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Pathology International Apr 2016Cancer of the ampulla of Vater is rare, though it has various histological types and its histogenesis is fascinating in view of the anatomically complex nature of the...
Cancer of the ampulla of Vater is rare, though it has various histological types and its histogenesis is fascinating in view of the anatomically complex nature of the ampulla. Fetal gut-like adenocarcinoma, usually found in the stomach, can also develop in the ampullary region in extremely rare cases. Here we present a case of ampullary adenocarcinoma with enteroblastic and neuroendocrine differentiation. A 55-year-old woman presented with an epigastric pain. Endoscopic examination revealed a 2-cm submucosal tumor-like lesion in the ampulla. The surgical specimen showed that an exposed protruding type of tumor appeared as a well-demarcated whitish-yellow solid mass. Microscopically, the tumor had proliferated in the common channel and invaded the duodenal submucosa with mucosal lesion of intestinal-type adenocarcinoma. The main tumor consisted of three different histological types showing transitional areas: adenocarcinoma with enteroblastic differentiation (ENT), neuroendocrine carcinoma (NEC), and well differentiated adenocarcinoma (WEL). Morphologically the ENT resembled fetal gut and immunohistochemically expressed SALL4 and glypican 3. The WEL was positive for CK20 and CDX2, revealing an intestinal-type phenotype. AFP and HepPar1 were not evident in any part of the lesion. We speculated this tumor had arisen from intestinal-type adenocarcinoma of the common channel and acquired enteroblastic and neuroendocrine differentiation during growth.
Topics: Adenocarcinoma; Ampulla of Vater; Biomarkers, Tumor; Carcinoma, Neuroendocrine; Cell Differentiation; Common Bile Duct Neoplasms; Duodenal Neoplasms; Endoscopy, Digestive System; Female; Humans; Middle Aged; Pancreatic Neoplasms; Pancreaticoduodenectomy
PubMed: 27016918
DOI: 10.1111/pin.12402 -
JNMA; Journal of the Nepal Medical... 2016Whipple's Pancreaticoduodenectomy has increasingly been used as an appropriate resectional procedure for tumors of the periampullary region which are pancreatic,...
INTRODUCTION
Whipple's Pancreaticoduodenectomy has increasingly been used as an appropriate resectional procedure for tumors of the periampullary region which are pancreatic, periampullary, ampullary and biliary tumors. Our aim was to study the distribution and histopathologic features of these tumors and to examine local trends of periampullary neoplasms resected with a PD.
METHODS
A descriptive study was conducted in the department of Pathology, Kathmandu Medical College Teaching Hospital from July 2013 to June 2016.
RESULTS
Thirty five patients underwent Whipple's Pancreaticoduodenectomy procedure during a period of 36 months from July 2013 to June 2016. Malignant tumor was present in 31 (88.57%) cases where as four cases (11.43%) harboured benign lesions. Periampullary mixed carcinoma was the predominant tumor (34.28%) followed by periampullary duodenal (20%), ampullary (14.28%), pancreatic adenocarcinoma (11.42%) and distal cholangiocarcinoma (5.71%). There was no significant difference in tumor size among periampullary, ampullary, pancreatic and biliary carcinomas. Ampullary carcinomas were predominantly well differentiated (80%) where as the other tumors were mostly moderately differentiated. Lymphovascular and perineural invasion varied in different tumor types. Four pancreatic adenocarcinomas showed lymphovascular and perineural invasion. Adequate surgical margin clearance was achieved in most of the cases except in one case each of periampullary duodenal carcinoma and distal cholangiocarcinoma and two cases of pancreatic adenocarcinoma..
CONCLUSIONS
Pancreaticoduodenectomy specimen requires thorough histopathological evaluation. Pathologists should also be aware of possibility of a benign diagnosis in PD specimens which have been resected presuming malignancy based on clinical judgement and radiological data.
Topics: Adenocarcinoma; Ampulla of Vater; Bile Duct Neoplasms; Cholangiocarcinoma; Common Bile Duct Neoplasms; Duodenal Neoplasms; Humans; Pancreatic Neoplasms; Pancreaticoduodenectomy
PubMed: 28029673
DOI: No ID Found -
Oncotarget Jun 2017Non-ampullary duodenal adenoma with activation of Wnt/β-catenin signalling is common in familial adenomatous polyposis (FAP) patients, whereas sporadic non-ampullary...
Non-ampullary duodenal adenoma with activation of Wnt/β-catenin signalling is common in familial adenomatous polyposis (FAP) patients, whereas sporadic non-ampullary adenoma is uncommon. The adenoma-carcinoma sequence similar to colon cancer is associated with duodenal tumors in FAP, but not always in sporadic tumors. We obtained 37 non-ampullary duodenal tumors, including 25 adenomas and 12 adenocarcinomas, were obtained from biopsies and endoscopic resections. We performed immunohistochemistry for β-catenin, the hallmark of Wnt activation, and aldehyde dehydrogenase 1 (ALDH1), a putative cancer stem cell marker. In non-ampullary lesions, abnormal nuclear localization of β-catenin was observed in 21 (84.0%) of 25 adenomas and 4 (33.3%) of 12 adenocarcinomas. In the proximal duodenum, nuclear β-catenin was less frequent in both adenomas and adenocarcinomas. Gastric duodenal metaplasia (GDM) was observed only in the proximal duodenum. All adenomas with GDM were the gastric foveolar and pyloric gland types, and showed only membranous β-catenin. The intestinal-type adenomas had nuclear β-catenin in the proximal and distal duodenum. ALDH1-positive cells were more frequent in adenocarcinomas than adenomas. Nuclear β-catenin accumulation frequently occurred in ALDH1-positive cells in adenoma, but not in adenocarcinoma. In the non-ampullary proximal duodenum, Wnt/β-catenin pathway activation was more closely associated with adenomas than adenocarcinomas, and while it might cooperate with ALDH1 in adenoma, it does not in adenocarcinoma. The pathogenesis thus may differ between sporadic adenoma and adenocarcinoma of non-ampullary duodenal lesions, especially in the proximal and distal duodenum.
Topics: Adenocarcinoma; Adenoma; Adult; Aged; Aldehyde Dehydrogenase 1 Family; Duodenal Neoplasms; Duodenum; Female; Humans; Immunohistochemistry; Isoenzymes; Male; Metaplasia; Middle Aged; Retinal Dehydrogenase; Wnt Signaling Pathway; beta Catenin
PubMed: 28467793
DOI: 10.18632/oncotarget.17051 -
The American Journal of Surgical... Dec 2021Nonampullary duodenal adenomas (NADAs) develop sporadically or in the setting of a hereditary syndrome such as familial adenomatous polyposis (FAP). Although they are...
Nonampullary Duodenal Adenomas in Familial Adenomatous Polyposis and Sporadic Patients Lack the DNA Content Abnormality That Is Characteristic of the Adenoma-Carcinoma Sequence Involved in the Development of Other Gastrointestinal Malignancies.
Nonampullary duodenal adenomas (NADAs) develop sporadically or in the setting of a hereditary syndrome such as familial adenomatous polyposis (FAP). Although they are thought to progress into duodenal adenocarcinomas via an adenoma to carcinoma sequence similar to colorectal cancer, limited data suggested that they may be biologically dissimilar to colorectal adenomas. The clinicopathologic features of 71 patients diagnosed with NADAs (37 FAP and 34 sporadic) were analyzed. From the 71 patients, 89 NADA biopsies (42 FAP and 47 sporadic) were evaluated by DNA flow cytometry. Eighty-two samples showed low-grade dysplasia, and 7 demonstrated high-grade dysplasia (HGD). Twenty-one low-grade adenomas of the ileal pouch (n=19) and jejunum (n=2) from 15 FAP patients who underwent total proctocolectomy were also analyzed by DNA flow cytometry. The FAP patients were more likely to be younger (mean: 28 y) and have multifocal disease (92%) than the sporadic patients (66 y and 24%, respectively) (P<0.001). Most NADAs presented as polypoid lesions (87%) in the duodenal bulb and/or second portion of the duodenum (94%). Sporadic NADAs (mean: 2.4 cm) were significantly larger than FAP-related NADAs (1.3 cm) (P=0.005). Three (4%) patients (2 sporadic and 1 FAP) had high-grade NADAs at the first endoscopy, while the remaining 68 (96%) patients had low-grade dysplasia. Two additional sporadic and 1 FAP patients developed HGD on follow-up. Although the overall detection rate of advanced neoplasia (either HGD or adenocarcinoma) was similar between the FAP (n=5; 14%) and sporadic groups (n=4; 12%) (P=1.000), 3 FAP patients (all with Spigelman stage III to IV) developed adenocarcinoma in the duodenum (n=2) or in the ileal pouch (n=1) within a mean follow-up time of 76 months, while no adenocarcinoma was found in the sporadic group. Of the 37 FAP patients, 29 (78%) had a history of total proctocolectomy, and 15 (52%) developed low-grade adenomas in the ileal pouch with (n=2) or without (n=13) jejunal involvement (vs. 0% in the sporadic patients, P<0.001). All 15 patients had ≥Spigelman stage II. Aneuploidy was detected in only 1 (1%) sporadic NADA with HGD, whereas the remaining 109 duodenal, ileal pouch, and jejunal adenomas showed normal DNA content. The overall 3-, 9-, and 15-year detection rates of adenocarcinoma (in the duodenum and ileal pouch) in all NADA patients were 1.4%, 7.2%, and 18.8%, respectively. Three-, 9-, and 15-year detection rates of adenocarcinoma in the FAP patients were 2.7%, 9.7%, and 22.6%, respectively, while these rates remained at 0% in the sporadic patients. In conclusion, FAP-related NADAs have distinct clinicopathologic features compared with their sporadic counterpart. However, the vast majority of both FAP-related and sporadic NADAs (99%) lack the DNA content abnormality that is characteristic of the typical adenoma-carcinoma sequence involved in other gastrointestinal carcinogenesis. Although adenocarcinoma is more likely to develop in FAP patients with a high adenoma burden, probably due to the higher likelihood that some advanced lesions are missed endoscopically, FAP-related and sporadic NADAs may have a comparable risk of developing advanced neoplasia on a per-adenoma basis.
Topics: Adenocarcinoma; Adenomatous Polyposis Coli; Adolescent; Adult; Aged; Aged, 80 and over; Aneuploidy; Biopsy; Child; DNA, Neoplasm; Disease Progression; Duodenal Neoplasms; Female; Flow Cytometry; Humans; Male; Middle Aged; Neoplasm Grading; Risk Assessment; Risk Factors; Young Adult
PubMed: 34138799
DOI: 10.1097/PAS.0000000000001754 -
Journal of Surgical Oncology Mar 2022For patients with periampullary adenocarcinoma (PAC), pancreatoduodenectomy (PD) provides the best survival. Surgery on a subset of patients is aborted during PD. We...
BACKGROUND
For patients with periampullary adenocarcinoma (PAC), pancreatoduodenectomy (PD) provides the best survival. Surgery on a subset of patients is aborted during PD. We analyzed these patients.
METHODS
Patients who underwent laparotomy for planned PD for PAC were identified (2006-2019). From operative notes, we identified the subset with intraoperative decision to abort. Patient, treatment, and outcome data were analyzed. The subset with pancreatic ductal adenocarcinoma (PDAC) was analyzed for survival.
RESULTS
Only 6.7% (n = 55/819) of cases were aborted. Majority 78% (n = 43) had pathologically-confirmed diagnoses at time of surgery, and 18.2% (n = 10) received preoperative chemotherapy. Reasons for aborted PD included: distant metastases (65.5%, n = 36) and local invasion (34.5%, n = 19). Of patients with metastatic disease, 75% (n = 27) had liver metastases. Eighty-nine percent (n = 49) of patients underwent at least one palliative bypass procedure and 81.8% (n = 45) had both gastric and biliary bypass. Patients with computed tomography (CT) scans before surgery more commonly had missed metastatic disease (79.2% CT compared to 54.8% magnetic resonance imaging [MRI], χ = 3.54, p = 0.059). In PDAC, 61.4% (n = 27/44) were aborted for metastatic disease and 38.7% (n = 17/44) for local invasion. Median overall survival for all PDAC patients after aborted PD was 334 days.
CONCLUSION
Majority of pancreatoduodenectomies for periampullary adenocarcinoma are done to completion. Liver metastases is the most common reason for aborting. Preoperative MRI may help identify hepatic metastases.
Topics: Adenocarcinoma; Aged; Ampulla of Vater; Common Bile Duct Neoplasms; Duodenal Neoplasms; Female; Follow-Up Studies; Humans; Liver Neoplasms; Male; Pancreatic Neoplasms; Pancreaticoduodenectomy; Prognosis; Prospective Studies; Survival Rate
PubMed: 35015302
DOI: 10.1002/jso.26781 -
Advances in Therapy Jun 2021Brunner's gland hamartoma is a benign tumor of the duodenum, but has malignant potential with a very low risk of progression into adenocarcinoma. It is uncommon with a... (Review)
Review
Brunner's gland hamartoma is a benign tumor of the duodenum, but has malignant potential with a very low risk of progression into adenocarcinoma. It is uncommon with a frequency of less than 1.0% among the primary tumors of the small intestine. In addition, its clinical manifestations are nonspecific, etiology remains unclear, and treatment strategy needs to be further refined. This literature review mainly discusses the epidemiology, clinical features, possible etiology and pathogenesis, diagnostic methods, malignant potential, treatment, and prognosis of Brunner's gland hamartoma.
Topics: Adenocarcinoma; Brunner Glands; Duodenum; Hamartoma; Humans
PubMed: 33914269
DOI: 10.1007/s12325-021-01750-6 -
HPB : the Official Journal of the... Jul 2023Pancreatoduodenectomy (PD) is recommended in fit patients with a resectable ampullary adenocarcinoma (AA). We aimed to identify predictors of five-year...
Predictors of actual five-year survival and recurrence after pancreatoduodenectomy for ampullary adenocarcinoma: results from an international multicentre retrospective cohort study.
BACKGROUND
Pancreatoduodenectomy (PD) is recommended in fit patients with a resectable ampullary adenocarcinoma (AA). We aimed to identify predictors of five-year recurrence/survival.
METHODS
Data were extracted from the Recurrence After Whipple's (RAW) study, a multicentre retrospective study of PD patients with a confirmed head of pancreas or periampullary malignancy (June 1st, 2012-May 31st, 2015). Patients with AA who developed recurrence/died within five-years were compared to those who did not.
RESULTS
394 patients were included and actual five-year survival was 54%. Recurrence affected 45% and the median time-to-recurrence was 14 months. Local only, local and distant, and distant only recurrence affected 34, 41 and 94 patients, respectively (site unknown: 7). Among those with recurrence, the most common sites were the liver (32%), local lymph nodes (14%) and lung/pleura (13%). Following multivariable tests, number of resected nodes, histological T stage > II, lymphatic invasion, perineural invasion (PNI), peripancreatic fat invasion (PPFI) and ≥1 positive resection margin correlated with increased recurrence and reduced survival. Furthermore, ≥1 positive margin, PPFI and PNI were all associated with reduced time-to-recurrence.
CONCLUSIONS
This multicentre retrospective study of PD outcomes identified numerous histopathological predictors of AA recurrence. Patients with these high-risk features might benefit from adjuvant therapy.
Topics: Humans; Pancreaticoduodenectomy; Retrospective Studies; Ampulla of Vater; Adenocarcinoma; Common Bile Duct Neoplasms; Duodenal Neoplasms; Neoplasm Recurrence, Local; Pancreatic Neoplasms
PubMed: 37149485
DOI: 10.1016/j.hpb.2023.03.010 -
The American Surgeon Nov 2016The surgical management of duodenal pathology is challenging because of its retroperitoneal position and shared blood supply with the pancreas. We present three types of... (Review)
Review
The surgical management of duodenal pathology is challenging because of its retroperitoneal position and shared blood supply with the pancreas. We present three types of limited resection of the duodenum for the removal of superficial or small nonampullary duodenal (NADL) lesions, and also a review of the English literature regarding management, such as endoscopic resection and limited duodenal resection. Ten cases underwent limited resections of the duodenum for superficial or small NADL lesions from 2011 to 2015. Pancreas-preserving segmental duodenectomy was performed in three cases, local full-thickness resection was performed in three and transduodenal submucosal dissection was performed in four. One patient experienced pancreatic fistula as a postoperative complication. Postoperative pathological diagnosis were adenoma (n = 2), mucosal adenocarcinomas (n = 5), and neuroendocrine tumor (n = 3). Surgical margin was negative in all cases, and no patient has experienced postoperative recurrence or metastasis. Limited resections of the duodenum were feasible and safe procedures for patients with superficial or small NADL lesions. Laparoscopic surgery may be considered in treatment for these tumors. However, the optimal surgical management for superficial or small nonampullary duodenal lesions remains controversial.
Topics: Adenocarcinoma; Adenoma; Adult; Aged; Aged, 80 and over; Carcinoma, Neuroendocrine; Dissection; Duodenal Neoplasms; Duodenum; Female; Humans; Male; Medical Illustration; Middle Aged; Pancreatic Fistula; Pancreaticoduodenectomy; Postoperative Complications
PubMed: 28206943
DOI: No ID Found -
Journal of Oncology Pharmacy Practice :... Oct 2015The scientific literature on adenocarcinoma of the ampulla (papilla) of Vater suggests that it either represents a distinct entity or is more closely related to small... (Review)
Review
The scientific literature on adenocarcinoma of the ampulla (papilla) of Vater suggests that it either represents a distinct entity or is more closely related to small bowel adenocarcinoma than to the biliary malignancies. The ambiguity surrounding this rare cancer has kindled research exploring its immunohistochemistry aspects and gene expression profiling. While the basis of management for resectable disease remains surgical intervention, the role of adjuvant chemotherapy is not clear. A recent large phase 3 clinical trial conducted in patients with resected ampulla of Vater adenocarcinoma favored adjuvant chemotherapy over observation alone. The standards of therapy for the advanced small bowel adenocarcinoma and biliary cancer are fluoropyrimidine derivatives and gemcitabine-based combinations, respectively. In addition, new biologic and targeted agents may enhance clinical results seen in this cancer type. Therefore, diligently designed clinical trials are necessary to establish its optimal treatment strategies. We describe herein a patient with ampulla of Vater adenocarcinoma who had an exceptional response to fluoropyrimidine-based chemotherapy. We further include a discussion reviewing the clinicopathologic aspects of this neoplasm as well as focus on currently available and future therapeutic options.
Topics: Adenocarcinoma; Aged; Ampulla of Vater; Antineoplastic Combined Chemotherapy Protocols; Chemotherapy, Adjuvant; Common Bile Duct Neoplasms; Female; Fluorouracil; Humans; Treatment Outcome
PubMed: 24906539
DOI: 10.1177/1078155214538086 -
World Journal of Gastroenterology Jun 2017To investigate the range of pathologies treated by pancreas preserving distal duodenectomy (PPDD) and present the outcome of follow-up.
AIM
To investigate the range of pathologies treated by pancreas preserving distal duodenectomy (PPDD) and present the outcome of follow-up.
METHODS
Neoplastic lesions of the duodenum are treated conventionally by pancreaticoduodenectomy. Lesions distal to the major papilla may be suitable for a pancreas-preserving distal duodenectomy, potentially reducing morbidity and mortality. We present our experience with this procedure. Selective intraoperative duodenoscopy assessed the relationship of the papilla to the lesion. After duodenal mobilisation and confirmation of the site of the lesion, the duodenum was transected distal to the papilla and beyond the duodenojejunal flexure and a side-to-side duodeno-jejunal anastomosis was formed. Patients were identified from a prospectively maintained database and outcomes determined from digital health records with a dataset including demographics, co-morbidities, mode of presentation, preoperative imaging and assessment, nutritional support needs, technical operative details, blood transfusion requirements, length of stay, pathology including lymph node yield and lymph node involvement, length of follow-up, complications and outcomes. Related published literature was also reviewed.
RESULTS
Twenty-four patients had surgery with the intent of performing PPDD from 2003 to 2016. Nineteen underwent PPDD successfully. Two patients planned for PPDD proceeded to formal pancreaticoduodenectomy (PD) while three had unresectable disease. Median post-operative follow-up was 32 mo. Pathologies resected included duodenal adenocarcinoma ( = 6), adenomas ( = 5), gastrointestinal stromal tumours ( = 4) and lipoma, bleeding duodenal diverticulum, locally advanced colonic adenocarcinoma and extrinsic compression ( = 1 each). Median postoperative length of stay (LOS) was 8 d and morbidity was low [pain and nausea/vomiting ( = 2), anastomotic stricture ( = 1), pneumonia ( = 1), and overwhelming post-splenectomy sepsis ( = 1, asplenic patient)]. PPDD was associated with a significantly shorter LOS than a contemporaneous PD series [PPDD 8 (6-14) d PD 11 (10-16) d, median (IQR), = 0.026]. The 30-d mortality was zero and 16 of 19 patients are alive to date. One patient died of recurrent duodenal adenocarcinoma 18 mo postoperatively and two died of unrelated disease (at 2 mo and at 8 years respectively).
CONCLUSION
PPDD is a versatile operation that can provide definitive treatment for a range of duodenal pathologies including adenocarcinoma.
Topics: Adenocarcinoma; Adult; Aged; Aged, 80 and over; Algorithms; Ampulla of Vater; Anastomosis, Surgical; Blood Transfusion; Case-Control Studies; Catheterization; Duodenal Neoplasms; Duodenoscopy; Duodenum; Female; Follow-Up Studies; Gastrointestinal Stromal Tumors; Humans; Lymph Nodes; Male; Middle Aged; Organ Sparing Treatments; Pancreas; Pancreaticoduodenectomy; Postoperative Period; Treatment Outcome
PubMed: 28694665
DOI: 10.3748/wjg.v23.i23.4252