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BACKGROUND: Primary adenocarcinoma of the duodenum is implicated in less than 0.5% of all gastrointestinal cancers. Although rare, benign looking duodenal ulcers at time of endoscopy may represent more sinister pathology, particularly in the absence of H. pylori or NSAID use. AIMS: We report a case of an elderly man with a benign appearing duodenal ulcer at endoscopy who ultimately developed metastatic carcinoma from a primary adenocarcinoma of the duodenum. METHODS: Case report RESULTS: A 72 year old male presented for a routine screening colonoscopy. At time of endoscopy, the patient gave a history of vague epigastric discomfort and melena stools over the past two days. He denied ASA or non-steroidal drug use and was a non-smoker. A gastroscopy and colonoscopy were pursued. Upon examination of the duodenal bulb, a small, benign appearing ulcer was noted on the anterior wall with no active bleeding. Antral biopsies for H.pylori were negative. No intestinal metaplasia was seen. He was prescribed proton pump inhibitor and instructed to seek follow up care with his family doctor. Approximately 8 months later, the patient began experiencing epigastric pain, nausea, early satiety and weight loss. He presented to the emergency room 11 months after the original endoscopy. A CT scan of the abdomen showed multiple hepatic metastases and lymphadenopathy at the porta-hepatis. A repeat gastroscopy revealed an ulcerated, stenotic mass at the pylorus, extending from the duodenal bulb. Biopsies of the pylorus revealed invasive adenocarcinoma, intestinal type. The patient was subsequently referred for palliative care. CONCLUSIONS: This case highlights that not all duodenal ulcers are benign. It may be worthwhile to ensure patients follow up with their endoscopist should they develop a recurrence of symptoms, at which time biopsy of the actual ulcer, if not healed, could be performed. FUNDING AGENCIES: None

As primary duodenal adenocarcinoma is rare, the prognostic factors of this disease remain insufficiently explored, especially in China. We identified postoperative duodenal adenocarcinoma patients at a Chinese double-center (from 2006 to 2016) or who were registered with the Surveillance, Epidemiology, and End Results (SEER) database (from 2004 to 2014). Clinicopathological features and significant prognostic factors for cancer-specific survival (CSS) were reviewed and analyzed by using univariate and multivariate Cox proportional hazards regression. Then, a nomogram predicting CSS was constructed based on the SEER database and validated externally by using the separate Chinese cohort. Totally, 137 patients from the Chinese double-center and 698 patients from the SEER database were included for analysis. The multivariate analyses showed that age, tumor grade and TNM stage were independent prognostic factors. The nomogram constructed using these factors showed a clear prognostic superiority to the AJCC-TNM classification, 7th ed. (C-index: SEER cohort, 0.693 vs 0.625, P P

Highlights • Laparoscopic and endoscopic cooperating surgery (LECS) enabled en bloc resection with adequate surgical margins and secure intra-abdominal suturing for duodenal neoplasms. • Thorough mobilization of the mesocolon and pancreas head is essential for this procedure because it facilitates correct resection and suturing. • LECS is a feasible treatment option for duodenal neoplasms., Introduction We report a case of primary adenocarcinoma in the third portion of the duodenum (D3) curatively resected by laparoscopic and endoscopic cooperating surgery (LECS). Presentation of case A 65-year-old woman had a routine visit to our hospital for a follow-up of rectal cancer resected curatively 2 years ago. A routine screening gastroduodenal endoscopy revealed an elevated lesion of 20 mm in diameter in the D3. The preoperative diagnosis was adenoma with high-grade dysplasia; however, suspicion about potential adenocarcinoma was undeniable. Curative resection was performed by LECS. Pathological examination revealed intramucosal adenocarcinoma arising from normal duodenal mucosa. The tumor was stage I (T1/N0/M0) in terms of the tumor, nodes, metastasis (TNM) classification. LECS for duodenal tumor has seldom been reported previously, and this is the first report of LECS for primary adenocarcinoma in the D3. The transverse mesocolon was removed from the head of pancreas to expose the duodenum, and the accessory right colic vein was cut; this was followed by the Kocher maneuver for mobilization of the lesion site. Discussion LECS enabled en bloc resection with adequate surgical margins and secure intra-abdominal suturing. Thorough mobilization of the mesocolon and pancreas head is essential for this procedure because it facilitates correct resection and suturing. Conclusion LECS is a feasible treatment option for duodenal neoplasms, including intramucosal adenocarcinoma, even though it exists in the D3.

The location of a primary duodenal adenocarcinoma is exceptional. Only few cases have been reported in the literature. We hereby report a case of primary adenocarcinoma of the duodenum with a 60 years old patient admitted because of chronic iron deficiency anemia. Endoscopic examination revealed a budding and ulcerated process in the second duodenum. The biopsy analysis showed primary adenocarcinoma. No other primary tumor was found. The staging directed showed cephalic pancreatic invasion. The patient died in the aftermath of a surgical management.

Pulmonary nodules (PN), frequently found on imaging studies, represent a diagnostic challenge during the follow up of cancer patients. However, published data regarding investigation of PNs incidentally found on chest imaging is scarce. The PN may be present at the time of cancer diagnosis, or arise during the treatment or follow-up periods. In the context of the oncologic patient these lesions are quite invariably considered as metastases, what impacts directly on patients treatment and prognosis. The present study reports 2 cases of pulmonary nodules found in two patients already diagnosed with cancer. Case 1 referred to a woman with squamous cell carcinoma and two pulmonary nodules, and in case 2 the patient was diagnosed with duodenal adenocarcinoma. Both patients were submitted to pulmonary biopsies before the oncologic treatment. In both cases the nodules were of infectious origin, what changed significantly the neoplasia staging and the oncologic treatment intention. The authors performed a literature review as well as a discussion about the management of PN in cancer patients.

Introduction Tumours involving the duodenum are usually treated with pancreaticoduodenectomy, which may be associated with considerable morbidity. Limited distal duodenal resection, a relatively smaller procedure, can be done in some of these patients. We describe our experience with this operation for such lesions. Methods We retrospectively analyzed, from prospectively collected data 10 consecutive patients who underwent limited duodenal and proximal jejunal resection between March 2011 and Nov 2015. Results There were 8 males and 2 females who had a median age of 47 years. Their common presentations were abdominal pain (50%) and upper gastrointestinal bleeding (40%). Five had malignancy (adenocarcinoma: 2, neuroendocrine tumours: 2, non Hodgkin's lymphoma 1). Three had gastrointestinal stromal tumours (GISTs) and 2 had other benign tumours (lipoma 1, ectopic pancreas 1). The 30-day post-operative morbidity rate was 60% (n = 6) with mostly minor complications (Clavien grade 1 or 2). Median post-operative stay was 9 (range, 6–13) days. All ten patients were alive without recurrence after a median follow up of 26.5 months. Conclusion Limited distal duodenal resection is a feasible surgical alternative to a pancreaticoduodenectomy in carefully selected patients with benign and some malignant tumours of the third and fourth part of the duodenum., Highlights • Pancreaticodudenctomy is the usual treatment for tumours of the duodenum, which still has 30–50% morbidity. • Limited resection of the duodenum without pancreatic head resection for benign as well carefully selected malignant tumours of the distal duodenum has considerably lesser morbidity. • Cattell and Braash maneuver and mobilization of the ligament of Treitz is very helpful during such resection. • The present study suggests the feasibility of performing such limited resection with equivalent survival and oncological outcomes.

The use of adjuvant treatment has not been sufficiently investigated in duodenal adenocarcinoma. This study evaluated the effect of postoperative radiotherapy (PORT) on survival outcomes in this rare malignancy. We identified patients who were diagnosed between 2004 and 2013 in the Surveillance, Epidemiology, and End Results database. Overall survival (OS) and disease-specific survival (DSS) were analyzed before and after propensity score matching. Among the 701 eligible patients, 116 (17%) underwent PORT. There were no significant differences in OS and DSS according to receipt of PORT in the unmatched population (P = 0.982 and 0.496, respectively), whereas the propensity-matched analysis showed improved OS and DSS with PORT (P = 0.053 and 0.019, respectively). No receipt of PORT was an independent poor prognostic factor in multivariate analysis of both OS (P = 0.022) and DSS (P = 0.005). The potential survival benefits of PORT were observed in subgroups of T4 stage, larger tumor size, higher lymph node ratio, and total/radical resection. We provide useful insights into the therapeutic role of PORT in adenocarcinoma of the duodenum. Adjuvant strategy with PORT needs to be considered in locally advanced tumors.

Despite the gradual increase in the incidence of non-ampullary duodenal adenocarcinoma (NADAC), the associated characteristics and risk factors remain poorly understood. The aim of this study was to clarify the clinical characteristics and risk factors of sporadic NADAC.In this retrospective case-control study, we enrolled 156 Japanese NADAC patients with 160 lesions from our hospital's cancer registry over 13 years. The patient demographics and clinical findings were compared to 468 age- and sex-matched controls selected from our medical health checkup recipients.The ratio of men to women among the NADAC patients was 2:1, the median age was 64 years (range 31 to 90 years), the majority of the lesions were located in the second portion of the duodenum, and well-differentiated adenocarcinoma was the dominant histology. A univariate analysis revealed that smoking was a common risk factor for both men and women, whereas Helicobacter pylori (HP) infection and a history of colorectal cancer (CRC) were risk factors for the men. The multivariate analysis revealed that smoking, a history of CRC, and HP positivity were also individual risk factors for NADAC.Smoking, a history of CRC, and HP positivity were identified as risk factors for NADAC.

Background: Small bowel adenocarcinoma (SBA) is a rare malignancy that accounts for 1–2% of gastrointestinal tumours. We investigated the clinical characteristics, outcomes, and prognostic factors of primary SBA. Methods: We retrospectively analysed the characteristics and clinical courses of 205 SBA patients from 11 institutions in Japan between June 2002 and August 2013. Results: The primary tumour was in the duodenum and jejunum/ileum in 149 (72.7%) and 56 (27.3%) patients, respectively. Sixty-four patients (43.0%) with duodenal adenocarcinoma were asymptomatic and most cases were detected by oesophagogastroduodenoscopy (EGD), which was not specifically performed for the detection or surveillance of duodenal tumours. In contrast, 47 patients (83.9%) with jejunoileal carcinoma were symptomatic. The 3-year survival rate for stage 0/I, II, III, and IV cancers was 93.4%, 73.1%, 50.9%, and 15.1%, respectively. Multivariate analysis revealed performance status 3–4, high carcinoembryonic antigen, high lactate dehydrogenase (LDH), low albumin, symptomatic at diagnosis, and stage III/IV disease were independent factors for overall survival (OS). Ten patients (18.5%) with stage IV disease were treated with a combination of resection of primary tumour, local treatment of metastasis, and chemotherapy; this group had a median OS of 36.9 months. Conclusions: Although most SBA patients were diagnosed with symptomatic, advanced stage disease, some patients with duodenal carcinoma were detected in early stage by EGD. High LDH and symptomatic at diagnosis were identified as novel independent prognostic factors for OS. The prognosis of advanced SBA was poor, but combined modality therapy with local treatment of metastasis might prolong patient survival.

794 Background: Duodenal adenocarcinoma (DA) is a rare cancer with limited data regarding the pattern of disease recurrence following resection. Methods: A retrospective review of 115 patients with Stage I-III DA from 3/1994 to 6/2018, at a single high-volume cancer center was conducted. Only patients (pts) who underwent a potentially curative surgical resection (R0/R1 margins) and had a postoperative follow-up radiographic evaluation were included. Periampullary adenocarcinomas were excluded. Clinicopathologic features and patterns of recurrence were compared across cohorts. Results: Of 76 patients who met inclusion criteria, 7 (9%) were stage I, 25 (33%) stage II, and 44 (57%) stage III. Histologic grade was moderate in 58% and poor in 38%. Median age was 63 years (range, 29-84), 38% were female, and R0 resection was 97%. Neoadjuvant therapy was given to 14% and adjuvant therapy to 61%. Radiation therapy (XRT) as either adjuvant/neoadjuvant therapy was used in 27%. Median follow-up was 44 (6-293) months. Median time to recurrence was 11mo, with 84% of recurrences occurring within 2 years. Median time to local recurrence (LR) vs. distant recurrence (DR) was 11mo vs. 12mo, respectively, p = 0.42. Stage impacted recurrence rate: 0% in stage 1 vs. 50% stage 2 vs. 71% stage 3 (p = 0.002). Median time to recurrence was 16mo for stage II and 11mo for stage III (p = 0.04). In total, 4 (5%) pts had LR only, 8 (10%) had LR concurrent with DR, and 32 (42%) had DR only. Recurrence distribution was similar across stage II (LR 8%, LR+DR 15%, DR 77%) and stage III (LR 10%, LR+DR 19%, DR 71%). LR was similar in patients that received XRT (10%) compared to those who did not (9%). Most common sites of DR were peritoneal (38%), liver (33%), distant lymph nodes (12%), and lung (10%). Conclusions: The recurrence pattern for resected DA is predominantly distant metastatic disease with the majority of recurrences occurring within the first two years. Future therapies should focus on improved systemic therapy, and surveillance should be most intensive in the first two years.

Background: Biliary tree obstruction and consequent jaundice occur in 70%–90% of these patients and have important consequences mainly for the patient's quality of life, morbidity, and overall mortality. Percutaneous transhepatic biliary drainage (PTBD) is an excellent palliative procedure to drain the bile ducts in malignant obstruction. We compared left- and right-sided approach in relief of malignant obstruction. The most common causes of malignant biliary obstruction (MBO) include pancreatic adenocarcinoma, hilar cholangiocarcinoma, ampullary/duodenal adenocarcinoma, gallbladder adenocarcinoma, lymphoma, and compressive metastatic peri-portal lymph node Materials and Methods: This study was a prospective, hospital-based study performed for 2 years from 2016 to 2018. PTBD was performed either through right in 16 patients or left approach in 15 patients. Two approaches were compared in terms of clinical and technological success, changes in laboratory data, internalization and survival. Results: (1) Rate of technological success was 100%. (2) There was a significant reduction in bilirubin, alanine transaminase (ALT), and alkaline phosphatase (ALP) levels after the procedure in both the approaches, but the decrease was more in the right-sided approach. Only the decrease in bilirubin levels was statistically significant. The decrease in albumin was attributed to the progression of the disease. (3) Clinical success was seen in 93.33% and 93.75% in left- and right-sided approach. (4) Internalization was done in 86.67% patients in the left-lobe approach, while as in right-lobe approach, it was done in 93.75% patients. Conclusion: PTBD causes a significant reduction in the bilirubin level, irrespective of the amount of liver drained or the type of drainage (external/internal). In our study, the reduction in bilirubin, ALT and ALP was more in the right-lobe approach. However, only reductions in levels of bilirubin were statistically significant.

Adenocarcinoma of the small bowel accounts for only one per cent of all gastrointestinal malignancies. Duodenal adenocarcinoma accounts for half of all small bowel adenocarcinomas. The duodenum is divided into four segments: D1 (proximal horizontal 5 cm beginning with the 3-cm duodenal bulb), D2 (descending), D3 (distal horizontal), and D4 (ascending). The most common location of duodenal adenocarcinomas is the ampullary region of D2. Based on observational experience, our hypothesis was that primary adenocarcinomas arising from the mucosa of the duodenal bulb are extremely rare or possibly nonexistent. Our institutional cancer registry provided a list of patients for the years 1990 through 2012 who had small bowel cancers. Only those patients with primary adenocarcinomas of the duodenal mucosa were reviewed. Ampullary cancers arising from bile duct mucosa were specifically excluded. Medical records were abstracted to obtain patient age, sex, race, anatomic location of the tumor, disease stage (as per American Joint Committee on Cancer 7th edition staging guidelines), operation performed, and current vital status. A total of 30 patients with primary duodenal adenocarcinomas were identified. The mean age was 58 years and 17 (57%) patients were male. The tumor locations were: D2 in 26 (87%), D3 in two (7%), and D4 in two (7%). No tumors arose from D1. The patients presented with the following stages of disease: Stage 0 is in three (10%), Stage I in three (10%), Stage II in five (17%), Stage III in 15 (50%), and Stage IV in four (13%). These findings combined with a diligent review of 724 reported cases in the English language literature yielded only five clearly defined cases of adenocarcinoma arising from the mucosa of the duodenal bulb. Although a 1991 published multicenter tumor registry series of 128 localized duodenal adenocarcinomas reported 29 D1 tumors, no anatomic distinction was made between duodenal bulb and more distal D1 tumors. Earlier reports used nonanatomic divisions of the duodenum or a simple breakdown into supra-ampullary, periampullary, and infra-ampullary portions. These data beg the question as to why primary duodenal bulb adenocarcinomas are so exceedingly rare. The obvious implication is that the duodenal bulb mucosa may be physiologically, immunologically, or otherwise uniquely privileged to virtually escape oncogenic transformation. The scientific challenge and opportunity is to explore and understand the important phenomena responsible for this finding.

This article focuses on the symptomatic and diagnostic problems of primary duodenal adenocarcinoma (PDA) by way of two case reports and a literature review. An 85-year-old woman with an adenocarcinoma in the 1st duodenal portion was offered palliative care. A 90-year-old woman with an adenocarcinoma in the 3rd duodenal portion was also offered palliative care. A unique finding in the two cases reported herein is that PDA did not cause stenosis and occlusion of the lumen. As no reports of PDA without stenosis have been published so far, these cases may add to our knowledge of PDA. The diagnosis of PDA is often delayed because its symptoms may be absent until the tumor has progressed, thus leading to a delay of several months. Patients typically present with a long history of variable and vague symptoms, and many are diagnosed with advanced disease. As regards clinical manifestations, abdominal pain is the most frequent symptom. The majority of these tumors are found to have infiltrated the duodenal wall at presentation, with many being unresectable due to local and distal invasion. Esophagogastroduodenoscopy and gastrointestinal barium radiography are the main diagnostic tests for PDA, detecting 88.6 and 83.3% of tumors, respectively. In some cases, ultrasonography or computed tomography are useful for detecting PDA and determining vascular invasion.

Primary adenocarcinoma of duodenum is a very rare tumour with a prevalence of only 0.3 to 1% of among all the tumours of gastrointestinal tracts. Localised tumours, if resected have good prognosis but those with metastates entails a poor prognosis, where generally palliation may be the only feasible option. Low dose continous cytotoxic treatment or metronomic chemotherapy prevents neoangiogenesis and chemoresistance thereby, provides excellent symptom relief and palliation in many advanced heavily pretreated solid malignancies. It offers as an affordable, less toxic therapy with moderate to good efficacy. Here we report a case of a 52 year female who, presented with history of maleana, pallor and pedal edema for last 2 months. Her performance status was poor (KPS 40) and she had enlarged left supraclavicular lymph node, palpable liver and vague mass in paraumbilical region. Upper GI endoscopy revealed large ulceroproliferative growth in the D2 segment and HPE showed moderately differentiated adenocarcinoma. CT scan revealed paratracheal and retroperitoneal lymphadenopathy and bone scan revealed vertebral metastasis. Patient received oral cyclophosphamide and hematinic and vitamin support, along with radiation to spine. There was near complete clinical response, and progression free period of about 32 weeks. Thus, single agent cyclophosphamide in the present case provided near total clinical response and prolonged period of freedom from disease progression with excellent palliation of symptoms. Hence in patient of advanced and metastatic small bowel cancer, with poor performance status metronomic therapy with single agent cyclophosphamide may provide viable option both for treatment and palliation.

Background Ampullary (AMP-A) and duodenal adenocarcinomas (DA) are rare tumors. The literature regarding treatment and outcome is very limited. The objective of this project is to compare the outcomes of AMP-A and DA. Methods The records for AMP-A and DA patients between July 1995 and July 2012 at Emory University were reviewed for demographics, pathology, treatment, and survival. Survival rates were estimated by Kaplan–Meier method and compared with log-rank test. A Cox proportional hazard model was fitted to estimate the adjusted effect of AMP-A versus DA on overall survival (OS). Results Ninety-five AMP-A and 66 DA patients were identified. No significant difference between patients with DA and AMP-A was observed for age, gender, or grade. DA presented with larger tumors and higher stages. Treatment included surgery, surgery followed by adjuvant therapy or chemotherapy alone. No OS difference was observed when controlled for stage. AMP-A was sub-classified into intestinal (IAMP), pancreaticobiliary (PBAMP), and unspecified types. IAMP tended to present at a higher grade (P = 0.045) than PBAMP. No OS difference between the IAMP and PBAMP was observed. Conclusions After accounting for stage, OS was not significantly different for AMP-A and DA patients. There was no OS difference comparing PBAMP with IAMP. J. Surg. Oncol. 2014 109:556–560. © 2013 Wiley Periodicals, Inc.

Background Because of the rarity of duodenal adenocarcinoma, little is known regarding its natural history or prognostic factors for survival. We therefore evaluated surgical treatment, and prognostic factors for survival in patients with duodenal adenocarcinoma. Methods We retrospectively reviewed the medical records of patients who were diagnosed with duodenal adenocarcinoma at Asan Medical Center between December 1999 and December 2009. Results Of the 76 patients, 47 (61%) underwent surgery with curative intent and 29 (39%) underwent palliative operation. Of the former, 25 underwent pancreaticoduodenectomy (PD), 19 underwent pylorus-preserving PD, 2 underwent segmental duodenectomy and 1 underwent transduodenal excision. The median survival of the 41 patients who achieved R0 resection was 25.1 months (range 4–134 months), with overall 1-, 3- and 5-year survival rates of 80.4%, 63.4% and 60.9%, respectively. Median survival was significantly longer in patients who underwent curative resection than in those who underwent palliative surgery (28.2 versus 6.6 months, P < 0.001). Univariate analysis showed that transfusion and lymph node metastasis were related to survival, and multivariate analysis revealed that lymph node metastasis was independently associated with survival (P = 0.036). Survival differences were observed between stages of the seventh edition of the American Joint Committee on Cancer staging system. Conclusion In the absence of distant metastasis, curative resection enhances the long-term survival of patients with duodenal adenocarcinoma. Lymph node metastasis is prognostic factor of overall survival.

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.

Pancreaticoduodenectomy (PD) is the procedure of choice for periampullary cancers, which include four separate malignancies occurring in the region of the ampulla of Vater: pancreatic ductal adenocarcinoma (PDAC), ampullary cancer, distal cholangiocarcinoma, and duodenal adenocarcinoma. The evolution of PD began as a series of isolated, independent resection attempts in the late 19th and early 20th centuries, but the procedure failed to gain momentum because of its great technical difficulty and dismal outcomes. Allen O. Whipple and colleagues were the first to adopt a systematic approach to radical pancreaticoduodenal resections and by 1935, they had demonstrated the technical feasibility of the procedure. However, the highly morbid operation was nearly abandoned altogether after postoperative outcomes failed to improve in the decades following Whipple’s initial success. Fortunately, a trend in centralization and standardization of care beginning in the 1980s fueled a decrease in perioperative mortality rates that now stands at 1-2% at high volume centers.

Summary An 11-year-old Spanish Pure Bred gelding was evaluated for progressive weight loss and exercise intolerance. An ante-mortem biopsy and post-mortem histopathological examination of the duodenum revealed a duodenal adenocarcinoma. Furthermore, a medullary thyroid carcinoma of the left thyroid gland and multiple bilateral follicular thyroid adenomas were diagnosed post-mortem. Serum concentration of calcitonin in this case was markedly increased in comparison to two breed and age matched control horses. This case report shows the rare and independent occurrence of two different types of carcinomas in one horse and highlights the diagnostic value of calcitonin measurements for diagnosis of medullary thyroid carcinoma in horses.

Duodenal adenocarcinoma is very rare among the general population. The diagnosis may be delayed until advanced stages, due to the subtle and nonspecific clinical manifestations of that rare pathology. Abdominal pain, upper gastrointestinal bleeding, weight loss and biliary obstruction may be the main patient’s complaints. We present a very interesting case of an old patient with dementia, hospitalized with a clinical, laboratory and imaging state consistent with cholangitis. Conservative therapy with antibiotics and an urgent ERCP was held, during the procedure, the major papilla could not be identified due to distorted anatomy of the second and third parts of the duodenum. Torsion like appearance of the duodenum was observed. Consequentially, the patient biliary tract was drained by inserting an internal– external drain percutaneously. Following the external drainage, a successful gastroscopy was done, with successful exploration of the proximal duodenum, revealing the true cause of the bile duct obstruction; a large pedunculated polypoid mass (approx. 30 mm), in proximate to the major papilla was found as well as the distal pigtailed plastic stent with was inserted as mentioned during angiography. The mass diagnosed as duodenal adenocarcinoma in pathology. This unique case describes presentation of an aggressive rare duodenal cancer, mimicking biliary cholangitis distorting the local anatomy. Endoscopic exploration became feasible due to primary percutaneous drainage.

Adenocarcinoma of the duodenum comprises 50-70% of duodenal tumors. There is an increase in extracellular matrix metalloproteinases in this disease and it has been suggested that they play an important role in the development and pathology. Therefore, new therapeutic recommendations based on inhibitors of these enzymes, such as doxycycline, are under investigation. The cytotoxic effect of doxycycline was evaluated in the HuTu-80 duodenal adenocarcinoma cell line and its antitumor effect was determined in an immunodeficient murine model. A 10-µM (4.4 µg/ml) concentration of doxycycline was capable of causing apoptosis in 90% of the culture cells. Doxycycline was also responsible for a decrease in tumor growth and an increase in the survival of the mice with HuTu-80-cell tumors. These results suggest that doxycycline is a potential cytotoxic and antitumor agent effective in the treatment of adenocarcinoma of the duodenum.

Background Clival metastases of adenocarcinomas are exceptionally rare tumors, especially when they arise from the small intestine. We present the first, to our knowledge, report of a metastasis of a duodenal adenocarcinoma to the clivus. We also present a systematic review detailing metastasis to the clivus. Methods Studies were identified using the search terms "clival metastasis," "skull base metastasis," and "clivus" in PubMed. We collected the following information: histopathology of the primary tumor, symptoms, history, treatment, and follow-up. Results A comprehensive review of the literature yielded 56 cases. Patients developed the first symptoms of clival metastasis at a mean age of 58 years. The most common primary neoplasms originated from the prostate, kidney, or liver. Most patients presented with an isolated sixth nerve palsy or diplopia. The time interval from diagnosis of the primary tumor to symptomatic presentation of clival metastasis ranged from 2 months to 33 years. Sixteen patients initially presented with symptoms of clival metastasis without a previously diagnosed primary tumor. Survival data were available for 35 patients, of which 63% died within a range of 2 days to 31 months after initial presentation. Conclusions Most primary neoplasms originated from the prostate, kidney, and liver, which differ from previous reports on skull base metastases. Abducens nerve palsy is often the first presentation of clival metastasis. Clival metastasis from duodenal carcinoma, although very rare, should be considered in the differential diagnosis of bony lesions of the clivus in a patient with a history of duodenal adenocarcinoma.

Background and Aims Endoscopic biopsy sampling for the diagnosis of duodenal adenocarcinoma is not perfect. We investigated the accuracy of biopsy specimen diagnoses of superficial nonampullary duodenal epithelial tumors (NADETs). Methods Clinicopathologic data were retrospectively reviewed for 95 superficial NADETs from 95 patients who underwent endoscopic resection. Preoperative diagnoses based on biopsy specimens were compared with histologic diagnoses of resected specimens. Results Among the 21 lesions with biopsy specimen diagnoses of carcinoma, 12 (57.1%) were histologically diagnosed as adenomas after resection. Among the 74 lesions with biopsy specimen diagnoses of adenoma, 15 (20.3%) were histologically diagnosed as carcinomas after resection. The duodenal biopsy specimen predicted final histologic diagnoses of carcinoma with a sensitivity of 37.5% (95% CI, 18.8-59.4), specificity of 83.1% (95% CI, 72.3-91.0), accuracy of 71.6% (95% CI, 61.4-80.4), positive predictive value of 42.9% (95% CI, 21.8-66.0), and negative predictive value of 79.7% (95% CI, 68.8-88.2). Among 61 cases considered suitable for EMR, treatment modality was converted from EMR to endoscopic submucosal dissection because of the nonlifting sign in 15 cases (24.6%). Conclusions The accuracy of duodenal biopsy sampling was relatively low. Duodenal biopsy sampling may induce unexpected fibrosis. New endoscopic modalities that can improve preoperative diagnosis yield of NADETs are eagerly awaited.

A 68-year-old African-American man with a history of duodenal adenocarcinoma was found to have innumerable 1 mm hyperkeratotic depressions along his palmar creases bilaterally (figures 1⇓–3). The patient reported having these lesions since early in childhood. He also noted that all men in his family, dating back to his great-great-grandfather, had similar palmar crease pitting. He stated he was diagnosed with keratosis punctata of the palmar creases (KPPC) at an early age and failed to respond to prior therapies including topical retinoid cream …

After colorectal cancer and desmoid tumors, duodenal adenocarcinoma is the next leading cause of death in familial adenomatous polyposis (FAP) patients, but it has not been thoroughly investigated. To investigate the clinical course of duodenal neoplasia, including adenoma and cancer, we investigated 77 Japanese FAP patients treated at the National Cancer Center Hospital, Tokyo, Japan. We evaluated the clinicopathologic features, Spigelman severity score, and management of duodenal neoplasms. Data were acquired from a prospectively enrolled database. Fifty-one (66%) of the 77 FAP patients had duodenal neoplasia during this observational period, and 47 of 51 patients had extra-ampulla duodenal neoplasia; 42 (58%) had duodenal neoplasms (extra-ampulla), 4 had duodenal adenomas with high-grade dysplasia (HGD), and 1 had invasive carcinoma. Among the 45 patients (extra-ampulla) with duodenal adenoma with HGD or low-grade dysplasia, 8 (18%) patients were treated using endoscopic resection (ER). During the short observation period, ER was performed only in HGD cases. None of the patients died from duodenal neoplasia. In total, during the surveillance period, duodenal HGD was detected in 5 (63%) of 8 patients graded as Spigelman stage IV; HGD was not detected in stage 0 (n=33), I (n=0), II (n=12), or III (n=20) patients. Short-interval endoscopic surveillance and appropriate ER may help prevent duodenal invasive carcinoma. In addition, there was little development of invasive carcinoma during the follow-up. The Spigelman classification is beneficial for the risk assessment of duodenal neoplasia in Japanese FAP patients.

Duodenal adenocarcinoma, a very rare malignant gastrointestinal tumor, mainly metastasizes via the lymphatic system. Metastases from duodenal adenocarcinomas to the breast are very uncommon. A 31-year-old woman presented at our department with a left breast tumor. She had a past medical history of duodenal adenocarcinoma. Physical examination on admission confirmed a 2.5-cm-diameter tumor in the outer lower quadrant of the left breast. Computed tomography (CT) examination showed a soft lesion with tissue-like density and enlarged axillary lymph nodes. Local excision was performed to remove the breast lesion. The findings of cytologic, histologic, and immunohistochemistry examination indicated a breast metastasis from the previous duodenal adenocarcinoma. The patient was treated with palliative chemotherapy. Metastases from duodenal adenocarcinoma to the breast are rare. The diagnosis depends on medical history, imaging, and pathologic examination including immunohistochemistry. An accurate diagnosis is important to avoid unnecessary surgery.

Background Lymph node (LN) metastasis in patients with duodenal adenocarcinoma is associated with poor prognosis; however, the optimal extent of LN assessment and the interaction between LN assessment and adjuvant systemic therapy is poorly understood. Methods Resected non-metastatic duodenal adenocarcinoma patients ( n = 1743) were identified in the National Cancer Database (1998–2011). Logistic regression analysis identified covariates associated with LN metastasis. The influence of increasing LN cut-off points on overall survival (OS) was analysed using the log-rank test and Cox proportional hazards modelling. Adjuvant chemotherapy (AC) and surgery alone cohorts were matched (1:1) by propensity scores based on the likelihood of nodal metastasis or survival hazard on Cox modelling. OS in the matched cohort was compared by Kaplan–Meier estimates. Results LN metastases were present in 865 (49.6%) patients. Increasing LN assessment was associated with an increased likelihood of nodal involvement ( P = 0.008). In node-negative patients, increasing LN assessment was associated with a decreased risk of death, with the largest actuarial survival differences observed for ≥15 LN (hazard ratio [HR] 0.63, 95% confidence interval [CI] 0.48–0.82, P = 0.001). In the propensity score-matched cohort of node-negative patients, AC was associated with non-significant improvements in 5-year actuarial (66.1% versus 58.7%, HR 0.79, 95% CI 0.53–1.18, P = 0.249), and did not vary by adequacy of LN counts ( P = 0.305; ≥15 LNs: HR 0.90, 95% CI 0.35–2.30, P = 0.900). Conclusions The extent of LN identification has prognostic significance in resected node-negative duodenal adenocarcinoma, but cannot be implicated in the selection of node-negative patients for AC.

Background: Small intestinal tumors are rare accounting for less than 5% of all gastrointestinal (GI) tumors. Primary duodenal carcinoma is very rare among GI tumors and usually presents with non-specific signs and symptoms rendering it difficult for early diagnosis. Importance of early diagnosis arises from the fact that the only curative treatment is radical surgical resection before metastasis. Whipple’s procedure remains the most accepted surgical intervention for resectable tumors. Case presentation: An 82-year-old female presented to the emergency department with a one-day history of epigastric pain and nausea without vomiting. Abdominal examination revealed tenderness in the epigastrium and right upper quadrant. Abdominal ultrasound and laboratory tests were consistent with acute cholecystitis and biliary pancreatitis. She underwent laparoscopic cholecystectomy and intra operative cholangiogram which revealed a dilated common bile duct with questionable cholelithiasis. Post-operative computed tomography (CT) scan revealed duodenal thickening at the periampullary region suggesting a mass without evidence of metastasis. Esophagogastroduodenoscopy (EGD) confirmed a periampullary mass. Histopathological examination of biopsied tissue revealed moderately differentiated adenocarcinoma. The patient underwent pancreaticoduodenectomy (Whipple’s procedure) and had a long, complicated post-operative course and eventually died. Conclusions: Duodenal adenocarcinoma (DAC) is rare and may present with concomitant acute cholecystitis and biliary pancreatitis.

MicroRNAs have potential as diagnostic cancer biomarkers. The aim of this study was (1) to define microRNA expression patterns in formalin-fixed parafin-embedded tissue from pancreatic ductal adenocarcinoma, ampullary adenocarcinoma, normal pancreas and chronic pancreatitis without using micro-dissection and (2) to discover new diagnostic microRNAs and combinations of microRNAs in cancer tissue. The expression of 664 microRNAs in tissue from 170 pancreatic adenocarcinomas and 107 ampullary adenocarcinomas were analyzed using a commercial microRNA assay. Results were compared with chronic pancreatitis, normal pancreas and duodenal adenocarcinoma. In all, 43 microRNAs had higher and 41 microRNAs reduced expression in pancreatic cancer compared with normal pancreas. In all, 32 microRNAs were differently expressed in pancreatic adenocarcinoma compared with chronic pancreatitis (17 higher; 15 reduced). Several of these microRNAs have not before been related to diagnosis of pancreatic cancer (eg, miR-492, miR-614, miR-622). MiR-614, miR-492, miR-622, miR-135b and miR-196 were most differently expressed. MicroRNA profiles of pancreatic and ampullary adenocarcinomas were correlated (0.990). MicroRNA expression profiles for pancreatic cancer described in the literature were consistent with our findings, and the microRNA profile for pancreatic adenocarcinoma (miR-196b-miR-217) was validated. We identified a more significant expression profile, the difference between miR-411 and miR-198 (P=2.06 × 10(-54)) and a diagnostic LASSO classifier using 19 microRNAs (sensitivity 98.5%; positive predictive value 97.8%; accuracy 97.0%). We also identified microRNA profiles to subclassify ampullary adenocarcinomas into pancreatobiliary or intestinal type. In conclusion, we found that combinations of two microRNAs could roughly separate neoplastic from non-neoplastic samples. A diagnostic 19 microRNA classifier was constructed which without micro-dissection could discriminate pancreatic and ampullary adenocarcinomas from chronic pancreatitis and normal pancreas with high sensitivity and accuracy. Ongoing prospective studies will evaluate if these microRNA profiles are useful on fine-needle biopsies for early diagnosis of pancreatic cancer.

Purpose: Little information is available on genetic and epigenetic changes in duodenal adenocarcinomas. The purpose was to identify possible subsets of duodenal adenocarcinomas based on microsatellite instability (MSI), DNA methylation, mutations in the KRAS and BRAF genes, clinicopathologic features, and prognosis. Experimental Design: Demographics, tumor characteristics, and survival were available for 99 duodenal adenocarcinoma patients. Testing for KRAS and BRAF mutations, MSI, MLH1 methylation, and CpG island methylator phenotype (CIMP) status was conducted. A Cox proportional hazard model was built to predict survival. Results: CIMP+ was detected in 27 of 99 (27.3%) duodenal adenocarcinomas and was associated with MSI (P = 0.011) and MLH1 methylation (P < 0.001), but not with KRAS mutations (P = 0.114), as compared with CIMP− tumors. No BRAF V600E mutation was detected. Among the CIMP+ tumors, 15 (55.6%) were CIMP+/MLH1-unmethylated (MLH1-U). Kaplan–Meier analysis showed that tumors classified by CIMP, CIMP/MLH1 methylation status, or CIMP/MSI status could predict overall survival (OS; P = 0.047, 0.002, and 0.002, respectively), whereas CIMP/MLH1 methylation status could also predict time-to-recurrence (TTR; P = 0.016). In multivariate analysis, CIMP/MLH1 methylation status showed a significant prognostic value in both OS (P < 0.001) and TTR (P = 0.023). Patients with CIMP+/MLH1-U tumors had the worst OS and TTR. Conclusions: Our results showed existence of CIMP in duodenal adenocarcinomas. The combination of CIMP+/MLH1-U seems to be independently associated with poor prognosis in patients with duodenal adenocarcinomas. This study also suggests that BRAF mutations are not involved in duodenal tumorigenesis, MSI, or CIMP development. Clin Cancer Res; 18(17); 4743–52. ©2012 AACR.

Background Increasing incidences of malignant small bowel tumours have been reported, but data from European populations are limited. This study aimed to clarify the incidence patterns of malignant small bowel tumours in Sweden. Methods Patients with a first and primary malignant small bowel tumour were identified from the Swedish Cancer Register during the study period 1960–2009. Sex-specific and age-standardised incidence rates of these tumours were calculated by their anatomical location and histological type in different time periods. Figures were plotted to show the proportions and incidence rates over time, and joinpoint loglinear regression models were estimated to assess any time trends. Results A total of 6604 patients with malignant small bowel tumours were identified. The age-standardised incidence of all malignant small bowel tumours increased from 14.2 to 19.7 per 1,000,000 person-years during the study period. The incidence of duodenal cancer increased more than 3-fold (from 1.6 to 5.4 per 1,000,000 person-years), which was mainly expained by a dramatical rising trend of adenocarcinoma of the duodenum (from 0.7 to 4.2 per 1,000,000 person-years). Malignant tumours of small bowel with unspecified anatomical locations showed a slight increase (from 7.0 to 7.9 per 1,000,000). The incidence of small bowel tumours in other locations or of other histological types was more stable. Conclusions The incidence of small bowel malignancies has increased during the period 1960–2009. Among the specific types of small bowel cancer, a particularly rapid increase was found for duodenal adenocarcinoma.

BACKGROUND: Primary duodenal adenocarcinoma is a rare entity with an uncertain biologic behaviour whose diagnosis is usually achieved by endoscopy and biopsy. However, it lacks specific symptoms and its diagnosis may be delayed with poor prognosis. Villous adenomas are considered as premalignant lesions with high risk malignant transformation. METHODS: We report a rare case of a patient with two synchronous adenocarcinomas, one of the first portion of the duodenum as a villous tumor and the other in cecum colon. The patient submitted successfully to pacreatoduodenectomy and right hemicolectomy, while postoperatively received also adjuvant chemotherapy. RESULTS: Seventeen months after the operations the patient is alive with no evidence of recurrent or metastatic disease. CONCLUSIONS: The radical resection of primary duodenal adenocarcinoma is considered the treatment of choice, which should be accompanied by adjuvant chemotherapy preventing a recurrence at distant sites.

Duodenal adenocarcinoma is a rare cancer usually studied as a group with periampullary or small bowel adenocarcinoma; therefore, its natural history is poorly understood.Patients with duodenal adenocarcinoma were identified from a single-institution pancreaticoduodenectomy database. Patients with adenocarcinoma arising from the ampulla of Vater were excluded. Univariate and multivariate analyses were performed to identify clinicopathologic variables associated with survival and recurrence after resection.From 1984 to 2006, a total of 122 patients with duodenal adenocarcinoma underwent pancreaticoduodenectomy. Overall survival after resection was 48% at 5 years and 41% at 10 years. Five-year survival decreased as the number of lymph nodes involved by metastasis increased from 0 to 1-3 to ≥ 4 (68%, 58%, 17%, respectively, P0.01) and as the lymph node ratio increased from 0 to0-0.2 to0.2-0.4 to0.4 (68%, 57%, 14%, 14%, respectively, P0.01). Lymph node metastasis was the only independent predictor of decreased survival in multivariate analysis. Recurrence after resection was predominantly distant (81%). Adjuvant chemoradiation did not decrease local recurrence or prolong overall survival; however, patients who received chemoradiation more commonly had nodal metastasis (P = 0.03).The prognostic significance of both the absolute number and ratio of involved lymph nodes emphasizes the need for adequate lymphadenectomy to accurately stage duodenal adenocarcinoma. The mostly distant pattern of recurrence underscores the need for the development of effective systemic therapies.

Familial adenomatous polyposis (FAP) is a hereditary multisystem disease dominantly characterized by diffuse colorectal adenomas and near 100% colorectal cancer risk. In addition to colorectal cancer, multiple extracolonic sites are affected by malignant and benign changes. Other leading causes of death for FAP patients include desmoid disease and duodenal adenocarcinoma. Benign lesions, such as lipomas, fibromas, sebaceous and epidermoid cysts, osteomas, and congenital hypertrophy of the retinal pigment epithelium, are also common. Cancers of the thyroid, pancreas, liver, and adrenal gland all occur but are much less frequent. As the lifespan of FAP patients has increased because of improved guidelines for surveillance and surgical prevention and treatment of colorectal cancer, the prevalence of these extracolonic conditions has become more clinically relevant. Clinicians need to be aware of the full spectrum of diseases in FAP to provide appropriate and systematic surveillance and intervention.

This single-institution experience retrospectively reviewed the outcomes in 21 patients with primary duodenal adenocarcinoma. Twelve patients underwent curative surgery, and 9 patients underwent palliative surgery at the Chiba University Hospital. The maximum follow-up period was 8650 days. All pathologic specimens from endoscopic biopsy and surgical specimens were reviewed and categorized. Twelve (57.1%) patients underwent curative surgery (R0): 4 pancreaticoduodenectomies (PD), 4 pylorus-preserving PDs (PpPD), 2 local resections of the duodenum and 2 endoscopic mucosal resections (EMR). Palliative surgery was performed for 9 patients (42.9%) following gastro-intestinal bypass. The median cause-specific survival times were 1784 days (range 160–8650 days) in the curative surgery group and 261 days (range 27–857 days) in the palliative surgery group (P = 0.0003, log-rank test). The resectability of primary duodenal adenocarcinoma was associated with a smaller tumor size, a lower degree of tumor depth invasiveness, and less spread to the lymph nodes and distant organs.

We present a case of a healthy 59-year-old woman who presented for a capsule endoscopy to evaluate melaena and iron deficiency anaemia. She had previously underwent an oesophagogastroduodenoscopy and colonoscopy at an outside institution which were unremarkable. Capsule endoscopy showed an ulcerated, bleeding lesion likely in the duodenum. Differential diagnosis included adenocarcinoma, carcinoid tumour, lymphoma, gastrointestinal stromal tumour and metastatic disease. A push enteroscopy was performed after which showed an ulcerated mass in the third portion of the duodenum. Biopsies confirmed adenocarcinoma. Computed tomography of the abdomen showed no signs of distant metastasis and the patient was referred to surgery for evaluation. The patient underwent a pancreaticoduodenectomy, with resection of the mass and negative lymph nodes in all nine that were removed (T3N0). The patient was classified as stage II duodenal adenocarcinoma. Duodenal adenocarcinoma is a rare but clinically significant cause of small bowel bleeding.

我々は，まれとされる十二指腸第4部に発生した十二指腸癌に対し根治切除を行った4例を経験した．いずれも全周性狭窄を来した進行癌であり，うち1例は同時性肝転移を認めた．肝転移の症例はリンパ節郭清を伴う十二指腸空腸部分切除術と肝切除術を2期的に行った．それ以外の3例は，幽門輪温存膵頭十二指腸切除術を行った．病理組織学的に，T3N0M0（Stage IIA）が1例，T3N1M0（Stage IIIA）が2例，T3N0M1（Stage IV）が1例であった．リンパ節転移を認めた2例は，いずれもNo.14リンパ節への転移であった．Stage IIAの症例は術後11年，Stage IIIAの2例は術後5年無再発生存中である．同時性肝転移の症例は術後12か月に肺転移で死亡した．第4部原発の十二指腸癌に対し膵頭十二指腸切除術を行うか局所切除術を行うかは議論の分かれるところであるが，その至適術式について文献的考察を加え報告する．

Duodenal adenocarcinoma (DA) is an aggressive malignancy. It represents the most common adenocarcinoma of the small intestine, with the majority arising in segment D2 (1-3). The incidence of DA however is rare; it represents less than 1% of all gastrointestinal cancers (4-6). DA usually presents with vague symptoms and can be difficult to diagnose until a patient develops gastrointestinal bleeding or obstruction which frequently results in delays of diagnosis (7). These factors influence the lack of high-quality evidence on outcomes for those with DA, relative scarcity of definitive treatment recommendations, and resulting overall modest prognosis.