine green-fluorescent imaging is effective in identifying the accessory pancreatic duct in pancreas-preserving duodenectomy.

Primary duodenal carcinoma suspected to arise from ectopic gastric mucosa: a case report.

BACKGROUND: Ectopic gastric mucosa mainly occurs in the duodenal bulb, and its etiology is thought to be congenital straying of gastric tissues. Primary duodenal carcinoma is a rare disease; however, reports of carcinoma arising from ectopic gastric mucosa are extremely rare. We report a case of primary duodenal carcinoma suspected to arise from ectopic gastric mucosa, which discovered as a result of duodenal stenosis. CASE PRESENTATION: The patient was a 71-year-old man with persistent weight loss and white stools. Enhanced computed tomography showed stenosis of the third portion of the duodenum and main pancreatic duct dilatation. Upper gastrointestinal endoscopy revealed irregularity of the duodenal mucosa from the anorectal side of the papilla of Vater to the stenosis of the third portion. No malignant cells were found by biopsies from the duodenal mucosa. Endoscopic ultrasonography did not detect the tumor in the pancreatic head. The possibility of a pancreatic tumor could not be ruled out based on findings of main pancreatic duct dilatation in the pancreatic head, and the patient had long-term poor oral intake because of duodenal stenosis; thus, surgical treatment was planned. Intraoperative findings showed palpable induration of the third portion of the duodenum and white nodules on the serosal surface. This was diagnosed as primary duodenal carcinoma, and pylorus-preserving pancreatoduodenectomy was performed. Histopathological diagnosis revealed ectopic gastric mucosa in the papilla of Vater and well-differentiated tubular adenocarcinoma invaded the normal duodenal submucosa and extended to the duodenal serosa. No mass lesion was detected in the pancreas, and an intraductal papillary mucinous neoplasm was observed in the branch pancreatic duct. The main pancreatic duct stricture was caused by the duodenal carcinoma invasion. CONCLUSIONS: This case of primary duodenal carcinoma was suspected to arise from ectopic gastric mucosa and review the relevant literature.

Undifferentiated Carcinoma With Osteoclast-Like Giant Cells: A Rare Cause of Upper Gastrointestinal Bleeding.

Undifferentiated osteoclast-like giant cell carcinomas (UOLGCCs) of the digestive tract are very rare, with only a few cases reported in the literature. An 82-year-old man was referred to the emergency department for melena. Endoscopic examination revealed a hemicircumferential ulcerovegetative lesion, involving the bulbar apex and extending to the second portion of the duodenum; biopsies revealed an UOLGCC. The patient underwent transfusion support therapy, and he was proposed for best supportive care. Duodenal UOLGCC is an extremely rare cause of upper gastrointestinal bleeding. Clinical findings and therapeutic approach represent a challenge in this pathology.

Gastric-type duodenal neoplasms with rapid growth: A report of two cases.

While duodenal neoplasms of the gastric phenotype are uncommon and their natural history is unknown, gastric neoplasms of gastric phenotype reportedly grow rapidly and can invade the submucosa. Several studies suggest that duodenal neoplasms of gastric phenotype might have a high risk of deep invasion and lymph node metastasis. Duodenal neoplasms of gastric phenotype might also have a high biological malignancy and likely require early treatment if detected. Here, we report two cases of intramucosal duodenal carcinoma with a gastric phenotype that grew rapidly but was successfully resected endoscopically.

Case Report: Duodenal Carcinoma in a 40-Year-Old Asian Man With Cowden Syndrome.

Introduction: Cowden syndrome is a rare autosomal dominant genetic disease associated with PTEN mutation and is mainly shown as systemic multisystem lesions. The incidence of adenocarcinoma of the duodenum with Cowden syndrome in Asian males is rare. We hereby describe the diagnosis, treatment, and prognosis of a patient with duodenal carcinoma and Cowden syndrome. Case Description: A 40-year-old Chinese man was hospitalized because of gastrointestinal hemorrhage and anemia due to infiltrating adenocarcinoma of the descending part of the duodenum. He also had typical signs of Cowden syndrome, such as multiple polyps of the gastrointestinal tract, macrocephaly, papilloma of the tongue, soles hyperkeratosis, and melanosis spots. After the pancreaticoduodenectomy (classic Whipple), the lesions revealed the presence of hamartomatoid polyps, and some of them mutated into non-mucinous adenocarcinoma (80%) and mucinous adenocarcinoma (20%). Further investigation showed a lack of PTEN protein expression in the duodenal neoplasm, and genetic analysis showed the mutation of p.E242fs in PTEN. The patient was followed up for 1 year. There was no appearance of recurrence or distant metastasis. Conclusion: It is suggested that we should pay more attention to the differential diagnosis of duodenal carcinoma combined with gastrointestinal polyps. If multiple gastrointestinal polyps with gastrointestinal bleeding are encountered, Cowden syndrome should be considered, and timely diagnosis and treatment should be implemented.

Segmental resection with partial mesopancreatic and mesojejunal excision (pMME) for duodenal carcinoma of the third or fourth portion.

INTRODUCTION: Pancreatoduodenectomy is the standard procedure for duodenal carcinoma of the third or fourth portion. As an alternative option, we developed a novel segmental resection (SR) with partial mesopancreatic and mesojejunal excision (pMME) that enhances radicality. In this report, the surgical technique with video and outcomes are described. METHOD: We performed SR with pMME on seven consecutive patients with third or fourth duodenal carcinoma between 2009 and 2021. We divided the procedure into four sections, including (1) wide Kocher's maneuver, (2) supracolic anterior artery-first approach, (3) dissection of the mesopancreas and mesojejunum, and (4) devascularization of the uncinate process and dissection of duodenum. RESULT: Median operative time was 348 min (range, 222-391 min), and median blood loss was 100 mL (range, 30-580 mL). Major complications of Clavien-Dindo classification grade 3a or more occurred in one patient. All patients achieved R0 resections with 10 mm or more proximal margin. Six cases (85%) were alive without recurrence. CONCLUSION: We developed a radical and safe procedure of SR with pMME as an alternative and less invasive approach for duodenal carcinoma of the third or fourth portion.

Tuberous sclerosis complex: a complex case.

Tuberous sclerosis complex (TSC) is an inheritable disorder characterized by the formation of benign yet disorganized tumors in multiple organ systems. Germline mutations in the TSC1 (hamartin) or more frequently TSC2 (tuberin) genes are causative for TSC. The malignant manifestations of TSC, pulmonary lymphangioleiomyomatosis (LAM) and renal angiomyolipoma (AML), may also occur as independent sporadic perivascular epithelial cell tumor (PEComa) characterized by somatic TSC2 mutations. Thus, discerning TSC from the copresentation of sporadic LAM and sporadic AML may be obscured in TSC patients lacking additional features. In this report, we present a case study on a single patient initially reported to have sporadic LAM and a mucinous duodenal adenocarcinoma deficient in DNA mismatch repair proteins. Moreover, the patient had a history of Wilms' tumor, which was reclassified as AML following the LAM diagnosis. Therefore, we investigated the origins and relatedness of these tumors. Using germline whole-genome sequencing, we identified a premature truncation in one of the patient's TSC2 alleles. Using immunohistochemistry, loss of tuberin expression was observed in AML and LAM tissue. However, no evidence of a somatic loss of heterozygosity or DNA methylation epimutations was observed at the TSC2 locus, suggesting alternate mechanisms may contribute to loss of the tumor suppressor protein. In the mucinous duodenal adenocarcinoma, no causative mutations were found in the DNA mismatch repair genes MLH1, MSH2, MSH6, or PMS2 Rather, clonal deconvolution analyses were used to identify mutations contributing to pathogenesis. This report highlights both the utility of using multiple sequencing techniques and the complexity of interpreting the data in a clinical context.

Node negative duodenal adenocarcinoma is associated with long-term patient survival following pancreaticoduodenal resection.

BACKGROUND: Duodenal adenocarcinoma (DA) is a rare gastrointestinal malignancy. There is limited data reporting patient outcomes following radical pancreatic resection for DA. We assessed the disease-free (DFS) and overall survival (OS) in patients undergoing pancreaticoduodenectomy for DA in our institution. METHODS: We retrospectively analysed all patients undergoing pancreatic resection of DA at our institution between January 2009 and March 2020 inclusive. RESULTS: Ninteen patients underwent pancreatic resection at our institution for DA during the study period. The overall postoperative morbidity and mortality was 37% and 5%, respectively. Nine patients (47%) had no nodal involvement. Median follow up was 25 months (range 1-108 months). Median DFS for our whole cohort was 17 months but was significantly higher in patients with no nodal metastasis [p < 0.001]. Median OS was 9.5 months for the whole cohort but was significantly higher in the patients with no nodal metastasis (60 months) compared to those with nodal metastasis (17.5 months) [p < 0.003]. CONCLUSION: Improved DFS and OS for patients undergoing pancreaticoduodenectomy for DAs is associated with lymph node negative disease.

Development and validation of a nomogram for primary duodenal carcinoma: a multicenter, population-based study.

Aim: This study aimed to develop a predictive model for patients with duodenal carcinoma. Methods: Duodenal carcinoma patients from the Surveillance, Epidemiology, and End Results database (2010-2015) and the First Affiliated Hospital of Nanchang University (2010-2021) were enrolled. A nomogram was constructed according to least absolute shrinkage and selection operator regression analysis, the Akaike information criterion approach and Cox regression analysis. Results: Five independent prognostic factors were significantly associated with the prognosis of the duodenal carcinoma patients. A nomogram was constructed with a C-index in the training and validation cohorts of 0.671 (95% CI: 0.578-0.716) and 0.662 (95% CI: 0.529-0.773), respectively. Conclusion: The established nomogram model provided visualization of the risk of each prognostic factor.

Successful pancreatoduodenectomy of de novo duodenal malignancy after orthotopic liver transplantation: A case report.

Introduction: Liver transplantation is a risk factor for premalignant and malignant changes of the duodenum. De novo duodenal malignancy is seldom reported after liver transplantation. Case Report: The present study reports a case of an asymptomatic 67-year-old male patient who underwent liver transplantation more than 10 years ago and subsequently developed duodenal malignancy. Endoscopic biopsy of the de novo duodenal malignancy indicated duodenal carcinoma and pancreatoduodenectomy (PD) was performed. The patient was successfully discharged 12 days after the surgery. A metastatic lesion occurred at the right seventh rib 14 months after the pancreatoduodenectomy. Postoperative pathological examination indicated hepatocellular carcinoma metastasis. Conclusions: To the best of our knowledge, this case type has not been previously reported. The present study sheds light on the development, the treatment, the prognosis, and the management of a new type of de novo duodenal malignancy.

Surgical treatment of duodenal adenocarcinoma: ampullary vs. non-ampullary, short- and long-term outcomes.

BACKGROUND: The aim of this study was to evaluate symptoms, diagnostic methods, short- and long-term outcomes of surgical treatment in patients with duodenal adenocarcinoma. PATIENTS AND METHODS: A single center, retrospective, observational study of 52 consecutive patients with duodenal adenocarcinoma operated on with curative intent between 2006 - 2019. Duodenectomy as part of a hemipancreatoduodenectomy or total pancreatectomy procedure was performed for ADAC (ampullary duodenal/intestinal adenocarcinoma) or NADAC (non-ampullary duodenal adenocarcinoma). RESULTS: Prevailing symptoms were obstructive jaundice in the ADAC group (P<0.0001) and bleeding in the NADAC group (P=0.005), with larger tumor size in patients with NADAC (P=0.001). Complication rate, morbidity and mortality were comparable. Primary total pancreatoduodenectomy predominated in the NADAC group, 16.6% vs. 2.9%, and salvage completion pancreatectomy in the ADAC group, 6% vs. 0%. Significant prognostic factors for OS were perineural invasion (P=0.006) and adjuvant chemotherapy (P=0.045) in the ADAC group, and for DFS the total number of resected lymph nodes (P=0.042) and lymph node ratio (P=0.031) in the NADAC group. Median OS is 21 months and 5-year survival 27.3% in the NADAC group and 41.5 months and 52% in the ADAC group. CONCLUSION: Ampullary duodenal/intestinal adenocarcinomas are smaller than non-ampullary at diagnosis, with a higher rate of lymph node metastases, but with a better prognosis and long-term outcome in the presented cohort. Oral localisation of NADAC prevailed in the present cohort. Perineural invasion and postoperative oncological therapy are significant prognostic factors for OS in ADAC, but the total number of lymph nodes and lymph node ratio are significant prognostic factors for DFS in NADAC.

Mismatch repair deficiency in early-onset duodenal, ampullary, and pancreatic carcinomas is a strong indicator for a hereditary defect.

Mismatch repair deficiency (dMMR) is a hallmark of Lynch syndrome (LS), but its prevalence in early-onset (diagnosed under the age of 50 years) duodenal, ampullary, and pancreatic carcinomas (DC, AC, and PC, respectively) is largely unknown. We explored the prevalence of dMMR and the underlying molecular mechanisms in a retrospectively collected cohort of 90 early-onset carcinomas of duodenal, ampullary, and pancreatic origin. dMMR was most prevalent in early-onset DCs (47.8%); more than half of those were associated with hereditary cancer syndromes (LS or constitutional mismatch repair deficiency syndrome). All dMMR AC and PC were due to LS. Concordance of dMMR with underlying hereditary condition warrants ubiquitous dMMR testing in all early-onset DC, AC, and PC.

[Preoperative diagnostics in periampullary adenocarcinomas]. / Präoperative Diagnostik bei periampullären Adenokarzinomen.

BACKGROUND: Approximately 30-40% of pancreatoduodenectomies for adenocarcinomas result in nonpancreatic periampullary adenocarcinoma as the final diagnosis. Depending on the origin, a distinction is made between four different carcinomas with histomorphological subtypes. OBJECTIVE: Carcinoma location and subtype are of prognostic and therapeutic relevance; however, the preoperative differentiation is often incorrect despite modern diagnostics. MATERIAL AND METHODS: Overview of the current literature on the classification and preoperative diagnostics of periampullary adenocarcinomas. RESULTS: A precise knowledge of the papillary anatomy is necessary for the correct classification of diagnostic findings. Current studies demonstrate diagnostically valuable information from the anamnesis, imaging and endoscopy. CONCLUSION: In ca. 70-80% of cases a correct diagnosis of the type of periampullary adenocarcinoma is possible on the basis of interdisciplinary diagnostics. This potentially enables a correspondingly individualized treatment planning in the preoperative phase.

Endoscopic features of submucosal invasive non-ampullary duodenal carcinomas.

BACKGROUND AND AIM: It is imperative to distinguish superficial non-ampullary duodenal carcinomas (NADCs) between intramucosal and submucosal invasive carcinoma for treatment selection. The aim of this study was to evaluate the clinicopathological differences of intramucosal and submucosal carcinoma. METHODS: This was a retrospective, single-center study comprising 134 patients with 137 superficial NADCs during May 2005 and March 2018. Clinicopathological characteristics and treatment outcomes data were used to perform a comparative analysis of endoscopic findings, preoperative diagnoses of depth of cancer, and treatment outcomes of histologically diagnosed intramucosal and submucosal carcinoma. RESULTS: Of the 137 NADCs, 125 (91%) were intramucosal, and 12 (9%) were submucosal. The proportion of submucosal carcinoma was significantly higher on the oral side of the papilla than on the anal side (16% vs 1%, P = 0.002). Submucosal tumor-like appearance was more frequent in submucosal than in intramucosal carcinoma (58% vs 13%, P = 0.001). There was no significant difference in tumor diameter between the groups, but 33% of submucosal carcinomas were ≤ 10 mm. Correct preoperative diagnosis of depth was achieved in 33% of submucosal carcinoma. Submucosal carcinoma was frequently underestimated when tumor diameters were ≤ 10 mm. Conversely, intramucosal carcinoma was frequently overestimated when the tumor was ≥ 30 mm and had thickness or giant nodules. Lymph node metastasis was found in one submucosal carcinoma patient. CONCLUSIONS: The possibility of submucosal invasion should be considered when NADCs are located on the oral side of the papilla or have submucosal tumor-like appearance even if tumor diameters are ≤ 10 mm.

Adenocarcinoma of the duodenum arising from Brunner's gland resected by partial duodenectomy: a case report.

BACKGROUND: Duodenal carcinoma originating in Brunner's gland is rare. Herein, we report a case of duodenal carcinoma arising from Brunner's gland in a 63-year-old man. CASE PRESENTATION: On diagnostic imaging, the lesion presented as a non-invasive carcinoma; the patient also had uncontrolled diabetes and liver cirrhosis. Hence, we decided to perform partial duodenectomy to reduce operative stress. Pathological examination revealed that the tumor consisted of tissue from Brunner's gland. Additionally, the carcinoma cells were strongly positive for Mucin-6 protein, which is an epithelial marker of Brunner's gland. The patient's post-operative course was uneventful, and he has been well for 2 years after the surgery. CONCLUSIONS: This a rare case of an adenocarcinoma arising from Brunner's gland of the duodenum that was resected by duodenectomy.

Surgical Strategy for T1 Duodenal or Ampullary Carcinoma According to the Depth of Tumor Invasion.

AIM: To investigate the utility of local resection (LR) for T1 duodenal carcinoma and T1 ampullary carcinoma. PATIENTS AND METHODS: Between June 2002 and November 2014, a total of 64 patients with pathological T1 (pT1) ampullary carcinoma (25 patients) and pT1 duodenal carcinoma (39 patients) were treated. Of these, 33 patients underwent local resection (LR group), while the other 31 patients underwent pancreatoduodenectomy (PD group). RESULTS: The LR group had 31 patients with pT1a and 2 patients with pT1b. PD group had 18 patients with pT1a and 13 patients with pT1b. One patient with pT1b duodenal carcinoma (20.0%) and one patient with pT1b ampullary carcinoma (10.0%) developed lymph node metastasis, while none of the patients with pT1a disease developed metastases. CONCLUSION: LR may be considered in the patients preoperatively diagnosed with T1a duodenal carcinoma and T1a ampullary carcinoma.

Helical tomotherapy for duodenal adenocarcinoma in an elderly patient: A case report.

To evaluate the efficacy and feasibility of external beam radiotherapy (EBRT) for duodenal adenocarcinoma in an 84-year-old female who underwent EBRT (2.2 Gy/d for a total dose of 46.2 Gy) using helical tomotherapy (HT). Toxicity was evaluated on the National Cancer Institute's common toxicity criteria (CTCAE 3.0). The patient completed the treatment without G3-G4 toxicity. After 22-mo follow-up, she is alive and well, in complete remission with no late side effects. HT seems to be feasible and effective for duodenal adenocarcinoma in old to very old patients.

[Moderately differentiated adenocarcinoma of the third duodenal portion]. / Adenocarcinoma de la tercera porción duodenal moderadamente diferenciado.

BACKGROUND: Duodenal carcinoma is very rare. It represents 33-45% of the all tumours of the small bowel. The symptoms are non-specific, and the diagnosis is often accidental. CLINICAL CASE: A 35-year old man was admitted to our hospital with post-prandial abdominal pain. Upper gastrointestinal examination revealed a tumour of the third duodenal portion, which was diagnosed, using endoscopic biopsy, as a tubular adenoma with high grade dysplasia. The computed axial tomography scan, the magnetic resonance imaging, and the endoscopic ultrasound showed the neoplasia of the third duodenal portion with no lymph node or peritoneal metastases. Partial resection of the duodenum was performed. The definitive histopathological diagnosis was primary adenocarcinoma of the third duodenal portion. pT2 N0M0, originated in a tubular adenoma. CONCLUSIONS: Primitive neoplasia of the duodenum is very rare. Duodeno-cephalo-pancreatectomy is recommended in proximally located tumours, while segmental resection of the duodenum is appropriate for distal locations, with the same survival.