Ampullary lesions are rare and can be locally treated either with endoscopic papillectomy or transduodenal surgical ampullectomy. Management of local recurrence after a first-line treatment has been poorly studied.

Patients with a local recurrence of an ampullary lesion initially treated with endoscopic papillectomy or transduodenal surgical ampullectomy were retrospectively included from a multi-institutional database (58 centers) between 2005 and 2018.

A total of 103 patients were included, 21 (20.4%) treated with redo endoscopic papillectomy, 14 (13.6%) with transduodenal surgical ampullectomy, and 68 (66%) with pancreaticoduodenectomy. Redo endoscopic papillectomy had low morbidity with 4.8% (n = 1) severe to fatal complications and a R0 rate of 81% (n = 17). Transduodenal surgical ampullectomy and pancreaticoduodenectomy after a first procedure had a higher morbidity with Clavien III and more complications, respectively, 28.6% (n = 4) and 25% (n = 17); R0 resection rates were 85.7% (n = 12) and 92.6% (n = 63), both without statistically significant difference compared to endoscopic papillectomy (P = .1 and 0.2). Pancreaticoduodenectomy had 4.4% (n = 2) mortality. No deaths were registered after transduodenal surgical ampullectomy or endoscopic papillectomy. Recurrences treated with pancreaticoduodenectomy were more likely to be adenocarcinomas (79.4%, n = 54 vs 21.4%, n = 3 for transduodenal surgical ampullectomy and 4.8%, n = 1 for endoscopic papillectomy, P < .0001). Three-year overall survival and disease-free survival were comparable.

Endoscopy is appropriate for noninvasive recurrences, with resection rate and survival outcomes comparable to surgery. Surgery applies more to invasive recurrences, with transduodenal surgical ampullectomy rather for carcinoma in situ and early cancers and pancreaticoduodenectomy for more advanced tumors.

Transduodenal ampullectomy (TDA) is not in wide clinical use due to its low radical effect and a high recurrence rate of tumors. However, TDA is still an effective treatment method; it has great clinical value in cases of duodenal benign tumors, precancerous lesions, and benign and malignant borderline tumors, and can avoid the risks associated with pancreaticoduodenectomy with larger resection range and greater thoroughness than endoscopic papillectomy.

To investigate the surgical method choice and the coincidence rate of pathological diagnoses in TDA for ampullary neoplasms.

Ten patients with ampullary neoplasms underwent TDA based on the fact that their endoscopic biopsy results suggested benign lesions, and the endoscopic ultrasound (EUS)-assessed tumors were resectable. All cases underwent duodenal ampullary lesion endoscopic biopsy, intraoperative frozen-section pathological examination, and postoperative pathological examination.

This study included seven patients with benign tumors and three with malignant tumors (1 pTis, 2 pT1), according to the postoperative pathology results. The coincidence rate of the postoperative pathology results with the intraoperative frozen-section biopsy results was 100% (10/10), and the coincidence rate with the endoscopic biopsy results was 70% (7/10) based on pathological characteristics. The endoscopic biopsy false-negative rate was 30% (3/10). All patients were followed for 6 to 70 mo without tumor recurrence or metastasis.

The coincidence rate of postoperative pathology results, intraoperative frozen-section pathology results, and endoscopic biopsy results is the restraining factor of TDA clinical application. Endoscopic biopsy results and EUS have importance relevance to surgical planning. Intraoperative frozen-section pathology results have a significant influence on the choice of surgical procedure.

In some patients with tumors located in the pancreas or in the periampullary region, the decision to perform a surgical resection can be difficult. In patients with concomitant chronic pancreatitis this decision can be even more challenging, since a definitive preoperative differentiation between non-malignant and malignant tumors in many cases is not possible. Clinical symptoms or complications from the tumor often direct a rational treatment strategy. For therapeutic decisions, an interdisciplinary discussion of all diagnostic findings by experienced clinicians is needed. However, in rare cases, like the one presented here, an uncommon non-malignant entity like a periampullary hamartoma may be only diagnosed after surgical resection.

Adenoma and adenocarcinoma are the most common ampullary lesions. Advances in diagnostic modalities including endoscopic ultrasonography and intraductal ultrasonography have provided useful information that aids in diagnosing and managing ampullary lesions. Endoscopic papillectomy can be a curative therapy for localized ampullary adenoma and have a role in the diagnosis of indeterminate ampullary lesions that may contain a hidden malignancy. However, the consensus on how and when to use endoscopic papillectomy has not been fully established. This article reviews the approach to the patient with benign or malignant ampullary lesion.

Heterotaxy designates rare congenital disorders of organ positioning in the thoracic and abdominal cavities, which can be associated with numerous anomalies, complicating the surgical management because of the loss of conventional anatomic landmarks.

A 72-year-old man was found to have asymptomatic cholestasis. Further workup included computed tomography and magnetic resonance cholangiopancreatography that revealed anomalies of lateralization of digestive organs associated with intestinal malrotation and polysplenia, and a stone-like element in the main bile duct. Endoscopic retrograde cholangiopancreatography failed to extract the lesion. Laparotomy found no stone, but a polypoid tumor with ampullary implantation. Pancreaticoduodenectomy was judged unreasonable due to the presence of macroscopic cirrhosis and a complete ampullectomy was performed. Histopathological examination revealed a hamartomatous polyp.

The unusual angle of the duodenoscope in a left-sided duodenum may have contributed to the improper pre-operative diagnosis. Endosonography could have recognized the tissular origin of the lesion and prompted a more detailed preoperative planning. It was fortunate that the patient ended up receiving the appropriate treatment despite the absence of an adequate pre-operative diagnosis, as the option of performing an extended resection was ruled out due to the presence of cirrhosis.

Although heterotaxy leads to increased technical difficulties in performing usual endoscopic and surgical procedures, it can be safely managed by experienced surgeons as illustrated by the present case. Imaging modalities have limited sensitivity in the diagnosis of small ampullary tumors. As false-negatives are likely to occur, this possibility should guide the choice of the best operation.

Endoscopic treatment of ampullary lesions has been well described, though it remains uncertain if specific features predict malignancy, and whether identifiable factors are associated with successful endoscopic resection of benign lesions.

Fifty-six consecutive patients undergoing endoscopic evaluation of ampullary neoplasia between March 2000 and May 2004 were included in the study. Clinical presentation, underlying medical conditions, endoscopic treatment, endoscopic ultrasound (EUS) to define extent of local involvement, pathology results, and outcome were documented. Data elements for analysis included EUS findings, lesion lifting with submucosal injection, age, gender, tumor size, and endoscopic intervention. Analyses were performed to determine the ability to predict malignancy and the ability to extirpate benign lesions.

Thirty-one males and 25 females were included; mean age was 62 yr. Final diagnoses included 29 adenomas, 20 adenocarcinomas, 4 adenomyomas, 2 paragangliomas, and 1 neuroendocrine tumor. Thirty of 35 patients with benign lesions had extirpation with a mean of two endoscopic procedures. Complications of endoscopic resection included cholangitis (1), bleeding (2), and pancreatitis (4). The presence of malignancy was associated by multivariate analysis with the inability to obtain a cleavage plane with saline injection. Univariate analysis also identified EUS T stage as a predictor of malignancy. In benign lesions, none of the analyzed variables predicted successful endoscopic resection.

In ampullary lesions, failure to achieve a cleavage plane with submucosal injection is the strongest predictor of malignancy followed by EUS T stage. Endoscopic treatment of benign ampullary neoplasia is effective; no factor was predictive of successful extirpation.

Ampullary adenomas occur sporadically and in the setting of familial polyposis syndromes. In either case, and whether symptomatic at presentation or found asymptomatically in the setting of endoscopic screening programs, they are premalignant lesions with risk for malignant degeneration to carcinoma following the adenoma-to-carcinoma sequence that is well recognized in colonic adenocarcinoma. Accordingly, many experts advocate excision, although others cite the low rate of histologic progression suggested by some recent studies as justification for close endoscopic surveillance rather than excision before demonstration of dysplastic change. This recommendation, however, is complicated by considerable data underscoring the limited accuracy of endoscopic forceps biopsy in detecting occult foci of carcinoma within ampullary adenoma. Thus, the optimal management of these lesions continues to generate considerable controversy. Indications for excision of an ampullary adenoma include treatment of immediate symptoms as well as prevention of malignant degeneration. Although pancreaticoduodenectomy has long been considered the standard procedure for ampullary carcinoma, much controversy exists regarding the procedure of choice for ampullary adenoma. Radical surgery (pancreaticoduodenectomy) possesses the advantage of low recurrence rate but at the expense of higher morbidity (25%-65%) and mortality (0%-10%). Local surgical excision (surgical ampullectomy) possesses the advantages of lower morbidity (0%-25%), essentially nil mortality, and possibly decreased length of hospital stay, but decidedly higher recurrence rates (generally 5%-30%) and the need for postoperative endoscopic surveillance. Snare ampullectomy is a newer endoscopic excisional technique for which limited data are available; advantages compared with radical surgery mirror those of local surgical excision, with apparent lower mortality (0%-1%) and lower morbidity (12%). Presumed advantages compared with local surgical excision include lack of necessity for general anesthesia and laparotomy with comparable morbidity. Disadvantages seem to include limited availability of experienced operators, procedural complexity sometimes requiring adjunctive modalities such as fulguration, the need for multiple procedures (mean, 2.0 procedures) to effect complete excision, and recurrence rates approaching 30%, with a requirement for continued endoscopic surveillance. Ultimately, choice is driven by availability of local expertise, patient tolerance of or expected compliance with long-term endoscopic surveillance programs, presence or absence of coexisting familial polyposis syndromes, medical comorbidities, and overall life expectancy.

Adenomyoma and adenomyomatous hyperplasia of the Vaterian system are consistently benign lesions. Clinically, adenomyoma mimics frequently ampullary adenoma or carcinoma, and biopsy analysis is often difficult. The histogenesis of ampullary adenomyoma and adenomyomatous hyperplasia is still subject to debate. We present a retrospective study of clinicopathological features of 13 cases of surgically resected ampullary adenomyoma. The age of our patients was between 38 and 78 years (mean: 63 y). The preoperative diagnosis was ampullary tumor or tumor of the head of the pancreas. On macroscopy, a white, firm lesion of the ampullary wall was observed; its size ranged between 10 and 30 mm. Histologically the lesion consisted of multiple glandular structures surrounded by a fibroblastic/myofibroblastic proliferation, resulting in a "pseudo-hypertrophy" of the Vaterian system. The immunophenotype of the epithelial component was cytokeratin 7+/cytokeratin 20-, similar to that of the normal biliary and pancreatic duct system. The epithelial cells exhibited low proliferative activity. The hyperplastic myofibroblastic cells expressed smooth muscle actin. A complete pancreatic heterotopy contiguous with the adenomyoma was noted in three cases. Adenomyoma and adenomyomatous hyperplasia of the Vaterian system are benign lesions frequently treated by extensive surgery because of long-term biliary obstruction. The clinicopathological characteristics suggest either a reactive and/or a malformative, nonneoplastic nature for this lesion, which could, in some cases, develop from heterotopic pancreas. The immunophenotype of epithelial cells may be a useful tool for differentiating it from ampullary adenoma on biopsy specimens.

Frequency of the lesions of the papilla Vateri is increasing in Hungary because of epidemiological reasons. Over two years nearly 300 ampullary endoscopic biopsies were taken in our hospital. In 36 percent of the patients the papillary specimens demonstrated acute or chronic inflammation, in 44 percent adenoma, including 5 percent with severe dysplasia, in 5 percent adenomatous hyperplasia and in 7 percent adenomyosis or other benign tumors (2%) were found. Around 7 percent of the ampullary samples proved to be malignant, but only in 2.6 percent were the malignancy of intraampullary origin. Nearly 25 percent of biopsies were repeated once and 10 percent twice or more. Concordance of endoscopic and pathologic diagnoses was 69 percent on average but it increased to 83 percent after including repeated biopsies. In the adenoma-carcinoma group the concordance was 90 percent. The sensitivity of the pathological diagnosis with forceps biopsy was only 77 percent, but it became at least 86 percent following papillectomy. In order to improve diagnostic reliability more extensive use of papillectomy is proposed with close cooperation between the endoscopist and pathologist.