Littérature scientifique sur le sujet « PRIMARY DUODENAL ADENOCARCINOMA »

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.

Aims and Background Primary duodenal adenocarcinoma is a rare tumor with a poorly defined natural history and prognostic factors. It presents with nonspecific symptoms, and for this reason the diagnosis is often delayed. It is a serious problem for the surgeon because of the difficulty in obtaining an early diagnosis and standardizing basic tenets for an appropriate surgical approach. The aim of this work was to conduct a review of the literature analyzing the points most frequently debated about this pathology. Methods and Study Design A bibliographic search was carried out on the main search engines to find studies regarding duodenal adenocarcinoma, published in English, from January 1992 to January 2007. Results A total of 19 articles was selected. Results concerning symptoms, location of the tumor, diagnostic examinations, surgical treatment, histopathology of the tumor, survival and follow-up were obtained and discussed. Conclusions All patients who are medically fit to undergo surgery should be given the option of aggressive resection regardless of tumor size, tumor invasion or appearance of positive lymph nodes. Hopefully, an early diagnosis will correlate with improved long-term survival.

Primary adenocarcinoma of the fourth portion of the duodenum (D-IV) is reported infrequently than other parts of the duodenum. Its diagnosis is often late because of non-specific symptoms and signs. We encountered a 48-year-old male patient who was diagnosed as duodeno-duodenal intussusception, underwent segmental duodenal resection with duodenojejunal anastomosis and confirmed as adenocarcinoma of D-IV. He received adjuvant chemotherapy and is doing well at 1 year of follow-up. This report describes about the rare case of isolated adenocarcinoma of the D-IV presented as intussusception which is never reported before and successfully treated by segmental resection of the duodenum and jejunum.

Natural history of duodenal adenocarcinoma is not well known. Although extremely rare, the incidence of primary small bowel adenocarcinoma has been increasing. Primary duodenal cancer is a very rare, which has accounted for only 0.3% of all gastrointestinal cancers. Isolated ovarian metastasis from duodenal carcinoma is extremely rare entity. We report a case of duodenal carcinoma with isolated bilateral ovarian metastasis in a 39-year-old female patient managed surgically with Whipple’s procedure with bilateral oophorectomy. She presented with complaints of dyspepsia and intermittent vomiting since 1½ years. History of anorexia and weight loss present. Upper gastrointestinal endoscopy was suggestive of circumferential ulcerated friable lesion at duodenum (D1-2) region and histopathology suggestive of moderately differentiated adenocarcinoma. Subsequent imaging studies revealed 6×5 cm duodenal mass in D1 and D2 without IHBRD with right ovarian mass. She underwent staging laparoscopy with open classic Whipple’s procedure with bilateral oophorectomy was done. Intraoperatively 6×6 cm mass involving first and second part of duodenum involving head of pancreas was found with 3×3 cm right ovarian mass. Post-operative period was uneventful and she discharged on POD6. Final biopsy was suggestive of moderately differentiated adenocarcinoma of duodenum with bilateral ovarian metastasis and she received adjuvant chemotherapy. Now at 1 year, on regular follow up she is completely asymptomatic and imaging showed no recurrent disease. Isolated ovarian metastasis may not be a contraindication for radical surgery in selected group of patients with duodenal carcinoma.

Abstract 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.

Primary adenocarcinoma of duodenum is a very rare and aggressive malignancy, found in middle or elderly age group and usually arising in the second portion of the duodenum, followed by third or fourth. Cancers of the first portion of the duodenum, especially the duodenal bulb are extremely rare. The causative factors for duodenal adenocarcinoma are dietary factors, ingestion of alcohol, coffee and use of tobacco. It may occur from duodenal polyps present in familial polyposis or may be associated. In young age it is associated with polyposis syndromes or with Crohn’s disease affecting small bowel. Very few reports discuss the diagnosis and treatment of such malignancy and very few cases have been reported in young age less than 30years. We are reporting a case of 22 years old gentleman who presented to us with history of recurrent abdominal pain and vomiting. After evaluation it was diagnosed as adenocarcinoma of third and fourth part of duodenum without any associated syndromes or inflammatory bowel diseases and was managed by surgical resection and chemotherapy.

L'adenocarcinoma del duodeno e' una rara patologia che rappresenta circa lo 0.3-0.5 % di tutte le neoplasie maligne del tratto gastroenterico. Malgrado il duodeno rappresenta meno del 10 % della totale lunghezza dell'intestino tenue, circa il 45% delle neoplasie dell'intestino tenue si presentano nel duodeno. Essendo i sintomi dei questa malattia non specifici la diagnosi di carcinoma del duodeno e' molto difficile e molto spesso tardiva. Tuttavia il carcinoma del duodeno ha una sopravvivenza a 5 anni per tumori resecati tra il 25 e il 75%. Nell'ultimo decennio differenti studi hanno identificato diversi fattori prognostici associati alla sopravvivenza. Questi studi hanno mostrato che il grado istologico, le dimensioni del tumore,il coinvolgimento transmurale e dei linfonodi possono influenzare la sopravvivenza. L'obbiettivo di questa tesi e' di migliorare la conoscenza di specifici fattori prognostici associati alla sopravvivenza in pazienti affetti da adenocarinoma primario del duodeno. Tramite uno studio retrospettivo , 37 pazienti con diagnosi di adenocarcinoma del duodeno tra il 1989 e il 2009 sono stati identificati tramite il database del dipartimento di Chirurgia B dell'Universita' di Verona. Dall'analisi dei dati e' risultato che le complicanze operatorie possono influenzare in maniera significativa la sopravivenza in pazienti affetti da adenocarcinoma del duodeno.
Primary Adenocarcinoma of the duodenum is a rare condition, accounting between 0, 3% and 0, 5% of all gastrointestinal malignancies . Despite the duodenum is representing less than 10 % of the total length of the small bowel, this organ is the site of between 25% and 45% of the small bowel cancers 4. However cancer of the duodenum appeared to be more frequent in the proximity of the Ampulla of Vater periampullary area , about 45 % of them demonstrated to arise in third and in fourth por-tions of this organ. Since the symptoms of this cancer are non-specific and similar to other benign conditions, the diagnosis is often difficult and delayed. Nonetheless, duodenal carcinoma has a reported 5-year survival rate for resected tumours between 25% and 75% which is significantly better than that for cancer of the ampulla (46%) and the head of pancreas (10%) . In the last decade different studies evaluated the correlations between clinical, pathological and treatment variables in order to identified specific prognostic factors associated with survival . Those recent studies showed that histological grade, transmural involvement, tumour size, lymph node involvement and distant metastasis can influence the survival in patients with duodenal adenocarcinoma. However, due to a low incidence of this disease in the general population, the debate on prognostic factors in duodenal adenocarcinoma is still open. Therefore, questions have been raised especially on the prognostic significance of the absolute number and ratio of involved lymph nodes. A recent study has led an increased interest on perineural invasion as single independent prognostic factor for patients with primary duodenal cancer . Aims of research The aim of this thesis was to improve understanding of specific prognostic factors associated to survival in primary duodenal adenocarcinoma. A retrospective review of 37 patients diagnosed with primary duodenal adenocarcinoma between 1989 and 2009 was performed at Hepato-pancreatico-biliary department, General Surgery B, Verona University, Borgo Roma Hospital. Data were acquired then analysed for impact on recurrence and 5-year overall survival rate. From this retrospective study, , the development of postoperative complications is an additional prognostic factor after potentially curative resection for primary duodenal adenocarcinoma. This emphasized the need for centralization to high-volume centres, where an appropriate postoperative care can be delivered.

Approximately 90% of primary pancreatic tumours are ductal adenocarcinomas, which usually occur in the head and neck of the pancreas. This chapter discusses the role of different imaging modalities in the staging, assessment of operability, and therapeutic response assessment of pancreatic ductal adenocarcinoma (PDAC). Current role of radiotherapy for PDAC is summarized. Most guidelines recommend consideration for radical chemo-radiotherapy or stereotactic ablative body radiotherapy (SABR) if there is at least stable localized disease after 3–4 months of chemotherapy. Palliative radiotherapy is given for pain relief or bleeding into the duodenum. The role of different imaging modalities in the planning for conventional radiotherapy and SABR techniques is highlighted. Pancreatic neuroendocrine tumours are uncommon and are discussed in in Chapter 19.

Pancreatic cancer, most commonly in the form of a solid ductal adenocarcinoma, accounts for 3% of all cancers but ranks in the top five leading causes of cancer deaths in most developed countries, reflecting the fact that it has a very poor prognosis (median survival 6–9 months). It is a disease of older age (85% of patients >65 years), and commoner in smokers. Most patients present with locally advanced or metastatic disease, often with obstructive jaundice. Pain is unusual in early disease, but when present is characteristically described as ‘gnawing’, ever present, and frequently radiating into the back. Weight loss is commonly due to anorexia as a result of jaundice or pain, but can occasionally be the only presenting symptom. Serum biochemistry will typically show elevated bilirubin and a cholestatic picture of liver enzymes, with particular elevation of alkaline phosphatase and γ‎-glutamyl transferase. Transabdominal ultrasonography is usually the primary investigation in a patient with jaundice and can detect pancreatic tumours greater than 2 cm in size or hepatic metastases with a diagnostic accuracy of 75%, but identifies smaller tumours much less reliably. The essential investigations for the diagnosis and staging of pancreatic cancer are contrast-phased CT scan and occasionally MRI. The only curative treatment for pancreatic cancer is surgical excision. This is technically feasible in up to 20% patients at presentation, but even after careful selection almost 40% of these will have positive microscopic resection margins, and overall postoperative survival is only around 10% at 5 years, the remainder experiencing metastatic disease in the peritoneum, liver, or lungs. Adjuvant chemotherapy with gemcitabine can double the 5-year survival rate. Palliative management may require biliary stenting for jaundice, duodenal stenting (or surgical bypass) for gastric outlet obstruction, pain control, and palliative chemotherapy.

Gastrin is a gastrointestinal hormone, produced predominantly by the G cells of the gastric antrum and duodenum, although small amounts of gastrin have been isolated in the pituitary and some vagal nerve fibres. The biologically active forms of gastrin include carboxy-amidated gastrin-17 and carboxy-amidated gastrin-34, which bind mainly to the cholecystokinin (CCK)-2 receptor. The main role of amidated gastrin is the stimulation of gastric acid secretion by regulation of histamine release from the gastric enterochromaffin-like (ECL) cells, while it may also have a trophic effect on gastric mucosa. There is evidence that the precursor forms of gastrin, such as progastrin and glycine-extended gastrin, are also of biological importance, binding to a separate CCK-C receptor. These precursor may induce cellular and tumour growth and they are implicated in several cancers, such as colon and pancreatic adenocarcinomas. Gastrinomas represent a group of functional pancreatic neuroendocrine tumours, characterized by autonomous release of gastrin by the tumour cells, which results in symptoms not only due to the tumour growth per se, but also to gastric acid hypersecretion. In 1955, at the annual meeting of American Surgical Association, Robert M. Zollinger and Edwin H. Ellison presented a study entitled Primary Peptic Ulcerations of the Jejunum Associated with Islet Cell Tumour of the Pancreas. They proposed a new clinical syndrome of: (1) ulceration in unusual locations in the upper gastrointestinal tract or recurrent ulcerations; (2) gastric acid hyperseretion; and (3) non-β‎ islet cell tumours of the pancreas. However, the potent gastric secretagogue for the Zollinger–Ellison syndrome was not identified until 1960, when Rodney Gregory and Hilda Tracy of the University of Liverpool discovered that the extract from the pancreas of a patient with Zollinger–Ellison syndrome was the hormone gastrin. Thus, these pancreatic tumours were termed ‘gastrinomas’.