ABSTRACT
We present a combination of distal cholangiocarcinoma of the intrapancreatic common bile duct and intraductal papillary mucinous tumor associated with ductal adenocarcinoma of the pancreatic tail. This clinical case is unique. When analyzing the literature, we found no any case of similar primary multiple malignant tumor. Importantly, final diagnosis of simultaneous malignant pancreatobiliary neoplasia is possible only via intraoperative biopsy after adequate morphological dissection and research of resected organ complex including molecular genetic analysis due to identical histological and immunohistochemical picture of ductal neoplasia.
Subject(s)
Adenocarcinoma, Mucinous , Carcinoma, Pancreatic Ductal , Cholangiocarcinoma , Neoplasms, Multiple Primary , Humans , Cholangiocarcinoma/diagnosis , Cholangiocarcinoma/surgery , Cholangiocarcinoma/pathology , Neoplasms, Multiple Primary/surgery , Neoplasms, Multiple Primary/pathology , Neoplasms, Multiple Primary/diagnosis , Carcinoma, Pancreatic Ductal/surgery , Carcinoma, Pancreatic Ductal/diagnosis , Carcinoma, Pancreatic Ductal/pathology , Adenocarcinoma, Mucinous/diagnosis , Adenocarcinoma, Mucinous/surgery , Adenocarcinoma, Mucinous/pathology , Male , Bile Duct Neoplasms/surgery , Bile Duct Neoplasms/diagnosis , Bile Duct Neoplasms/pathology , Pancreatic Neoplasms/surgery , Pancreatic Neoplasms/diagnosis , Pancreatic Neoplasms/pathology , Common Bile Duct Neoplasms/surgery , Common Bile Duct Neoplasms/diagnosis , Common Bile Duct Neoplasms/pathology , Common Bile Duct/surgery , Common Bile Duct/pathology , Middle Aged , Pancreatectomy/methods , Treatment Outcome , Aged , Tomography, X-Ray Computed/methodsABSTRACT
BACKGROUND: Management of ampullary tumors (AT) is challenging because of a low level of scientific evidence. This document is a summary of the French intergroup guidelines regarding the management of AT, either adenoma (AA) or carcinoma (AC), published in July 2023, available on the website of the French Society of Gastroenterology (SNFGE) (www.tncd.org). METHODS: A collaborative work was conducted under the auspices of French medical, endoscopic, oncological and surgical societies involved in the management of AT. Recommendations are based on recent literature review and expert opinions and graded in three categories (A, B, C), according to quality of evidence. RESULTS: Accurate diagnosis of AT requires at least duodenoscopy and EUS. All patients should be discussed in multidisciplinary tumor board before treatment. Surveillance may only be proposed for small AA in familial adenomatous polyposis. For AA, endoscopic papillectomy is the preferred option only if R0 resection can be achieved. When not possible, surgical papillectomy should be considered. For AC beyond pT1a N0, pancreaticoduodenectomy is the procedure of choice. Adjuvant monochemotherapy (gemcitabine, 5FU) may be proposed. For aggressive tumors (pT3/T4, pN+, R1, poorly differentiated AC, pancreatobiliary differentiation) with high risk of recurrence, 6 months polychemotherapy (CAPOX/FOLFOX for the intestinal subtype and mFOLFIRINOX for the pancreatobiliary or the mixed subtype) may be a valid alternative. Clinical and radiological follow up is recommended for 5 years. CONCLUSIONS: These guidelines help to homogenize and highlight unmet needs in the management of AA and AC. Each individual case should be discussed by a multidisciplinary team.
Subject(s)
Adenoma , Ampulla of Vater , Common Bile Duct Neoplasms , Humans , Ampulla of Vater/pathology , France , Common Bile Duct Neoplasms/therapy , Common Bile Duct Neoplasms/diagnosis , Adenoma/therapy , Adenoma/diagnosis , Endosonography , Societies, Medical , Duodenoscopy , Gastroenterology/standards , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Carcinoma/therapy , Carcinoma/diagnosis , PancreaticoduodenectomyABSTRACT
OBJECTIVE: This study primarily aimed to assess the expression of MUC4 in patients with pancreatic ductal adenocarcinoma (PDAC) as compared with controls and assess its clinical relevance. MATERIALS AND METHODS: Serum MUC4 levels and MUC4 gene expression in snap-frozen tissue were analyzed through surface plasmon resonance and quantitative polymerase chain reaction, respectively. Tumor tissues and control tissues were analyzed for MUC4 and other mucins through immunohistochemistry. RESULT: MUC4 expression in tumor tissue was found to be significantly elevated in PDAC patients as compared with chronic pancreatitis tissues and normal pancreatic tissues. Periampullary carcinoma and cholangiocarcinoma tissue also showed increased expression of MUC4 and other mucins. CONCLUSIONS: Differential expression of MUC4 in pancreatic tumor tissues can help to differentiate PDAC from benign conditions.
Subject(s)
Carcinoma, Pancreatic Ductal , Cholangiocarcinoma , Immunohistochemistry , Mucin-4 , Pancreatic Neoplasms , Humans , Mucin-4/metabolism , Mucin-4/genetics , Pancreatic Neoplasms/genetics , Pancreatic Neoplasms/metabolism , Pancreatic Neoplasms/diagnosis , Pancreatic Neoplasms/blood , Carcinoma, Pancreatic Ductal/genetics , Carcinoma, Pancreatic Ductal/metabolism , Carcinoma, Pancreatic Ductal/diagnosis , Carcinoma, Pancreatic Ductal/pathology , Male , Middle Aged , Female , Aged , Cholangiocarcinoma/genetics , Cholangiocarcinoma/metabolism , Cholangiocarcinoma/diagnosis , Biomarkers, Tumor/genetics , Biomarkers, Tumor/metabolism , Biomarkers, Tumor/blood , Adult , Pancreatitis, Chronic/metabolism , Pancreatitis, Chronic/genetics , Pancreatitis, Chronic/diagnosis , Pancreatitis, Chronic/blood , Case-Control Studies , Ampulla of Vater/metabolism , Ampulla of Vater/pathology , Gene Expression Regulation, Neoplastic , Common Bile Duct Neoplasms/metabolism , Common Bile Duct Neoplasms/genetics , Common Bile Duct Neoplasms/diagnosis , Common Bile Duct Neoplasms/pathology , Clinical RelevanceABSTRACT
Periampullary cancers, including pancreatic ductal adenocarcinoma, ampullary-, cholangio-, and duodenal carcinoma, are frequently diagnosed in an advanced stage and are associated with poor overall survival. They are difficult to differentiate from each other and challenging to distinguish from benign periampullary disease preoperatively. To improve the preoperative diagnostics of periampullary neoplasms, clinical or biological markers are warranted.In this study, 28 blood plasma amino acids and derivatives from preoperative patients with benign (N = 45) and malignant (N = 72) periampullary disease were analyzed by LC-MS/MS.Principal component analysis and consensus clustering both separated the patients with cancer and the patients with benign disease. Glutamic acid had significantly higher plasma expression and 15 other metabolites significantly lower plasma expression in patients with malignant disease compared with patients having benign disease. Phenylalanine was the only metabolite associated with improved overall survival (HR = 0.50, CI 0.30-0.83, P < 0.01).Taken together, plasma metabolite profiles from patients with malignant and benign periampullary disease were significantly different and have the potential to distinguish malignant from benign disease preoperatively.
Subject(s)
Amino Acids , Biomarkers, Tumor , Humans , Male , Female , Amino Acids/blood , Middle Aged , Aged , Biomarkers, Tumor/blood , Ampulla of Vater/pathology , Tandem Mass Spectrometry , Diagnosis, Differential , Common Bile Duct Neoplasms/blood , Common Bile Duct Neoplasms/diagnosis , Common Bile Duct Neoplasms/surgery , Common Bile Duct Neoplasms/pathology , Duodenal Neoplasms/blood , Duodenal Neoplasms/diagnosis , Duodenal Neoplasms/pathology , Duodenal Neoplasms/surgery , Adult , Pancreatic Neoplasms/blood , Pancreatic Neoplasms/diagnosis , Pancreatic Neoplasms/surgery , Pancreatic Neoplasms/mortality , Chromatography, Liquid , Principal Component Analysis , Carcinoma, Pancreatic Ductal/blood , Carcinoma, Pancreatic Ductal/diagnosis , Carcinoma, Pancreatic Ductal/pathologyABSTRACT
Malignant melanoma (MM) is an aggressive tumor that can metastasize to any organ, but biliary tract metastasis is scarce. We describe a very rare case of MM metastasis to the common bile duct (CBD), presented with only dyspeptic symptoms. The patient had mildly elevated alkaline phosphatase and gamma-glutamyl transferase levels. Magnetic resonance cholangiopancreatography demonstrated a dilated common bile duct with a distal stricture. The MM diagnosis was established with the ampulla of Vater biopsy specimens obtained by endoscopic retrograde cholangiopancreatography (ERCP), and the patient's symptoms were resolved after biliary stenting. Both primary CBD cancer and other cancer types like MM that metastasize to CBD can cause obstruction and can be manifested only by dyspeptic symptoms. MM metastasis to CBD can cause obstruction manifested only by dyspeptic symptoms without obstructive jaundice. ERCP can be employed as a promising option for treatment and diagnosis. New-onset dyspeptic symptoms in patients with a history of MM should be investigated thoroughly, especially in the context of biliary metastasis.
Subject(s)
Cholangiopancreatography, Endoscopic Retrograde , Cholangiopancreatography, Magnetic Resonance , Dyspepsia , Melanoma , Tomography, X-Ray Computed , Humans , Melanoma/diagnosis , Melanoma/secondary , Melanoma/pathology , Melanoma/complications , Dyspepsia/diagnosis , Dyspepsia/etiology , Male , Middle Aged , Common Bile Duct/pathology , gamma-Glutamyltransferase/blood , Common Bile Duct Neoplasms/diagnosis , Common Bile Duct Neoplasms/pathology , Common Bile Duct Neoplasms/complications , Common Bile Duct Neoplasms/secondary , Alkaline Phosphatase/blood , Alkaline Phosphatase/metabolismABSTRACT
BACKGROUND AND AIM: An early and accurate diagnosis of ampullary neoplasia is crucial; however, sampling bias is still a major concern. New-generation endocytoscopy enables real-time visualization of cellular structures and enables an accurate pathological prediction; however, its feasibility for small ampullary lesions has never been investigated. METHODS: We developed a novel endocytoscopic (EC) classification system for ampullary lesions after an expert review and agreement from five experienced endoscopists and one pathologist. We then consecutively enrolled a total of 43 patients with an enlarged ampulla (< 3 cm), all of whom received an endocytoscopic examination. The feasibility of endocytoscopy was evaluated, and the performance of the EC classification system was then correlated with the final histopathology. RESULTS: In five cases (11.6%), the endocytoscope could not approach the ampulla, and these cases were defined as technical failure. Among the remaining 38 patients, 8 had histopathology-confirmed adenocarcinoma, 15 had adenoma, and 15 had non-neoplastic lesions. The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of the EC classification system to diagnose ampullary neoplasias were 95.7%, 86.7%, 91.7%, 92.9%, and 92.1%, respectively. Moreover, the sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of the EC classification to diagnose ampullary cancer were 62.5%, 100%, 100%, 90.9%, and 92.1%, respectively. One case with intra-ampullary papillary-tubular carcinoma was classified as having a non-neoplastic lesion by endocytoscopy. CONCLUSIONS: Endocytoscopy and the novel EC classification system demonstrated good feasibility to discriminate ampullary neoplasias from non-neoplastic lesions and may be useful for optical biopsies of clinically suspicious ampullary lesions.
Subject(s)
Adenocarcinoma , Ampulla of Vater , Common Bile Duct Neoplasms , Feasibility Studies , Humans , Ampulla of Vater/pathology , Ampulla of Vater/diagnostic imaging , Pilot Projects , Female , Aged , Common Bile Duct Neoplasms/pathology , Common Bile Duct Neoplasms/diagnostic imaging , Common Bile Duct Neoplasms/diagnosis , Male , Middle Aged , Adenocarcinoma/pathology , Adenocarcinoma/diagnosis , Adenocarcinoma/diagnostic imaging , Adenoma/pathology , Adenoma/diagnosis , Adenoma/diagnostic imaging , Predictive Value of Tests , Aged, 80 and over , Sensitivity and Specificity , AdultABSTRACT
Ampullary carcinomas are rare but increasing in incidence. Ampullary cancers have molecular alterations that guide choice of therapy, particularly in nonresectable cases. These alterations can be more common by subtype (intestinal, pancreaticobiliary, or mixed), and next-generation sequencing is recommended for all patients who cannot undergo surgery. In this article, we review the approach to tissue acquisition and consideration for molecular testing. Common molecular targets of interest in ampullary cancer are also discussed in this review, including HER2/ERBB2, HER3, tumor mutational burden, microsatellite instability, KRAS, and germline BRCA and ATM mutations, along with emerging and rarer alterations.
Subject(s)
Ampulla of Vater , Common Bile Duct Neoplasms , Molecular Targeted Therapy , Humans , Ampulla of Vater/pathology , Molecular Targeted Therapy/methods , Common Bile Duct Neoplasms/therapy , Common Bile Duct Neoplasms/diagnosis , Common Bile Duct Neoplasms/genetics , Common Bile Duct Neoplasms/pathology , Mutation , Biomarkers, Tumor/geneticsABSTRACT
The ampulla of Vater is a small projection formed by the confluence of the main pancreatic duct and common bile duct in the second part of the duodenum. Primary ampullary adenocarcinoma is a rare malignancy, accounting for only 0.2% of gastrointestinal cancers and approximately 7% of all periampullary cancers. Jaundice from a biliary obstruction is the most common symptom of ampullary adenocarcinoma. In the early stages, radical pancreatoduodenectomy is the standard surgical approach. On the other hand, no randomized controlled trial has provided evidence to guide physicians on the choice of adjuvant/palliative chemotherapy because of the rarity of the disease and the paucity of related research. This paper reports the biology, histology, current therapeutic strategies, and potential future therapies of ampullary adenocarcinoma.
Subject(s)
Adenocarcinoma , Ampulla of Vater , Common Bile Duct Neoplasms , Duodenal Neoplasms , Pancreatic Neoplasms , Humans , Ampulla of Vater/pathology , Ampulla of Vater/surgery , Adenocarcinoma/diagnosis , Adenocarcinoma/drug therapy , Adenocarcinoma/pathology , Pancreatic Neoplasms/pathology , Common Bile Duct Neoplasms/diagnosis , Common Bile Duct Neoplasms/surgery , Common Bile Duct Neoplasms/pathology , Duodenal Neoplasms/pathology , Pancreatic NeoplasmsABSTRACT
BACKGROUND: The prevalence of different periampullary cancers (pancreatic ductal adenocarcinoma, distal cholangiocarcinoma, ampullary cancer and duodenal cancer) is heterogeneous in the literature. During the 2010s, a standardized histopathological protocol for pancreatoduodenectomy specimens based on axial slicing was adopted in Sweden. The present study sought to provide information about periampullary cancers with regard to tumour types in curative and noncurative settings, preoperative diagnostic accuracy and the impact of a standardized evaluation of the surgical specimen on diagnosis, R status and lymph node assessment. METHODS: Data from patients diagnosed with periampullary cancer from 2010 to 2019 were retrieved from the Swedish National Registry for Pancreatic and Periampullary Cancer. RESULTS: Among non-curative patients, 3704 (83.6 per cent) were diagnosed with pancreatic ductal adenocarcinoma. Among patients treated with pancreatoduodenectomy, diagnosis was pancreatic ductal adenocarcinoma in 1380 (50.0 per cent), distal cholangiocarcinoma in 284 (10.3 per cent), ampullary cancer in 376 (13.6 per cent), duodenal cancer in 160 (5.8 per cent) and other diagnoses in 560 (20.3 per cent) patients. The preoperative diagnosis corresponded to the postoperative in 1177 (67.5 per cent) patients for pancreatic ductal adenocarcinoma, 162 (37.4 per cent) patients for distal cholangiocarcinoma, 220 (61.3 per cent) patients for ampullary cancer and 120 (53.6 per cent) patients for duodenal cancer. A higher rate of pancreatic ductal adenocarcinoma was seen in surgical specimens who underwent standardized evaluation, from 56.8 per cent to 64.3 per cent (P = 0.003). After standardization, higher rates of R1 resection (31.7 per cent versus 44.6 per cent, P < 0.001) and N1 stage (62.1 per cent versus 77.0 per cent, P < 0.001) were found. CONCLUSION: The proportion of pancreatic ductal adenocarcinoma was higher in patients in a non-curative setting compared with patients who underwent surgery. The rate of misdiagnosis for periampullary cancers was confirmed to be high. Thus, it should be taken into account when preoperative oncological treatment is considered. Standardized evaluation of the surgical specimen has increased pancreatic ductal adenocarcinoma, R1 and N1 rates.
Subject(s)
Adenocarcinoma , Ampulla of Vater , Bile Duct Neoplasms , Carcinoma, Pancreatic Ductal , Cholangiocarcinoma , Common Bile Duct Neoplasms , Duodenal Neoplasms , Pancreatic Neoplasms , Humans , Ampulla of Vater/surgery , Duodenal Neoplasms/diagnosis , Duodenal Neoplasms/surgery , Duodenal Neoplasms/pathology , Cohort Studies , Adenocarcinoma/diagnosis , Adenocarcinoma/surgery , Adenocarcinoma/pathology , Common Bile Duct Neoplasms/diagnosis , Common Bile Duct Neoplasms/surgery , Common Bile Duct Neoplasms/pathology , Pancreatic Neoplasms/diagnosis , Pancreatic Neoplasms/surgery , Pancreatic Neoplasms/pathology , Carcinoma, Pancreatic Ductal/diagnosis , Carcinoma, Pancreatic Ductal/surgery , Carcinoma, Pancreatic Ductal/pathology , Cholangiocarcinoma/diagnosis , Cholangiocarcinoma/epidemiology , Cholangiocarcinoma/surgery , Bile Duct Neoplasms/diagnosis , Bile Duct Neoplasms/surgery , Bile Duct Neoplasms/pathology , Bile Ducts, Intrahepatic/pathology , Pancreatic NeoplasmsABSTRACT
Endoscopic biopsies from the ampulla of Vater are challenging due to specimen sampling limitation, small size, interventional artifacts, and the nature of local complex anatomy. We retrospectively reviewed 318 in-house ampulla of Vater biopsy specimens from 252 patients over a 10-year period. The biopsy findings were compared to those in subsequent biopsy and/or resection specimens. Of the 318 biopsy cases, 104 (32.7 %) cases were diagnosed as adenoma (96 with low-grade dysplasia; 8 with high-grade dysplasia), 19 (6.0 %) adenocarcinomas (ampullary-12, distal bile duct-6, pancreatic-1), 5 (1.6 %) other carcinomas/tumors, and the rest were benign findings (unremarkable, ulcer and acute inflammation, reactive changes, and rare atypical cells/gland). Of the 90 cases with follow-up specimens, 55 cases (61.1 %) had concordant results and 35 (38.9 %) were discordant. Eight (22.9 %) of the 35 discordant cases had major discrepancies (benign biopsy diagnosis with malignant resection diagnosis); 27 (77.1 %) cases had minor discrepancies (normal, reactive, atypical, and dysplastic). We found that vast majority of the false negative biopsy results were due to sampling limitations. Combined biopsy and cytology specimens may help decrease the false negative rate. Careful correlation with endoscopic/cytology/clinical findings and acknowledging the limitation of the biopsy material in the pathology report are important, when malignancy is suspected but cannot be established in a small ampullary biopsy.
Subject(s)
Ampulla of Vater , Common Bile Duct Neoplasms , Humans , Ampulla of Vater/pathology , Ampulla of Vater/surgery , Retrospective Studies , Common Bile Duct Neoplasms/diagnosis , Common Bile Duct Neoplasms/pathology , Biopsy , Bile Ducts/pathologyABSTRACT
Adenoma of the major duodenal papilla can cause bile flow blockade and obstructive jaundice. We present successful endoscopic radiofrequency ablation for adenoma of the major duodenal papilla with intraductal growth in the common bile duct.
Subject(s)
Adenoma , Ampulla of Vater , Common Bile Duct Neoplasms , Radiofrequency Ablation , Humans , Ampulla of Vater/surgery , Common Bile Duct Neoplasms/diagnosis , Common Bile Duct Neoplasms/surgery , Common Bile Duct/surgery , Adenoma/diagnosis , Adenoma/surgery , Treatment OutcomeABSTRACT
There are different cancers in the peri-ampullary region, including pancreatic ductal adenocarcinoma (PDAC), duodenum cancers (DCs), and ampullary adenocarcinoma (AAC). Here, significant morphological-molecular characterizations should be necessary for the distinction of primary tumours and classifications of their subtypes of cancers. The sub classification of AACs might include up to five different variants, according to different points of view, concerning the prevalence of the two more-cellular components found in the ampulla. In particular, regarding the AACs, the most important subtypes are represented by the intestinal (INT) and the pancreato-biliary (PB) ones. The subtyping of AACs is essential for diagnosis, and their identifications have been impacting clinical management responses to treatments and overall survival (os) after surgery. Pb is associated with a worse clinical outcome. Otherwise, the criteria, through which are possible to attribute its subtype classification, are not well established. A triage of immune markers represented by CK7, CK20, and CDX-2 seem to represent the best compromise in order to split the cohort of AAC patients in the INT and PB groups. The test of choice for the sub-classification of AACs is represented by the immuno-histochemical approach, in which its molecular classification acquires its diagnostic, predictive, and prognostic value for both the INT and PB patients.
Subject(s)
Adenocarcinoma , Ampulla of Vater , Common Bile Duct Neoplasms , Pancreatic Neoplasms , Humans , Biomarkers, Tumor/analysis , Lead/analysis , Common Bile Duct Neoplasms/diagnosis , Pancreatic Neoplasms/pathology , Adenocarcinoma/diagnosis , Pancreatic NeoplasmsABSTRACT
Ampullary cancers refer to tumors originating from the ampulla of Vater (the ampulla, the intraduodenal portion of the bile duct, and the intraduodenal portion of the pancreatic duct), while periampullary cancers may arise from locations encompassing the head of the pancreas, distal bile duct, duodenum, or ampulla of Vater. Ampullary cancers are rare gastrointestinal malignancies, and prognosis varies greatly based on factors such as patient age, TNM classification, differentiation grade, and treatment modality received. Systemic therapy is used in all stages of ampullary cancer, including neoadjuvant therapy, adjuvant therapy, and first-line or subsequent-line therapy for locally advanced, metastatic, and recurrent disease. Radiation therapy may be used in localized ampullary cancer, sometimes in combination with chemotherapy, but there is no high-level evidence to support its utility. Select tumors may be treated surgically. This article describes NCCN recommendations regarding management of ampullary adenocarcinoma.
Subject(s)
Adenocarcinoma , Ampulla of Vater , Common Bile Duct Neoplasms , Duodenal Neoplasms , Humans , Common Bile Duct Neoplasms/diagnosis , Common Bile Duct Neoplasms/therapy , Duodenal Neoplasms/diagnosis , Duodenal Neoplasms/therapy , Adenocarcinoma/diagnosis , Adenocarcinoma/therapy , Pancreatic NeoplasmsABSTRACT
RATIONALE: Adenomyoma is a rare reactive, hamartomatous benign tumor-like lesion. Although adenomyoma can occur anywhere in the gastrointestinal tract, including the gallbladder, stomach, duodenum, and jejunum, it is very rarely observed in the extrahepatic bile duct and ampulla of Vater (AOV). The preoperative accurate diagnosis of adenomyoma of the Vaterian system, including the AOV and common bile duct, is significant to appropriate patient management. However, discriminating between benign and malignancy is highly challenging. Patients are frequently mistaken as having periampullary malignancy, thereby leading to unnecessary extensive surgical resection with a high risk of complications. PATIENT CONCERNS: A 47-year-old woman visited a local hospital owing to epigastric and right upper-quadrant abdominal pain for 2 days. DIAGNOSES: Abdominal ultrasonography performed in the local hospital revealed suspicious of a distal common bile duct malignancy. She was transferred to our hospital for further evaluation and management. INTERVENTIONS: After consulting with the patient, a multidisciplinary team, including a gastroenterologist, finally decided to perform surgery under the impression of an ampullary malignancy, and pylorus-preserving pancreatoduodenectomy was performed without any complications. She was histopathologically diagnosed with an adenomyoma of the AOV. OUTCOMES: At the 5-year follow-up, she was well and did not develop further symptoms or complications. LESSONS: Although adenomyoma is very rare, it should be included in the differential diagnosis of mass-like lesions of the AOV to avoid unnecessary surgeries.
Subject(s)
Adenomyoma , Ampulla of Vater , Common Bile Duct Neoplasms , Female , Humans , Middle Aged , Ampulla of Vater/surgery , Ampulla of Vater/pathology , Adenomyoma/diagnostic imaging , Adenomyoma/surgery , Common Bile Duct Neoplasms/diagnosis , Common Bile Duct Neoplasms/surgery , Common Bile Duct Neoplasms/pathology , Common Bile Duct/pathology , PancreaticoduodenectomyABSTRACT
BACKGROUND: Tumors of mixed neuroendocrine and nonneuroendocrine histology are classified as collision, combined, or amphicrine and can occur in most organs, including the hepato-pancreato-biliary tract. Given the rarity of mixed adenoneuroendocrine carcinoma (MANEC) of the ampulla of Vater, the patient characteristics, management, and outcomes remain unclear. We sought to systematically review the worldwide literature on ampullary MANECs. METHODS: Eligible studies were identified through a systematic search of the MEDLINE (via PubMed), Scopus, and Cochrane Library databases (end-of-search-date: January 5th, 2022), according to the PRISMA 2020 statement. RESULTS: A total of 39 studies reporting on 56 patients with ampullary MANEC were included. The median age was 63.0 (interquartile range [IQR]: 51.0-69.0) years and 55.6% were male (n = 25/45). Most had combined tumors (64.4%; n = 29/45), followed by collision (24.4%; n = 11/45), and amphicrine tumors (11.1%; n = 5/45). More than half had lymph node metastasis (56.8%; n = 25/44), yet only 7.9% had distant metastasis (n = 3/38). Tumor resection (i.e., mostly pancreaticoduodenectomy) was performed in 96.3% (n = 52/54), followed by adjuvant chemotherapy in 61.8% (n = 21/34). Nearly half experienced disease recurrence (47.2%; n = 17/36) over a median follow-up of 12.0 (IQR: 3.0-16.0) months, and 42.1% (n = 16/38) died over a median follow-up of 12.0 (IQR: 4.0-18.0) months. The most common cause of death was disease progression/recurrence in 81.3% (n = 13/16). CONCLUSION: Early diagnosis and management of ampullary MANEC is challenging yet crucial to improve outcomes since many patients are diagnosed at an advanced disease stage and have unfavorable outcomes. Multicenter granular data are warranted to further understand and improve outcomes in these patients.
Subject(s)
Adenocarcinoma , Ampulla of Vater , Carcinoma, Neuroendocrine , Common Bile Duct Neoplasms , Gastrointestinal Neoplasms , Humans , Male , Middle Aged , Aged , Female , Ampulla of Vater/pathology , Neoplasm Recurrence, Local/pathology , Adenocarcinoma/pathology , Pancreaticoduodenectomy , Gastrointestinal Neoplasms/pathology , Common Bile Duct Neoplasms/surgery , Common Bile Duct Neoplasms/diagnosis , Carcinoma, Neuroendocrine/surgery , Carcinoma, Neuroendocrine/diagnosis , Multicenter Studies as TopicABSTRACT
In this case report is discussed the diagnosis of papillary carcinoma in the case of a patient in whose age the occurrence of the disease is very rare. The patient underwent a series of non-invasive and invasive examinations, the results of which eventually led to a surgical solution of the suspected lesion (Wipples procedure). Histological examination of the resection confirmed mixed type ampular adenocarcinoma. In the context of this finding, the results of the examination, their information value, interpretation and mutual interaction are discussed. In the postoperative period, the patient suffered from gastrointestinal bleeding, subileum and poor postoperative healing. After hospitalization, the patient was transferred to oncology care and indicated for genetic testing.
Subject(s)
Adenocarcinoma , Adenomatous Polyposis Coli , Ampulla of Vater , Common Bile Duct Neoplasms , Jaundice , Humans , Ampulla of Vater/pathology , Ampulla of Vater/surgery , Adenomatous Polyposis Coli/surgery , Common Bile Duct Neoplasms/diagnosis , Common Bile Duct Neoplasms/pathology , Common Bile Duct Neoplasms/surgery , Adenocarcinoma/complications , Adenocarcinoma/diagnosis , Jaundice/pathologyABSTRACT
Most tumours in the head of the pancreas are adenocarcinomas of the exocrine pancreas. However, carcinomas located in the head of the pancreas may originate from the papilla of Vater, the distal part of the common bile duct, or the duodenum. Tumours of that region, within 2 cm of the greater duodenal papilla, have been usually described as periampullary neoplasms. Adenocarcinomas separated from the major duodenal papilla and located in the major pancreatic duct, common bile duct, or duodenum are identified as ductal pancreatic carcinomas, distal bile duct cholangiocarcinomas or duodenal carcinomas. Surgical treatment is the only chance for cure. Pancreatoduodenectomy is the procedure of choice. Regional lymphadenectomy and removal of at least 16 lymph nodes are necessary for optimal long-term outcomes. Indications for adjuvant chemotherapy remain controversial. This review evaluates the available data on the pathological assessment of periampullary tumours and discusses the controversies of therapeutic management, emphasising adjuvant treatment.
Subject(s)
Adenocarcinoma , Ampulla of Vater , Carcinoma, Pancreatic Ductal , Common Bile Duct Neoplasms , Pancreatic Neoplasms , Adenocarcinoma/pathology , Ampulla of Vater/pathology , Carcinoma, Pancreatic Ductal/pathology , Common Bile Duct Neoplasms/diagnosis , Common Bile Duct Neoplasms/surgery , Humans , Pancreatic Neoplasms/diagnosis , Pancreatic Neoplasms/pathology , Pancreatic Neoplasms/therapyABSTRACT
BACKGROUND: A metastatic lesion located in the ampulla of Vater is considered extremely rare, with only 32 cases reported globally. CASE: A 65-year-old patient was primarily diagnosed with a rectal adenocarcinoma. Twenty-four months later as part of the oncological follow-up, the patient was diagnosed with a single secondary tumor in the ampulla of Vater. After undergoing a pancreaticoduodenectomy (Whipple procedure), the patient experienced an uneventful recovery and received adjuvant chemotherapy. Sixteen months later the patient remained disease-free. CONCLUSION: To the best of our knowledge, the present case represents the first reported metastatic tumor in the ampulla of Vater, originating from a rectal adenocarcinoma. This case underlines the critical role of immunohistochemistry in arriving at a correct diagnosis in order to guide clinical decision-making.