Primary small bowel adenocarcinoma: current view on clinical features, risk and prognostic factors, treatment and outcome.

Small bowel adenocarcinoma (SBA) is a rare but increasing cause of gastrointestinal malignancy, being both a diagnostic and therapeutic challenge. The goal of treatment is margin negative resection of a lesion and local lymphadenectomy, followed by modern adjuvant chemotherapy combinations in selected cases. Improved outcomes in patients with SBA are encouraging, but elucidation of mechanisms of carcinogenesis and risk factors as well as improved treatment for this malignancy is very needed.

FEATURES OF THE ADAPTATION STATUS OF THE PATIENTS WITH DIFFERENCES IN THE PREVALENCE OF CANCER PROCESS IN BILIOPANCREATICODUODENAL ZONE AND IN THE VOLUME OF A SURGICAL INTERVENTION.

The differences in the type and tension of the general unspecific adaptational reactions in cancer patients with varying prevalence of the process in biliopancreaticoduodenal zone and volume of surgical intervention were revealed. It was shown prognostic significance of the numerical score of the type and tension of the adaptational reaction a day after pancreatoduodenal resection for the development of postoperative complications.

[The clinical features of 16 cases of primary adenocarcinoma of the third portion of duodenum].

OBJECTIVE: To summarize the clinical features of the third portion of duodenum (PATD) for improving the understanding of PATD. METHODS: Sixteen cases with PATD in Peking Union Medical College Hospital(PUMCH) were retrospectively analyzed. RESULTS: The most common symptoms of PATD were upper abdominal pain (12/16) , vomiting (9/16) and distention (7/16).On average, the disease had progressed 5.5 months (including 2.5 months of diagnostic workup) before the diagnosis was established. Patients with pathologically poorly differentiated PATD had shorter course of disease (6.5 vs 16.6 months, P = 0.56) and lower chance of cancer-directed surgery (1/8 vs 6/8, P = 0.04) than those with well differentiated PATD. The diagnostic rate was 11/14 by CT scan while only 2/7 by upper gastrointestinal radiography. Three cases were misdiagnosed as superior mesenteric artery syndrome by barium examination. CONCLUSIONS: PATD should be considered in patients presenting upper abdominal symptoms with negative gastro endoscopy and barium examination.Overall, CT scan plays a pivotal role in diagnosing PATD. Making a correct diagnosis timely can improve the outcome of PATD patients, particularly, in those with poorly differentiated pathology.

A Rare Presentation of Choledochoduodenal Fistula Due to Ovarian Cancer Metastasis.

Choledochoduodenal fistula (CDF) is an abnormal communication between the common bile duct and the duodenum. It accounts for about 5% to 25% of the total biliary fistulas and is usually due to a perforated duodenal ulcer, choledocholithiasis, and complications secondary to tuberculosis or could be iatrogenic. Primary intrabilliary tumors usually cause obstructive jaundice and rarely biliary metastasis arising from other organs like colon, breast, and lungs can cause obstructive jaundice. There has been a case report of metastasis from ovarian cancer to the major papilla of the duodenum but no reported cases of it causing a CDF. We report a rare case of an 83-year-old female with ovarian cancer who developed a metastatic lesion to the duodenum eventually resulting in a CDF.

Metastatic clear cell endometrial carcinoma: an unusual cause of a common clinical presentation.

A 60-year-old woman presented with melena for 2 weeks. She had undergone hysterectomy and bilateral salpingo-oophorectomy to treat clear cell endometrial carcinoma 10 months before the presentation. She was anaemic and tachycardic; abdominal CT scan revealed a large duodenal mass. Her gastrointestinal bleed was not amenable to endoscopic intervention, so she had emergent laparotomy, pancreaticoduodenectomy with duodenal mass excision. Histopathology confirmed that the duodenal mass was a metastatic deposit from her clear cell endometrial cancer. Postoperatively, she was frail and chose hospice care and she died 90 days postoperatively. Clear cell endometrial cancer is a rare subtype of endometrial cancer, that has a worse prognosis compared with the more common endometrioid subtype. The duodenum is a rare site for metastatic endometrial cancer, and we report this case to alert clinicians to the possibility of metastases to the small intestine in patients with clear cell endometrial cancer.

Features of duodenal cancer in patients with familial adenomatous polyposis.

BACKGROUND & AIMS: Most patients with familial adenomatous polyposis (FAP) develop duodenal adenomas; duodenal cancer is a major cause of mortality in this patient group. We reviewed cases of duodenal cancer in patients with FAP to identify factors that determine long-term cancer risk. METHODS: Twenty FAP patients (12 male) were identified from a registry database search. Data from registry and medical notes and endoscopic and histopathologic reports were evaluated. RESULTS: Of the cancers that developed in these patients, 11 were ampullary and 9 were duodenal. The median age at cancer diagnosis was 53 years. Seventeen patients died (median age at death, 57 y; median survival from diagnosis, 11 mo); the cause of death was metastatic or duodenal/ampullary cancer in 14 patients. Fifteen patients presented symptomatically (including 3 interval cancers while on surveillance). Two were diagnosed at surveillance and 3 were diagnosed during surgery performed for endoscopic features of advanced benign disease. Duodenal cancers were associated with a significantly lower mean colonic polyp count than ampullary cancers (496 +/- 282 vs 1322 +/- 735; P = .025); there appeared to be familial clustering of this cancer. When endoscopic data were available (n = 11 of 20), all ampullary cancers arose from ampullas greater than 1 cm. The Spigelman stage did not predict risk of ampullary cancer but did predict duodenal cancer (median stage 2 vs stage 4 for duodenal cancer). CONCLUSIONS: Once cancer arises in patients with FAP, prognosis is poor, so cancer prevention should be the main goal. Surveillance intervals should reflect both Spigelman staging and ampullary disease.

Evaluation of duodenal perfusion by contrast-enhanced ultrasonography in dogs with chronic inflammatory enteropathy and intestinal lymphoma.

BACKGROUND: Contrast-enhanced ultrasonography (CEUS) can be used to evaluate intestinal perfusion in healthy dogs. It is helpful for diagnosing and monitoring inflammatory bowel disease in humans and could be useful for dogs with chronic intestinal diseases. OBJECTIVES: To examine duodenal perfusion in dogs with chronic inflammatory enteropathy (CIE) and intestinal lymphoma. ANIMALS: Client-owned dogs with CIE (n = 26) or intestinal lymphoma (n = 7) and dogs with gastrointestinal signs but histopathologically normal duodenum (controls, n = 14). METHODS: In this cross-sectional study, dogs with CIE were classified into remission (n = 16) and symptomatic (n = 10) groups based on clinical scores determined at the time of CEUS. The duodenum was scanned after IV injection of Sonazoid® (0.01 mL/kg). CEUS-derived perfusion parameters, including time-to-peak, peak intensity (PI), area under the curve (AUC), and wash-in and wash-out rates were evaluated. RESULTS: The PI was significantly higher in the symptomatic CIE group (median (range); 105.4 (89.3-128.8) MPV) than in the control group (89.9 (68.5-112.2) MPV). The AUC was significantly higher in the symptomatic CIE group (4847.9 (3824.3-8462.8) MPV.sec) than in the control (3448.9 (1559.5-4736.9) MPV.sec) and remission CIE (3862.3 (2094.5-6899.0) MPV.sec) groups. The PI and clinical score were positively correlated in the CIE group. No significant differences in perfusion parameters were detected between the lymphoma and CIE groups or the lymphoma and control groups. CONCLUSIONS AND CLINICAL IMPORTANCE: The PI and AUC can detect duodenal inflammation and hence are potentially useful for excluding a diagnosis of CIE.

Total robotic surgery for pancreaticoduodenectomy combined with rectal cancer anterior resection: A case report and literature review.

RATIONALE: Synchronous double malignancies, including carcinoma of the ampulla of Vater and rectal carcinoma, are generally uncommon occurrences in the gastrointestinal tract. PATIENT CONCERNS: The present study report a case of a 37-year-old man who was incidentally found to suffer from carcinoma of the ampulla of Vater and rectal carcinoma. DIAGNOSES: The duodenoscopy was performed and revealed an ulcerated and bulky ampulla of Vater, the biopsy from which revealed a moderate-differentiated adenocarcinoma, A local hospital colonoscopy confirmed a tumor located in rectal 7 cm from the anal margin and biopsy-confirmed poorly differentiated adenocarcinoma. INTERVENTIONS: About such patient treatment, both open and laparoscopic surgery are restricted because of operation complexity, large injury, and poor cosmetic effect. surgery performed using Da Vinci robotic surgical system (DVSS). OUTCOMES: No evidence of recurrence or relapses was found in the first year after surgery. LESSONS: Although sporadic double malignancies are uncommon, they should be considered when evaluating cancer patients. Complex surgery performed by robotic surgery may became surgeon's preferred treatment modality.

Primary duodenal cancer: case report.

Primary carcinoma of the duodenum is uncommonly encountered. This is a report of a 64-year-old diabetic/hypertensive who was admitted in our unit with six months history of upper abdominal pain, vomiting on and off and weight loss of greater than 10 kgs. Endoscopy revealed complete obstruction of the third part of the duodenum. Lesion biopsy revealed moderately differentiated adenocarcinoma and associated duodenitis. A staging CT scan showed thickening of the duodenal wall over a span of six centimetres, luminal narrowing, mucosal irregularity and multiple para-vascular large nodes some greater than or equal to two centimetres. Palliative bypass surgery was suggested as the preferred mode of treatment. He underwent cholecysto-jejunostomy/jejunojejunostomy to palliate biliary and intestinal obstruction.

Duodenal plexiform fibromyxoma as a cause of obscure upper gastrointestinal bleeding: A case report.

RATIONALE: We are reporting the first-to our knowledge-case of duodenal Plexiform Fibromyxoma causing obscure upper gastrointestinal bleeding. PATIENT CONCERNS: Plexiform fibromyxoma triggered recurrent upper gastrointestinal bleeding episodes in a 63-year-old man who remained undiagnosed, despite multiple hospitalizations, extensive diagnostic workups and surgical interventions (including gastrectomies), for almost 17 years. DIAGNOSES-INTERVENTIONS: During hospitalization for the last bleeding episode, an upper gastrointestinal endoscopy revealed an intestinal hemorrhagic nodule. The lesion was deemed unresectable by endoscopic means. An abdominal computerized tomography disclosed no further lesions and surgery was decided. The lesion at operation was found near the edge of the duodenal stump and treated with pancreas-preserving duodenectomy (1st and 2nd portion). OUTCOMES: Postoperative recovery was mainly uneventful and a 20-month follow-up finds the patient in good health with no need for blood transfusions.Plexiform fibromyxomas stand for a rare and widely unknown mesenchymal entity. Despite the fact that they closely resemble other gastrointestinal tumors, they distinctly vary in clinical management as well as the histopathology. Clinical awareness and further research are compulsory to elucidate its clinical course and prognosis.

Inherent pacemaker function of duodenal GIST.

Gastrointestinal stromal tumours (GIST) are thought to derive from interstitial cells of Cajal (ICCs), which are putative pacemaker cells for gut motility. Isolated cells were obtained by enzymatic treatment of human duodenum GIST tissue having a frequent gain-of-function gene mutation. After cell culturing, c-Kit immunoreactivity was preserved and the cells developed long processes. Whole cell patch clamp recordings revealed voltage-dependent outward currents, without transient inward currents. Intracellular Ca(2+) measurements showed oscillation-like spontaneous activity in some GIST cells. RT-PCR revealed expression of ion channels (Kv1.1, Kv1.6 and KCNH2; IP3R1, and IP3R2; TRPC1, 3, 6 and 7; Cx43), which have been suggested to play important roles in pacemaker activity. However, SCN5A, a TTX-resistant Na(+) channel known to be expressed in human ICCs, was below detectable levels. These data suggest that GIST cells appear to preserve some, but not all ionic mechanisms underlying pacemaker activity in ICC.

Delayed gastric emptying is associated with pylorus-preserving but not classical Whipple pancreaticoduodenectomy: a review of the literature and critical reappraisal of the implicated pathomechanism.

Pylorus-preserving pancreaticoduodenectomy (PPPD) is nowadays considered the treatment of choice for periampullary tumors, namely carcinoma of the head, neck, or uncinate process of the pancreas, the ampulla of Vater, distal common bile duct or carcinoma of the peri-Vaterian duodenum. Delayed gastric emptying (DGE) comprises one of the most troublesome complications of this procedure. A search of the literature using Pubmed/Medline was performed to identify clinical trials examining the incidence rate of DGE following standard Whipple pancreaticoduodenectomy (PD) vs PPPD. Additionally we performed a thorough in-depth analysis of the implicated pathomechanism underlying the occurrence of DGE after PPPD. In contrast to early studies, the majority of recently performed clinical trials demonstrated no significant association between the occurrence of DGE with either PD or PPPD. PD and PPPD procedures are equally effective operations regarding the postoperative occurrence of DGE. Further randomized trials are required to investigate the efficacy of a recently reported (but not yet tested in large-scale studies) modification, that is, PPPD with antecolic duodenojejunostomy.

Surveillance of duodenal adenomas in familial adenomatous polyposis reveals high cumulative risk of advanced disease.

PURPOSE: The development of high-grade dysplasia (HGD) on duodenal or jejunal adenomas and of late-stage (stage IV) duodenal polyposis are major clinical events for familial adenomatous polyposis (FAP) patients. Our aim was to determine their respective frequency, risk factors, and cumulative risk. PATIENTS AND METHODS: A prospective, optimized, endoscopic surveillance protocol was applied to 58 FAP patients in a university hospital. The number, size, and histology of duodenojejunal polyps were assessed, and the Spigelman's score was calculated at each endoscopy. Cox regression and linear regression analysis were used to determine risk factors for HGD development and the cumulative risk of stage IV duodenal polyposis, respectively. RESULTS: During a median (+/- standard deviation) follow-up of 47.9 +/- 15.6 months, 35 patients with at least two consecutive examinations had 107 duodenojejunal examinations. The Spigelman's score increased in 21 patients (60.0%), and HGD developed in 12 patients (34.2%). High initial Spigelman's score (> 7 points), but not age or APC mutation site, was a risk factor for HGD development. Estimated cumulative risk of developing stage IV duodenal polyposis was of 42.9% at age 60 (95% CI, 35.7% to 50.0%) and 50.0% at age 70 (95% CI, 42.9% to 57.1%). CONCLUSION: This prospective series shows a higher duodenal polyposis progression rate and cumulative risk of late-stage (stage IV) duodenal polyposis in FAP patients compared with previous series. These results suggest that current modalities for surveillance and management of these patients need revision.

Diagnosis of functioning pancreaticoduodenal neuroendocrine tumors.

Functioning pancreaticoduodenal neuroendocrine tumors (PD-NETs) are popular in a textbook, but they are still unfamiliar to a general clinician, and delay of diagnosis or misdiagnosis has been reported even today. It is a consensus that sporadic functioning PD-NET is cured only by surgical resection. So, early detection and early resection is the gold standard for the treatment of functioning PD-NET. Functioning PD-NETs in patients with multiple endocrine neoplasia type 1 (MEN 1) are often multiple. You should check about MEN 1 whenever you encountered multiple PD-NET. They are diagnosed in younger age than sporadic cases. In most cases they are accompanied with numerous microscopic or macroscopic nonfunctioning P-NETs, which are potentially metastatic and the most common cause of death in MEN 1 patients.

Perioperative Enteral Immunonutrition Modulates Systemic and Mucosal Immunity and the Inflammatory Response in Patients With Periampullary Cancer Scheduled for Pancreaticoduodenectomy: A Randomized Clinical Trial.

OBJECTIVES: Nutritional deficiencies and immune dysfunction in cancer patients may contribute to postoperative septic morbidity. This trial compared the effects of perioperative enteral immunonutrition (EIN) versus standard enteral nutrition (SEN) on systemic and mucosal immunity in patients undergoing pancreaticoduodenectomy for periampullary cancer. METHODS: Thirty-seven patients were randomized (EIN, n = 17; SEN, n = 20) to receive feed for 14 days preoperatively and 7 days postoperatively. Mediators of systemic immunity (interleukin 1α, tumor necrosis factor α, lymphocytes subsets, and complement components) and of mucosal immunity in duodenal biopsies, nutritional markers and parameters were evaluated. RESULTS: The groups were comparable for demographics, the concentrations of mediators of systemic and mucosal immunity at time of recruitment, and for the duration and amount of feed received. Preoperative EIN rather than SEN was associated with significant reductions in plasma tumor necrosis factor α and total hemolytic complement. Enteral immunonutrition-fed patients had significantly higher total lymphocyte count on the third postoperative day and significantly greater rise in CD4/CD8 ratio from day 3 to day 7 postoperatively compared with SEN-fed patients. CONCLUSIONS: The perioperative administration of EIN rather than SEN is associated with a favorable modulation of the inflammatory response and enhancement of systemic immunity in patients undergoing pancreaticoduodenectomy for periampullary cancer.

Duodenal obstruction: diagnosis by gastroduodenal manometry.

Establishing the diagnosis of adenocarcinoma of the distal duodenum is often difficult based on findings on barium radiography and routine endoscopy of the upper gastrointestinal tract. A characteristic manometric pattern of simultaneous, prolonged contractions of the small intestine after a meal has been associated with mechanical obstruction of the small intestine. Herein we describe a 68-year-old woman who had a 4-month history of nausea, vomiting, and weight loss. Findings on endoscopy of the upper gastrointestinal tract and a barium contrast examination of the stomach, duodenum, and small bowel were interpreted as normal. A radionuclide scan suggested mildly delayed gastric emptying. Gastroduodenal manometry revealed high-amplitude, simultaneous contractions in the third and fourth portions of the duodenum but not in the jejunum, findings highly suggestive of a mechanical obstruction in the distal duodenum. At laparotomy, an obstructing adenocarcinoma of the duodenum proximal to the ligament of Treitz was resected. Subtle abnormalities were detected retrospectively on the barium contrast study of the small bowel. In patients with features suggestive of intestinal obstruction, gastroduodenal manometry may be helpful in distinguishing mechanical causes from pseudo-obstruction.

Zollinger-Ellison syndrome. A new look at regression of gastrinomas.

Of 44 patients with the Zollinger-Ellison syndrome treated at our institution, nine appeared to have undergone "regression" of their gastrinomas. Six of the nine patients had sporadic gastrinomas and became permanently eugastrinemic following excision of nodal metastases and total gastrectomy (n = 4), antrectomy (n = 1), or pancreatoduodenectomy (n = 1) (mean survival, 13 years). The other three patients had Zollinger-Ellison syndrome as part of the multiple endocrine adenopathy type 1 syndrome and became temporarily eugastrinemic after total gastrectomy (mean survival, 11 years). Occult submucosal duodenal-wall microgastrinomas (mean size, 3.0 mm) were found to have been serendipitously excised in four patients. Long-term follow-up of these nine patients, as well as of six other patients described in the literature, demonstrates that excision of occult duodenal-wall gastrinomas provides a plausible explanation for the phenomenon of apparent regression of primary gastrinomas and the eugastrinemia that may follow total gastrectomy.

Adenocarcinoma of the duodenum: factors influencing long-term survival.

This single-institution retrospective analysis reviews the management and outcome of patients with surgically treated adenocarcinoma of the duodenum. Between February 1984 and August 1996, fifty-five patients with adenocarcinoma of the duodenum underwent surgery at The Johns Hopkins Hospital. Univariate analysis was performed to identify possible prognostic indicators. Curative resection was performed in 48 patients (87%): 35 of these patients (73%) underwent a pancreaticoduodenectomy (PD), whereas 27% (n = 13) underwent a pancreas-sparing duodenectomy (PSD). Patients undergoing PD were comparable to those undergoing PSD with respect to demographic factors, presenting symptoms, and tumor pathology. The remaining 13% of patients (n = 7) were deemed unresectable at the time of surgery and underwent biopsy and/or palliative bypass. PD was associated with an increase in postoperative complications when compared to PSD (57% vs. 30%), but this difference was not statistically significant. One perioperative death occurred following PD (mortality 2.9%). The overall 5-year survival rate for the 48 patients undergoing potentially curative resection was 53%. Negative resection margins (P <0.001), PD (P <0.005), and tumors in the first and second portions of the duodenum (P <0.05) were favorable predictors of long-term survival by univariate analysis. Nodal status, tumor diameter, degree of differentiation, and the use of adjuvant chemoradiation therapy did not influence survival. These data support an aggressive role for resection in patients with adenocarcinoma of the dueodenum

Heterotopic pancreas. Review of a 26 year experience.

A retrospective review of 37 patients (22 men and 15 women) with histologically verified heterotopic pancreas treated at the department of surgery of the University of California at Los Angeles Medical Center from 1959 to 1985 was carried out. There were 31 adults (mean age 50 years) and 6 children (mean age 2.8 years). The majority of lesions were in the stomach, duodenum, and jejunum. One was found inside a duplicated stomach. Symptomatic lesions were confined to the gastroduodenal region and were larger, with frequent mucosal ulceration. Upper gastrointestinal contrast roentgenograms were sensitive tools for detection (87.5 percent of patients) and diagnosis (71.4 percent of patients) of these lesions. Endoscopy should be performed whenever epigastric pain is the presenting symptom. Resection of the tissue-bearing segment of small intestine is advisable when encountered incidentally at operation. In the absence of endoscopic biopsy confirmation, we recommend surgical exploration and frozen section histopathologic study for all symptomatic patients. Limited local excision has been shown to be a safe and adequate procedure for patients with these congenital anomalies.