Duodenal Carcinoma Research Articles

Familial adenomatous polyposis gene MUTYH mutations are identified in anaplastic thyroid carcinoma

Abstract Introduction/Objective Anaplastic thyroid carcinoma (ATC) is a rare aggressive disease unresponsive to treatment. Next-Generation Sequencing (NGS) allows identification of targets for precision therapies, such as BRAF, with implications in treating ATC. Our prior work identified ATC clusters with characteristic gene mutations and multiple previously unreported mutations. One of these is MUTYH, which is associated with colon (CRC), ovarian, breast, bladder, and duodenal carcinomas in MUTYH-associated polyposis (MAP). Its association with ATC is not defined. Methods/Case Report Exploratory analysis of pathogenic mutations in 727 ATC cases using a 324-gene panel (FoundationOneCDx) and an interactive visualization of association rules was conducted for informative maximal frequent patterns among mutated gene combinations to identify gene clusters including genes not commonly associated with ATC. TCGA database was reviewed for frequency of MUTYH mutations. My Cancer Genome and the Drug-Gene Interaction Database were used to identify potential targeted drugs for MUTYH gene-mutated cancers. Results (if a Case Study enter NA) NGS showed MUTYH short-variant mutations in 17 cases (2.34%). Germline MUTYH variants incidence is 1.43% in normal samples. 5 cases harbored only Y165C and 7 had only G382D, with 1 case having both Y165C and G382D. The other 4 cases contained sporadic truncating events in MUTYH. All instances of Y165C and G382D were predicted to be germline. 6 patients (35%) had BRAF co-mutations, correlating with expected frequency of ATC thought to transform from papillary carcinoma (PC). One case had only MUTYH mutation while a second had MUTYH with TERT and TP53. TCGA database contained 6(0.2%) cases of PC with MUTYH mutations (total 2871 MUTYH-mutated carcinomas), but no cases of ATC or other thyroid tumors were described. Conclusion MUTYH is a DNA repair enzyme linked to CRC and other cancers. MUTYH mutations occur in 6.7% of MAP patients and 1–2.2% of sporadic CRC cases that are mutually exclusive of BRAF mutations. While MAP increases the lifetime risk of CRC by over 60%, the link of MUTYH mutations to PC has only been observed in MAP and not been described outside of it. To our knowledge this is the first description of MUTYH mutations in ATC, especially without concurrent BRAF mutation (11 cases), suggesting MUTYH may independently contribute to pathogenesis of ATC. PARP inhibitors, PD-1/PD-L1 inhibitors, and ATR inhibitors demonstrate promising results in clinical trials of MUTYH-mutated cancers, warranting further research.

7410 Bevacizumab Induced Hypothyroidism

Abstract Disclosure: A.L. McKenna: None. S. Thota-Kammili: None. K. Grennan: None. S. Rao: None. It is known that thyroid dysfunction is an adverse effect of tyrosine kinase inhibitors (TKIs) via inhibition of the vascular endothelial growth factor receptor (VEGFR). The VEGF signaling pathway is important in regulating angiogenesis in tumors and normal tissue. As a highly vascularized gland, it is expected the thyroid may be compromised because of VEGFR inhibitors. Proposed mechanisms of thyroid dysfunction include capillary regression and constriction leading to reduced vascular perfusion to the gland. Bevacizumab is a monoclonal antibody that targets VEGF and inhibits activation of VEGFR, reducing angiogenesis; interestingly, this is not commonly reported in the literature. Here we present a case where bevacizumab altered thyroid function in a patient with pre-existing primary hypothyroidism. A 60-year-old woman presented with worsening hypothyroidism. She reported a history of nonautoimmune primary hypothyroidism diagnosed in her early 20s; she still had an intact thyroid with no history of head/neck radiation. In the months leading to the presentation, she had been receiving maintenance chemotherapy with 5 fluorouracil and bevacizumab every 2 weeks for metastatic duodenal carcinoma. Prior to starting chemotherapy, she had been on a steady dose of 112 mcg of levothyroxine (LT4) daily. After 6 months on chemotherapy, she reported symptoms of fatigue and hair loss. Workup revealed an elevated TSH of 41 mIU/L (reference 0.3-4.2 mIU/L). She denied any significant changes in weight or malabsorption issues. Aside from the chemotherapy, no other new medications were introduced. Her LT4 dose was increased to 137 mcg daily, and 3 months later, there was improvement in TSH to 6.5 mIU/L as well as her symptoms. At this point, bevacizumab was briefly on hold for 2 months due to an upcoming procedure, hence there was no change to LT4 to avoid over correction. Her TSH normalized and was trending lower while bevacizumab was on hold. Once bevacizumab resumed, LT4 was preemptively increased to 150 mcg daily. Four months after resuming bevacizumab, her TSH remains within the normal reference range on 150 mcg LT4. It is hypothesized that this VEGF blocker led to worsening of her underlying hypothyroidism.Given the shared down regulation in the VEGF signaling pathway, it would be expected that TKIs and monoclonal antibodies against VEGF would share similar adverse reactions to the thyroid. However, to our knowledge, there have been limited reports of bevacizumab induced thyroid dysfunction in the literature. Explanations to this may include the more targeted VEGF inhibition with bevacizumab versus the inhibition of multiple kinase receptors from the TKIs. Further research into this will be needed. This case demonstrates that bevacizumab can impact thyroid function and thyroid labs may need to be monitored at the onset and during VEGF inhibitor therapies. Presentation: 6/3/2024

Initial Tumor Size and Narrow-Band Image Findings Estimate Growth Speed in Duodenal Tumors.

Recently, the detection of superficial non-ampullary duodenal epithelial tumors (SNADETs) including adenomas and superficial duodenal carcinomas has increased. Various endoscopic treatment methods have also been reported for SNADETs, but there are few reports on the natural history. The aim of this study was to analyze factors related to tumor growth and determine the characteristics of SNADETs which need early therapeutic intervention. A single-center, retrospective study was performed on the medical records of 309 patients with SNADETs who underwent endoscopic or surgical resection between January 2010 and May 2021. Of these, 41 patients who were followed up for more than 1 year by endoscopy were analyzed. The primary outcome was an analysis of the tumor growth speed. Secondary outcomes were the relationship between the tumor growth speed and mucin phenotype, tumor size and findings of magnifying endoscopy with narrow-band imaging (M-NBI). The observation period was 24 months (13-182). Tumor growth speed was 1.1 mm/year (0-21.6). Tumor diameter ≥10 mm at first detection (p = 0.004; odds ratio 19.5 [2.03-186.96]) and mixed type by M-NBI (p = 0.036; odds ratio 9.69 [1.05-89.88]) were identified as risk factors of tumors growing at a rate of ≥3 mm/year. There was no statistically significant difference in the speed of tumor growth between the different mucin immunohistochemical phenotypes. Initial tumor size and findings of M-NBI are useful to predict tumor growth and consider early intervention.

Clinicopathological Study of Gastroduodenal Biopsies and Correlate with Endoscopic Findings in Northeast India- One- Year Cross-sectional Study in a Tertiary Care Centre

Background: The gastrointestinal tract (GIT) is a hollow muscular tube extending from the oral cavity to the anus. Acid peptic disease is among the most common disorders affecting this region globally, caused by an imbalance between acid secretion and gastric mucosal defenses. Endoscopy has enhanced the accuracy and early histologic diagnosis of mucosal lesions. This cross-sectional study examines 100 gastroduodenal biopsies over a one-year period. Methods: The study categorized histopathological findings from gastro-duodenal biopsies collected via endoscopy and analyzed their correlation with endoscopic results. Results: Gastroduodenal lesions were more prevalent in males, particularly in the fifth decade of life. Endoscopic findings included 12 normal cases, 31 ulcers, 15 erosions, 4 polyps, and 38 malignancies. Among the 38 malignancies, 10 were premalignant, 23 were diagnosed as gastric carcinoma, and 4 as duodenal carcinoma, demonstrating a strong correlation between endoscopic and histopathological findings. Conclusion: Endoscopic gastroduodenal biopsies are instrumental in diagnosing both benign and malignant lesions across various age groups and sites. The combination of endoscopy and histopathological analysis provides a powerful diagnostic tool for better patient management.

Radiomics analysis based on CT for predicting lymph node metastasis and prognosis in duodenal papillary carcinoma

ObjectivesRadiomics has been demonstrated to be strongly associated with TNM stage and patient prognosis. We aimed to develop a model for predicting lymph node metastasis (LNM) and survival.MethodsFor radiomics texture selection, 3D Slicer 5.0.3 software and the least absolute shrinkage and selection operator (LASSO) algorithm were used. Subsequently, the radiomics model, computed tomography (CT) image, and clinical risk model were compared. The performance of the three models was evaluated using receiver operating characteristic (ROC) curves, decision curve analysis (DCA), calibration plots, and clinical impact curves (CICs).ResultsFor the LNM prediction model, 224 patients with LNM information were used to construct a model that was applied to predict LNM. According to the CT data and clinical characteristics, we constructed a radiomics model, CT imaging model and clinical model. The radiomics model for evaluating LNM status showed excellent calibration and discrimination in the training cohort (AUC = 0.926, 95% CI = 0.869–0.982) and the validation cohort (AUC = 0.872, 95% CI = 0.802–0.941). DeLong’s test demonstrated that the difference among the three models was significant. Similarly, DCA and CIC showed that the radiomics model has better clinical utility than the CT imaging model and clinical model. Our model also exhibited good performance in predicting survival—in line with the findings of the model built with clinical risk factors.ConclusionsCT radiomics models exhibited better predictive performance for LNM than models built based on clinical risk characteristics and CT imaging and had comparative clinical utility for predicting patient prognosis.Critical relevance statementThe radiomics model showed excellent performance and discrimination for predicting LNM and survival of duodenal papillary carcinoma (DPC).Key PointsLNM status determines the most appropriate treatment for DPC.Our radiomics model for evaluating the LNM status of DPC performed excellently.The radiomics model had high sensitivity and specificity for predicting survival, exhibiting great clinical value.Graphical

MDB-02. RECURRNET SPINAL DESMOID FIBROMATOSIS IN A CHILD WITH APC GERMLINE MUTATION-RELATED MEDULLOBLASTOMA

Abstract BACKGROUND Familial adenomatous polyposis (FAP) is an autosomal-dominant dominant Familial Cancer Syndrome FCs Caused by Germline inactivation of adenomatous polyposis coli (APC) tumor suppressor gene, results in up-regulation of the WNT signaling pathway which increases the risk of colorectal cancer CRC, colon polyposis and extracolonic tumors like gastric and duodenal carcinomas WNT-activated Medulloblastoma MBL and Desmoid fibromatosis (DF) DF are locally aggressive soft tissue tumors with high local recurrence rates after Surgical excision role of adjuvant chemotherapy or Radiotherapy is still unclear. METHODS A 6-year-old boy product of non-consanguineous marriage with positive family HX brain tumor Diagnosed with Non-Metastatic Average Risk MBL WHO grade IV classic histology, he had craniotomy and Gross tumor resection followed by craniospinal irradiation at 23.4 Gy, plus a boost to posterior fossa/tumor bed up to 54 Gy, followed by 8 maintenance chemotherapy cycles as HIT-MED protocol Cisplatin/VCR/CCNU total. 3 years post end of therapy the pateint was in complete remission from MBL when routing MRI surveillance showed a new left paravertebral muscle lesion within the irradiation field from T1 to T4 levels which progressed gradually over a short follow-up time he had surgical resection of the tumor with Pathological diagnosis of Desmoid fibromatosis. after six months he had a second recurrence of the spinal tumor that needed a second surgical resection. Result: positive Genetic testing for Germline pathogenic mutation of APC gene exons 7 to 16 confirmed the diagnosis of autosomal familial adenomatous polyposis type 1(FAP1). Up to date, he is stable on endoscopic and MRI surveillance with radiological evidence of slow progression of the paraspinal tumor CONCLUSION Medulloblastomas associated with APC germline mutation have favorable outcomes that may need de-escalating therapeutic protocol with reduced intensity craniospinal irradiation aiming to reduce the incidence of secondary tumors after adjuvant treatment for MBL

Effect of cancer screening with multi-cancer early detection testing and whole body MRI on cancer worry in a high-risk population.

10520 Background: Multi-cancer Early Detection tests (MCED) and whole-body imaging are being increasingly utilized for early cancer detection but are not included in cancer screening guidelines. Our aims were to 1) determine the feasibility of MCED testing and whole-body MRI (WBM) to screen people at high-risk for cancer after germline testing and cancer genetics evaluation and 2) understand the impact of MCED and WBM on cancer worry and anxiety. Methods: We conducted a feasibility trial of people >50 years old with a strong family history of cancer and/or a pathogenic/likely pathogenic germline variant in a cancer susceptibility gene for which WBM is not part of standard risk management. Participants completed a baseline survey including the SF12, State Trait Anxiety Index (STAI), and modified cancer worry scale (CWS). They then underwent non-contrast whole body 3T MRI and liquid biopsy using DEEPGEN, a mutational-based MCED. Results were reported to participants and additional imaging studies or consultations were obtained as needed. Participants were surveyed 6 months after testing using SF12, STAI, CWS, and a questionnaire on acceptability of the intervention. Results: 100 participants were enrolled; 73 have completed 6-month follow-up. Median age was 62 years (IQR 57-66) and 64% of participants were women. MCED was positive in 4 participants, none of which had suspicious findings on WBM. 27 (27%) underwent additional imaging to further evaluate findings identified on WBM. 4 (4%) had cancer diagnosed based on WBM findings and subsequent work up (lung adenocarcinoma, prostate cancer, duodenal neuroendocrine carcinoma, and ovarian Brenner tumor); all 4 underwent surgical resection. One participant underwent bone marrow biopsy due to JAK2 mutation and thrombocytosis (evaluation ongoing) and one participant had a thyroid biopsy for a benign nodule. 62 (85%) somewhat/strongly agreed that study participation reduced cancer worry. Composite CWS scores showed decreased worry at 6 months compared with baseline (12.7 vs 13.7, p=0.003). At baseline, 30 (41%) reported that they somewhat/very often worry about getting cancer, compared with 14 (18%) at 6 months (p<0.001). 8 (11%) reported at baseline that cancer worry affected their mood somewhat/very often, compared with 2 (3%) at 6 months (p<0.001). There were no significant differences in STAI or SF12 composite scores between baseline and 6 months. 69 (95%) somewhat/strongly agreed that they would like to participate in future testing with WBM and MCED. Conclusions: MCED and WBM testing is feasible and was associated with decreased cancer worry at 6 months. In this high-risk cohort, cancer was detected in 4% of patients. Further evaluation of cancer screening in high-risk populations using WBM and MCED testing is warranted particularly with respect to patient-centered outcomes, cancer outcomes, and healthcare resource utilization. Clinical trial information: NCT05868486 .

Preoperative profiles of plasma amino acids and derivatives distinguish periampullary cancer and benign disease

Periampullary cancers, including pancreatic ductal adenocarcinoma, ampullary-, cholangio-, and duodenal carcinoma, are frequently diagnosed in an advanced stage and are associated with poor overall survival. They are difficult to differentiate from each other and challenging to distinguish from benign periampullary disease preoperatively. To improve the preoperative diagnostics of periampullary neoplasms, clinical or biological markers are warranted.In this study, 28 blood plasma amino acids and derivatives from preoperative patients with benign (N = 45) and malignant (N = 72) periampullary disease were analyzed by LC-MS/MS.Principal component analysis and consensus clustering both separated the patients with cancer and the patients with benign disease. Glutamic acid had significantly higher plasma expression and 15 other metabolites significantly lower plasma expression in patients with malignant disease compared with patients having benign disease. Phenylalanine was the only metabolite associated with improved overall survival (HR = 0.50, CI 0.30–0.83, P < 0.01).Taken together, plasma metabolite profiles from patients with malignant and benign periampullary disease were significantly different and have the potential to distinguish malignant from benign disease preoperatively.

Endoscopic treatment for early duodenal papillary carcinoma: long-term outcomes.

This study aims to determine whether endoscopic papillectomy (EP) is a safe and effective treatment for early duodenal papillary carcinoma with long-term follow-up. From June 2012 to September 2022, 48 patients with early duodenal papilloma carcinoma who received endoscopic treatment were included. The histological types, percentage of complete resections, postoperative residuals, adverse events, and recurrences were evaluated. EP was successful in all patients; 46 were lumped, and two were fragmented, with a 95.8% intact removal rate (46/48). The preoperative biopsy pathological positive rate was 70.8% (34/48). The incidence of early postoperative adverse events (within 1month after EP) were 16.7% (8/48), including four cases of acute pancreatitis, three cases of delayed bleeding, and one case of acute cholangitis. In addition, 4.2% (2/48) of the late adverse events were bile duct stenosis. After 6months, the postoperative residual rate was 0%. The median time to recurrence was 17.5months, and the postoperative recurrence rate was 16.7% (8/48) in patients treated with radiofrequency ablation. The median progression-free survival was 18.6months (95% CI, 12.1-25.1), and the median overall survival was 121.5months (95% CI, 105.6-120.9). EP is a safe and efficient alternative therapy for early duodenal papillary carcinoma. Endoscopic follow-up and treatment are essential because of the potential for recurrence.

Endoscopic papillectomy could be rewarding to patients with early stage duodenal ampullary carcinoma?

There is currently no consensus on the use of endoscopic papillectomy (EP) for early stage duodenal ampullary adenocarcinoma. This study aimed to evaluate the feasibility of EP for patients with early stage duodenal ampullary adenocarcinoma. Patients who underwent EP for ampullary adenocarcinomas were investigated. Complete and clinical complete resection rates were evaluated. Clinical complete resection was defined as either complete resection or resection with positive or unknown margins but no cancer in the surgically resected specimen, or no recurrence on endoscopy after at least a 1-year follow-up. Adenocarcinoma developed in 30 patients (carcinoma insitu [Tis]: 21, mucosal tumors [T1a(M)]: 4, tumors in the sphincter of Oddi [T1a(OD)]: 5). The complete resection rate was 60.0% (18/30) (Tis: 66.7% [14/21], T1a[M]: 50.0% [2/4], and T1a[OD]: 40.0% [2/5]). The mean follow-up period was 46.8 months. The recurrence rate for all patients was 6.7% (2/30). The clinical complete resection rates of adenocarcinoma were 89.2% (25/28); rates for Tis, T1a(M), and T1a(OD) were 89.4% (17/19), 100% (4/4), and 80% (4/5), respectively. EP may potentially achieve clinical complete resection of early stage (Tis and T1a) duodenal ampullary adenocarcinomas.

Development and validation of web calculators to predict early recurrence and long-term survival in patients with duodenal papilla carcinoma after pancreaticoduodenectomy

BackgroundDuodenal papilla carcinoma (DPC) is prone to relapse even after radical pancreaticoduodenectomy (PD) (including robotic, laparoscopic and open approach). This study aimed to develop web calculators to predict early recurrence (ER) (within two years after surgery) and long-term survival in patients with DPC after PD.MethodsPatients with DPC after radical PD were included. Univariate and multivariate logistic regression analyses were used to identify independent risk factors. Two web calculators were developed based on independent risk factors in the training cohort and then tested in the validation cohort.ResultsOf the 251 patients who met the inclusion criteria, 180 and 71 patients were enrolled in the training and validation cohorts, respectively. Multivariate logistic regression analysis revealed that tumor size [Odds Ratio (OR) 1.386; 95% confidence interval (CI) 1070–1.797; P = 0.014]; number of lymph node metastasis (OR 2.535; 95% CI 1.114–5.769; P = 0.027), perineural invasion (OR 3.078; 95% CI 1.147–8.257; P = 0.026), and tumor differentiation (OR 3.552; 95% CI 1.132–11.152; P = 0.030) were independent risk factors for ER. Nomogram based on the above four factors achieved good C-statistics of 0.759 and 0.729 in predicting ER in the training and the validation cohorts, respectively. Time-dependent ROC analysis (timeROC) and decision curve analysis (DCA) revealed that the nomogram provided superior diagnostic capacity and net benefit compared with single variable.ConclusionsThis study developed and validated two web calculators that can predict ER and long-term survival in patients with DPC with high degree of stability and accuracy.

Predictive value of preoperative albumin-bilirubin score and other risk factors for short-term outcomes after open pancreatoduodenectomy.

Pancreatoduodenectomy represents a complex procedure involving extensive organ resection and multiple alimentary reconstructions. It is still associated with high morbidity, even in high-volume centres. Prediction tools including preoperative patient-related factors to preoperatively identify patients at high risk for postoperative complications could enable tailored perioperative management and improve patient outcomes. To evaluate the clinical significance of preoperative albumin-bilirubin score and other risk factors in relation to short-term postoperative outcomes in patients after open pancreatoduodenectomy. This retrospective study included all patients who underwent open pancreatic head resection (pylorus-preserving pancreatoduodenectomy or Whipple resection) for various pathologies during a five-year period (2017-2021) in a tertiary care setting at University Medical Centre Ljubljana, Slovenia and Cattinara Hospital, Trieste, Italy. Short-term postoperative outcomes, namely, postoperative complications, postoperative pancreatic fistula, reoperation, and mortality, were evaluated in association with albumin-bilirubin score and other risk factors. Multiple logistic regression models were built to identify risk factors associated with these short-term postoperative outcomes. Data from 347 patients were collected. Postoperative complications, major postoperative complications, postoperative pancreatic fistula, reoperation, and mortality were observed in 52.7%, 22.2%, 23.9%, 21.3%, and 5.2% of patients, respectively. There was no statistically significant association between the albumin-bilirubin score and any of these short-term postoperative complications based on univariate analysis. When controlling for other predictor variables in a logistic regression model, soft pancreatic texture was statistically significantly associated with postoperative complications [odds ratio (OR): 2.09; 95% confidence interval (95%CI): 1.19-3.67]; male gender (OR: 2.12; 95%CI: 1.15-3.93), soft pancreatic texture (OR: 3.06; 95%CI: 1.56-5.97), and blood loss (OR: 1.07; 95%CI: 1.00-1.14) were statistically significantly associated with major postoperative complications; soft pancreatic texture was statistically significantly associated with the development of postoperative pancreatic fistula (OR: 5.11; 95%CI: 2.38-10.95); male gender (OR: 1.97; 95%CI: 1.01-3.83), soft pancreatic texture (OR: 2.95; 95%CI: 1.42-6.11), blood loss (OR: 1.08; 95%CI: 1.01-1.16), and resection due to duodenal carcinoma (OR: 6.58; 95%CI: 1.20-36.15) were statistically significantly associated with reoperation. The albumin-bilirubin score failed to predict short-term postoperative outcomes in patients undergoing pancreatoduodenectomy. However, other risk factors seem to influence postoperative outcomes, including male sex, soft pancreatic texture, blood loss, and resection due to duodenal carcinoma.

A case of non-ampullary duodenal adenosquamous carcinoma with successful emergency pancreaticoduodenectomy for gastrointestinal hemorrhage

BackgroundAlthough most duodenal carcinomas are pathological adenocarcinomas, a small number of cases have been reported of adenosquamous carcinoma, characterized by variable combinations of two malignant components: adenocarcinoma and squamous cell carcinoma. However, owing to the small number of cases of non-ampullary duodenal adenosquamous carcinoma, there have been no reported cases of emergency pancreaticoduodenectomy for gastrointestinal hemorrhage due to non-ampullary duodenal adenosquamous carcinoma.Case presentationA 66-year-old Japanese male presented to the referring hospital with a chief complaint of abdominal pain, diarrhea, and dark urine that had persisted for 1 month. The patient was referred to our hospital because of liver dysfunction on a blood examination. Laboratory results of the blood on the day of admission showed that total and direct bilirubin levels (12.0 mg/dl and 9.6 mg/dl) were markedly increased. An endoscopic retrograde biliary drainage tube was inserted for the treatment of obstructive jaundice, and imaging studies were continuously performed. Contrast-enhanced computed tomography and endoscopy revealed an ill-defined lesion involving the second portion of the duodenum, predominantly along the medial wall, and measuring 60 mm in diameter. No metastases were observed by positron emission tomography. Pancreaticoduodenectomy was planned based on the pathological findings of poorly differentiated adenocarcinoma. However, 2 days before the scheduled surgery, the patient experienced hemorrhagic shock with melena. Owing to poor hemostasis after endoscopic treatment and poor control of hemodynamic circulation despite blood transfusion, radiological embolization and hemostasis were attempted but were incomplete. An emergency pancreaticoduodenectomy was performed after embolizing the route from the gastroduodenal artery and pseudoaneurysm area to reduce bleeding. The operation was completed using an anterior approach without Kocherization or tunneling due to the huge tumor. The operation time was 4 h and 32 min, and blood loss was 595 mL The pathological diagnosis was adenosquamous carcinoma. The postoperative course was uneventful with 17 day hospital stay and the patient is currently well, with no signs of recurrence 9 months after surgery.ConclusionsThis report presents an extremely rare case of successful emergency pancreaticoduodenectomy for gastrointestinal hemorrhage caused by non-ampullary duodenal adenosquamous carcinoma.

A novel case of EUS-guided targeted radiofrequency ablation of metastatic duodenal renal cell carcinoma.

A novel case of EUS-guided targeted radiofrequency ablation of metastatic duodenal renal cell carcinoma.

Metastatic duodenal carcinoma presenting as severe anaemia and extensive bilateral lower limb deep venous thrombosis

No abstract available

An autopsy case of alpha-fetoprotein-producing large duodenal adenocarcinoma.

We report an autopsy case of a large duodenal adenocarcinoma that produced alpha-fetoprotein (AFP). The patient was a man in his 70s with diabetes mellitus. He presented with epigastralgia and was referred to our hospital. Upper gastrointestinal endoscopy and abdominal computed tomography revealed a large tumor of 11cm in diameter in the descending limb of the duodenum. A tumor biopsy showed poorly differentiated adenocarcinoma. Although his carcinoembryonic antigen (CEA) and carbohydrate antigen 19-9 (CA19-9) levels were within the normal range, his AFP levels were significantly elevated (42,078.4ng/mL). Due to vascular invasion, curative resection was not feasible, and chemotherapy was chosen as the treatment option. After gastrojejunostomy was performed to enable oral intake, one cycle of modified leucovorin/5-fluorouracil/oxaliplatin (mFOLFOX6) therapy was administered. However, it proved ineffective, and the patient's anorexia gradually worsened. Ultimately, he succumbed to the progression of cancer cachexia. Autopsy findings revealed a 14-cm-long duodenal carcinoma primarily located in the duodenal bulb, with direct invasion into the stomach, pancreas, and liver. A pathological examination confirmed a diagnosis of poorly differentiated adenocarcinoma with AFP production. Duodenal cancer is rare, and AFP-producing duodenal cancer is even rarer, with only 21 reported cases, including our own. We present this autopsy case of AFP-producing duodenal adenocarcinoma and review the cases reported in the relevant literature.

Type 2 Endoleak Detection After Endovascular Aneurysm Repair: Can Low-Flow Endoleaks Also Be Visualized in Silicon-Photomultiplier-Based Positron Emission Tomography/Computed Tomography Scans?

To present a novel clinical application of silicon-photomultiplier-based positron emission tomography (SiPM-based PET)/computed tomography (CT), detecting a type II endoleak 5 years after endovascular aneurysm repair (EVAR). SiPM-based PET/CT scans with a standard whole-body protocol were performed for a 73-year-old man with a past medical history of abdominal aortic aneurysms treated with EVAR and currently under investigation of his duodenal papillary carcinoma. The PET/CT demonstrated 18F-fluorodeoxyglucose (FDG) accumulation outside the stent graft in the native sac of the aneurysm. The site of accumulation corresponded to that of the contrast enhancement depicted in the CT angiography taken 1 month earlier. Another CT scan performed 3 months later revealed enlargement of the aneurysm. SiPM-based PET/CT, with its superior sensitivity and spatial resolution over conventional PET/CT, can detect type II low-flow endoleaks. Abnormal intra-aneurysmal FDG activity incidentally detected on SiPM-based PET/CT is worthy of attention because it may be indicative of endoleaks. Additional imaging using different modalities should be considered so that the patient would not miss the additional treatment opportunity upon observing sac enlargement. For patients with contraindications for iodine CT contrast media, SiPM-based PET/CT would serve as a suitable alternative.

Survival outcomes in duodenal carcinoma based on facility type: A National Cancer Database analysis.

e16328 Background: Duodenal Carcinoma (DC) is a rare entity, accounting for 0.5% of gastrointestinal tumors and up to 50% of all small intestinal tumors. Few studies have characterized survival outcomes in DC due to its rarity. This study aims to evaluate if Academic centers (AC) have better survival outcomes compared to Non-academic centers (NAC). Methods: A retrospective analysis of the National Cancer Database was conducted from 2004-2019 for subjects with DC, identified by ICD code C17.0 (n=26,070). Features were compared between patients (pts) at AC (n=15,020) and NAC (n=11,050). Overall survival (OS) was estimated using Kaplan Meier method and difference in OS was measured using pairwise log-rank test. Multivariate analysis was performed using binary logistic regression. Results: The median OS in pts with DC was 44 months. Treatment at AC was associated with a longer median OS than NAC (65 vs 32 months, p&lt;0.001). Pts with higher Charleson/Deyo scores (CDS) of 2-3 were less likely to go to AC (OR 0.75, 95% CI 0.67-0.83, p&lt;0.001). AC attracted age ranges &lt;65 years compared to &gt;70 years (OR 0.81, 95% CI 0.74 - 0.88, p&lt;0.001) and &gt;85 years (OR 0.53, 95% CI 0.47-0.60, p&lt;0.001). African Americans (AA) and Asians were more likely to go to AC compared to Whites [(AA OR 1.21, 95% CI 1.13-1.3, p&lt;0.001); (Asian OR 1.43, 95% CI 1.24-1.65, p&lt; 0.001)]. Spanish/Hispanic pts were more likely to go to AC than non-Spanish/Hispanic (OR 1.26, 95% CI 1.13-1.41, p&lt;0.001). Pts in upper income quartiles were more likely to be treated at AC (OR 1.19, 95% CI 1.08-1.32, p&lt;0.001). Pts with Medicare (OR 0.71, 95% CI 0.60-0.83, p&lt;0.001) were less likely to go to AC compared to uninsured pts. Pts from Central USA (OR 0.67, 95% CI 0.63-0.70, p &lt;0.001) and the West Coast (OR 0.46, 95% CI 0.43-0.70, p&lt;0.001) were less likely to go to AC compared to pts from the East Coast. Pts in rural areas were less likely to go to AC compared to those in metros (OR 0.70, 95% CI 0.57-0.87, p&lt;0.001). The 30-day readmission rates after surgery were also studied. Unplanned 30-day readmission rates for surgically treated pts at AC were higher than those at NAC (OR 1.15, 95% CI 1.00-1.31, p=0.041). Conclusions: The median OS of pts with DC who were treated at AC was significantly longer compared to those treated at NAC. Pts with higher CDS were less likely to go to AC. AC had higher unplanned 30-day readmission rates for surgically treated pts than NAC. Pts with DC treated at AC were more likely to be uninsured, younger, and from ethnic minorities. Significant geographical differences also existed where pts from the East Coast and metros were more likely to go to AC compared to those in the West coast, Central and rural areas. Geography may play a role in serving densely populated urban centers where socio-economic factors drive larger minority population to AC facilities. Nonetheless, the difference in overall survival between AC and NAC deserves additional studies to improve outcomes of pts served by NAC.

Efficacy and safety of preoperative immunotherapy in patients with mismatch repair-deficient or microsatellite instability-high gastrointestinal malignancies.

Programmed death protein (PD)-1 blockade immunotherapy significantly prolongs survival in patients with metastatic mismatch repair-deficient (dMMR)/microsatellite instability-high (MSI-H) gastrointestinal malignancies such gastric and colorectal cancer. However, the data on preoperative immunotherapy are limited. To evaluate the short-term efficacy and toxicity of preoperative PD-1 blockade immunotherapy. In this retrospective study, we enrolled 36 patients with dMMR/MSI-H gastrointestinal malignancies. All the patients received PD-1 blockade with or without chemotherapy of CapOx regime preoperatively. PD1 blockade 200 mg was given intravenously over 30 min on day 1 of each 21-d cycle. Three patients with locally advanced gastric cancer achieved pathological complete response (pCR). Three patients with locally advanced duodenal carcinoma achieved clinical complete response (cCR), followed by watch and wait. Eight of 16 patients with locally advanced colon cancer achieved pCR. All four patients with liver metastasis from colon cancer reached CR, including three with pCR and one with cCR. pCR was achieved in two of five patients with non-liver metastatic colorectal cancer. CR was achieved in four of five patients with low rectal cancer, including three with cCR and one with pCR. cCR was achieved in seven of 36 cases, among which, six were selected for watch and wait strategy. No cCR was observed in gastric or colon cancer. Preoperative PD-1 blockade immunotherapy in dMMR/MSI-H gastrointestinal malignancies can achieve a high CR, especially in patients with duodenal or low rectal cancer, and can achieve high organ function protection.

Well-concealed advanced duodenal carcinoma with Muir–Torre syndrome: a case report and review of literature

BackgroundMuir–Torre syndrome is an autosomal-dominant mutation in mismatch repair genes that gives rise to sebaceous tumors and visceral malignancies over time. Because colorectal and genitourinary cancers are common in Muir–Torre syndrome, duodenal carcinoma diagnoses are often delayed.Case presentationA 58-year-old woman presented with severe emaciation, anorexia, and upper abdominal pain. She had a history of rectal carcinoma, ascending colon carcinoma, and a right shoulder sebaceous carcinoma. Upper gastrointestinal endoscopy and computed tomography examinations suggested duodenal obstruction due to superior mesenteric artery syndrome, leading to long-term observation. Seven months later, she was finally diagnosed with duodenal carcinoma of the third portion. As the papilla of Vater was preservable due to tumor location, she received a partial duodenectomy in lieu of a pancreatoduodenectomy. Pathologically, the tumor was a well-differentiated adenocarcinoma with a classification of T3N0M0 Stage IIA (UICC, 8th edition). The postoperative course was uneventful and her appetite returned. A mutation in mismatch repair gene MSH2 confirmed the diagnosis of Muir–Torre syndrome genetically. Three years later, her nutritional status has fully recovered and she is free from both recurrence and metastasis.ConclusionIn patients with comorbid skin sebaceous tumors and gastrointestinal malignancies, genetic screening is strongly recommended. Patients with Muir–Torre syndrome require long-term follow-up, and function-preserving treatment is desirable.