Carcinoid of the ampulla of Vater

Endocrine neoplasms only rarely occur at the ampulla of Vater, comprising mostly carcinoids and malignant carcinoids, as well as few cases of poorly differentiated endocrine carcinomas (small cell carcinomas). Only 105 cases are reported in the literature, most as single case reports. For many years, the neoplasms of the disseminated neuroendocrine cell system of the gastrointestinal tract have been subsumed as 'carcinoids'. Instead, in the latest World Health Organization (WHO) classification published in 2000, it is recommended to distinguish between (i) well-differentiated endocrine tumors (carcinoids); (ii) well-differentiated endocrine carcinomas (malignant carcinoids); and (iii) poorly differentiated endocrine carcinomas (small cell carcinomas). Patients with carcinoid tumors of the ampulla of Vater are very often free of clinical and laboratory findings that belong to the carcinoid syndrome. Approximately 26% of all patients with carcinoid tumor reported in the literature had neurofibromatosis. Besides endoscopic retrograde cholangiopancreatography, endosonography, computed tomography or magnetic resonance imaging may complete the staging approach of this tumor. The Kausch-Whipple procedure or pylorus-preserving pancreaticoduodenectomy is considered the treatment of choice for ampullary, well-differentiated carcinoids >2.0 cm and for ampullary neuroendocrine carcinomas. However, it should be considered that long-term survival of patients with ampullary carcinoids is also reported after local tumor excision (5-year survival rate of 90%). The dilemma is that the differentiation of neuroendocrine tumors cannot be assessed intraoperatively in most cases. Therefore, considering that the 5-year survival rate in patients with neuroendocrine carcinomas of the ampulla of Vater is very low without radical resection, neuroendocrine tumors of the ampulla of Vater without definite histological differentiation should undergo extended surgery.     

Small-cell neuroendocrine carcinoma of the ampulla of Vater

We report a patient (an 80-year-old woman) with anemia and fecal occult blood, who had an emergency operation for carcinoma of the cecum (well-differentiated adenocarcinoma without local lymph node metastasis). Postoperative magnetic resonance cholangiopancreatography, cholangiography, and upper gastroduodenal endoscopy showed a tumor of the ampulla of Vater, and pylorus-preserving pancreatoduodenectomy was performed. Histology of the resected tumor was that of small-cell carcinoma, and immunohistochemistry showed positive staining for neuron-specific enolase, chromogranin A, and synaptophysin, confirming the neuroendocrine nature of the tumor. As the histology of the tumor was distinct from cecal carcinoma, and no tumors were found in other organs, the tumor was diagnosed as primary small-cell neuroendocrine carcinoma of the ampulla of Vater. The patient died due to multiple liver metastases of the carcinoma of the ampulla of Vater 7 months after the pancreatoduodenectomy. The clinical and morphological features of this disease have been reported in nine individuals previously.     

Large cell neuroendocrine carcinoma of the ampulla of Vater

BACKGROUND: Neuroendocrine tumors of the ampulla of Vater are extremely rare, and few cases of large cell neuroendocrine carcinoma (LCNEC) of the ampulla have been reported. METHODS: A 48-year-old male with obstructive jaundice was admitted to our hospital. On examination the patient was found to have a periampullary growth and subsequently underwent the Whipple's procedure. RESULTS: Histopathological examination and immunohistochemistry revealed features of LCNEC of the ampulla of Vater. The patient developed multiple liver metastases 6 months after Whipple's procedure. CONCLUSION: LCNEC of the ampulla of Vater is rare and highly aggressive, with a dismal prognosis.     

内镜下十二指肠乳头切除术在乳头部肿瘤诊断中的价值

目的:评价内镜下十二指肠乳头切除术(endos-copic papillectomy,EP)在乳头部肿瘤诊断中的临床价值.方法:分析2008-01/2012-03来我院的16例经病理检查确诊的十二指肠乳头部肿瘤患者的相关资料,评价EP在十二指肠乳头部肿瘤诊断中的临床价值.结果:16例患者中,内镜活检诊断为腺瘤者12例,慢性炎症改变者4例.EP将乳头部肿瘤组织切除后整体送检行病理学检查确诊为低分化腺癌者2例,高分化腺癌1例,腺瘤13例(其中1例腺瘤恶变,4例常规内镜活检诊断为慢性炎症改变者术后均确诊为腺瘤),EP术后准确率明显高于内镜活检法(P<0.05).超声内镜(endoscopic ultrasonography,EUS)检查提示病变均起源于黏膜层,胰胆管扩张者9例,未见黏膜下层连续性中断及胰胆管受累者,无腹膜后淋巴结肿大者;12例高回声病变者术后病理检查结果均为腺瘤,1例高回声病变局部见低回声区者术后病理检查结果为腺瘤恶变,3例低回声病变者术后病理检查结果均为腺癌.EP术后2例低分化腺癌者、1例腺瘤恶变者及1例腺瘤者胆管开口处见病变残留,提示胆管受累,但术前EUS检查均未提示胆管受累;1例高分化腺癌及11例腺瘤者病灶均完整切除,术后切缘阴性.EP术后除2例出现黑便外,无急性胰腺炎、穿孔及其他并发症发生,无患者死亡.结论:EP术将乳头部肿瘤组织切除后整体送检行病理学检查可提高诊断准确率,为治疗方法的抉择提供科学依据.     

Coexistent Ampullary Squamous Cell Carcinoma with Adenocarcinoma of the Pancreatic Duct

Primary squamous cell carcinoma (SCC) of ampulla has seldom been reported. However, metastatic SCC to ampulla of Vater is well known. We report a case of primary SCC of ampulla of Vater coexistent with well-differentiated adenocarcinoma of the distal pancreatic duct. A 50-year-old female presented with evidence of obstructive jaundice. Endoscopic retrograde cholangio-pancreatography revealed bulging papilla with ulcero-infiltrative growth at the ampulla of Vater. An initial endoscopic biopsy of the ampullary mass showed a well-differentiated SCC. The patient underwent Whipple''s operation. Thorough sampling of the dilated portion of the pancreatic duct showed presence of well-differentiated adenocarcinoma of the distal pancreatic duct. Immunohistochemical study with synaptophysin and chromogranin was done with negative result, ruling out neuroendocrine differentiation. Also, a detailed clinical, endoscopic and radiological examination was carried out, that excluded the presence of primary SCC elsewhere.     

A case of hepatectomy for liver metastasis after pancreatoduodenectomy for carcinoma of ampulla of Vater]

After curative resection of carcinoma of ampulla of Vater, 5-year survival rate has been reported ranging from 40% to 60%. Two major causes of the treatment failure are local recurrence and liver metastasis. Liver metastases are often multiple and are associated with poor prognosis. There have been few reports on long-term survivors after hepatectomy for metastatic liver tumors from carcinoma of ampulla of Vater. We report a 42 year-old female patient with solitary hepatic metastasis from carcinoma of ampulla of Vater, which was successfully treated by hepatectomy 69 months after curative Whipple's operation. Histologic examination of the resected specimen had revealed stage IB moderately-differentiated, intestinal type adenocarcinoma (T2N0M0). Since neither local recurrence or distant metastasis were detected, the patient underwent liver segmentectomy. Histologic study confirmed the presence of metastatic liver tumor from carcinoma of ampulla of Vater. She is doing well without evidence of recurrence at 20 months after hepatectomy.     

An inflammatory myofibroblastic tumor of the ampulla of vater successfully managed with endoscopic papillectomy: report of a case

Inflammatory myofibroblastic tumors (IMTs) are solid neoplastic mesenchymal proliferations composed of myofibroblastic spindle cells admixed with inflammatory infiltrates. The documented sites in the gastrointestinal tract include the esophagus, small intestine, colon, appendix, rectum, pancreas, spleen, liver, and Meckel's diverticulum. Biliary IMTs are rare, and IMTs arising from the ampulla of Vater have not been reported previously. Herein we report the case of a 65-year-old woman with an extrahepatic biliary obstruction due to IMT of the ampulla of Vater, and a successful therapeutic approach using endoscopic ultrasonography and endoscopic papillectomy.     

Proposed indications for limited resection of early ampulla of Vater carcinoma: clinico-histopathological criteria to confirm cure

Background

Limited resection is reserved for patients with high operative risk or benign adenomas. We aimed to define indications for limited resection of early ampulla of Vater carcinoma with curative intent through detailed preoperative examinations and histopathological evaluations.

Methods

We performed a retrospective cohort study of all consecutive Japanese patients who underwent resection for ampulla of Vater neoplasms at our hospital from 1986 to 2010.

Results

A total of 75 patients were identified. Moderately/poorly differentiated histology, lympho-vascular/perineural invasion, and duodenal/pancreatic invasion were significant risk factors for lymph node metastases. Macroscopically, non-exposed protruded- or ulcerative-type disease did not correlate directly with lymph node metastases; however, these tumor types were associated with other invasive features. In a subset of early carcinomas fulfilling the conditions of exposed protruded adenoma or papillary/well-differentiated adenocarcinoma determined by endoscopic biopsy, negative duodenal invasion determined by endoscopic ultrasonography, no tumor infiltration into the pancreatic duct determined by intraductal ultrasound, and diameter of the pancreatic duct ≤3?mm determined by endoscopic retrograde cholangiopancreatography (N?=?11), the incidence of lymph node metastasis and tumor infiltration into the pancreatic duct was 0%.

Conclusion

Strictly selected patients with early ampulla of Vater carcinomas may benefit from limited resection if the resected specimen is evaluated to confirm all histopathological criteria.     

Cephalic duodenopancreatectomy for endocrine tumor of the ampulla of Vater and of the minor papilla]

OBJECTIVES: Endocrine tumors of the ampulla of Vater and minor papilla are rare. This study describes the mode of presentation and evaluates the correlation between pathological features and prognosis. PATIENTS: Between 1982 and 1998, 6 patients (3 M, 3 F, mean age: 47.6 years, range: 36-58) for whom a diagnosis of endocrine tumor of the ampulla of Vater or minor papilla was made between 1982 and 1998 after histological examination of an operative specimen of pancreaticoduodenectomy. RESULTS: One patient was detected incidentally, two had a Zollinger-Ellison syndrome, two had pain and one had obstructive jaundice with pain. The tumor was located in the ampulla of Vater in 5 cases and at the minor papilla in 1 case. All patients underwent a pancreaticoduodenectomy, with histological examination showing tumor diameter varying from 5 to 40 mm and positive lymph nodes. Five patients had a well differentiated endocrine tumor and one a poorly differentiated tumor. All patients had positive Grimelius staining. The secretory profile analyzed by immunohistochemistry was heterogeneous. Median duration of follow-up was 51 months (range: 6 months-16 years) with all patients currently still alive. The patient with a poorly differentiated tumor had diffuse liver metastases, the others were disease-free. CONCLUSION: This study demonstrates the frequency of metastatic spread to adjacent lymph nodes and the inconsistent secretory profiles of these tumors. Pancreaticoduodenectomy may offer long term disease-free survival in well differentiated tumors, and such histology may be useful in advising on prognosis.     

Synchronous double primary cancer of the esophagus and ampulla of Vater successfully treated by a two-stage operation: report of a case

A 68-year-old man was diagnosed with local recurrent cancer of the ampulla of Vater by follow-up endoscopy 3 years after an endoscopic papillectomy. A screening endoscopy found superficial middle thoracic esophageal cancer. The patient required an esophagectomy and pancreatoduodenectomy. We chose a two-stage operation for the esophageal cancer and the local recurrent cancer of the ampulla of Vater, both to reduce surgical invasiveness and to circumvent the lower curability. The first-stage operation consisted of a right transthoracic subtotal esophagectomy with mediastinal and cervical lymph node dissection, external esophagostomy of the neck, and gastrostomy. Forty days after the first surgery, a gastroduodenal artery- and right gastroepiploic vessel-preserving pancreatoduodenectomy with Child’s reconstruction was performed as the second-stage surgery. Esophageal reconstruction was achieved using a gastric tube via the percutaneous route with vascular anastomosis.     

Gastrointestinal stromal tumor of the ampulla of Vater:A case report

Gastrointestinal stromal tumors(GISTs)usually develop in the stomach and small intestine and only rarely occur at the ampulla of Vater,with only 11 cases reported in the literature.We report a case of a GIST of the ampulla of Vater.A 36-year-old,previously healthy man presented with a loss of consciousness lasting a few minutes.A gastroduodenal endoscopy revealed a submucosal tumor with central ulceration at the ampulla of Vater.The enhanced computed tomography scan revealed a smooth-outlined hypervascular solid mass(24 mm×30 mm)in the second part of the duodenum.Neither lymphadenopathy nor metastasis was observed.Magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography showed normal bile and pancreatic ducts.Biopsies were collected from the ulcerative lesion,and the tumor was diagnosed as a GIST.A submucosal tumor with central ulceration may be a characteristic form of GISTs of the ampulla of Vater,and biopsy studies are useful for the diagnosing such tumors.The patient underwent pancreatoduodenectomy,and the operative specimen revealed a 2.2-cm GIST with 1 mitosis per 50 highpower fields.The gold standard for treatment of GISTs is surgical resection without rupture of a capsule.If technically possible,local resection may be considered.However,when the location of the lesion presents challenges,a pancreatoduodenectomy should be performed for GIST of the ampulla of Vater.     

Case of early ampullary cancer treated by endoscopic papillectomy

We herein report a case of ampullary cancer in a 65‐year‐old man who underwent endoscopic papillectomy. Duodenoscopy revealed an exposed‐type tumor mass at the ampulla of Vater. Histology of the biopsy specimen demonstrated well‐differentiated adenocarcinoma. Endoscopic ultrasonography and intraductal ultrasonography showed a hypoechoic mass limited to the ampulla of Vater (clinical stage, T1). Endoscopic papillectomy was performed after informed consent was obtained. Histological examination of the resected specimen revealed adenocarcinoma limited to the ampulla of Vater (final stage, pT1). Both accurate preoperative T staging and proper histological evaluation of the resected specimen appear to justify endoscopic treatment of early ampullary cancer.     

Clinicopathological characteristics of biliary neuroendocrine neoplasms: a multicenter study

Objectives: This study assessed the clinicopathological features, therapeutic approaches, and prognosis of patients with biliary neuroendocrine neoplasm (NENs).

Materials and methods: Multicenter retrospective study of patients with biliary tract NENs in the gallbladder, the extrahepatic bile duct, or the ampulla of Vater between 2005 and 2014.

Results: Total of 43 patients were included in the study. The median age was 62 years (range: 29–84 years) and 58.1% of the patients were male. The tumors occurred in the gallbladder (n?=?11), the extrahepatic bile duct (n?=?5) or the ampulla of Vater (n?=?27). The liver was the most common metastatic site. Based on the 2010 World Health Organization classification, more patients with gallbladder NENs (11/11 (100%)) had neuroendocrine carcinoma G3 than those with NENs in the ampulla of Vater (10/27 (37.1%)). The median progression free survival time (39.3 vs 5.1 months, p?=?0.001) and median overall survival time (46.9 vs 7.9 months, p?<?0.001) were significantly longer in patients with ampulla of Vater NENs than gallbladder NENs. A 2010 World Health Organization classification of neuroendocrine carcinoma G3 was independently related to poor overall survival (hazard ratio (HR), 27.1; 95% confidence intervals (CI), 2.81-260.68; p?=?0.004).

Conclusion: The 2010 World Health Organization classification of neuroendocrine carcinoma G3 was the only factor related to poor prognosis in patients with biliary neuroendocrine neoplasms.     

Can endoscopic resection be applied for early stage ampulla of Vater cancer?

BACKGROUND: Although endoscopic resection can provide a wide tumor resection with a negative resection margin, it is not yet recommended as a curative therapy for ampulla of Vater cancer. METHODS: To investigate the microinvasion rate and the diagnostic accuracy of endoscopic biopsy to properly judge the safety of endoscopic resection for ampulla of Vater cancer. DESIGN: Single-center, retrospective study. SETTING: Academic medical center. PATIENTS: One hundred fifty-nine patients who were finally diagnosed with ampulla of Vater cancer after curative surgical resection. INTERVENTIONS: We surveyed the pathologic concordance rate of endoscopic biopsy and the surgical pathology. For the 36 early stage (Tis or T1) cancers, we surveyed the presence of microlymphovascular invasion, gross appearance (intra-ampullary type, periampullary type, or mixed type), and pathologic subtype (intestinal type or pancreaticobiliary type). MAIN OUTCOME MEASUREMENTS: Presence of microinvasion in early staged ampulla of Vater cancer. RESULTS: Endoscopic biopsy failed to reveal malignancy in 15.9% of the 126 cases. Microlymphovascular invasion was present in 17 cases (56.7%) of the 30 T1 cancers but was absent in all cases of the 6 Tis cancers (P = .02). Neither the gross appearance (P = .51) nor the pathologic subtype (P = .28) could predict the microinvasion rate. LIMITATIONS: Single-center, retrospective study with small number of patients. CONCLUSIONS: Although endoscopic resection improves the low predictability of endoscopic biopsy, surgical resection should be performed for the T1 stage ampulla of Vater cancer because of the high lymphovascular invasion rate. On the other hand, the safety of endoscopic resection should be evaluated by a large-scale study on Tis cancers to consider the absence of microinvasion.     

Incidental Small Ampullary Somatostatinoma Treated with Ampullectomy 2 Years After Diagnosis

Somatostatinomas are rare tumors; ampullary somatostatinomas are very rare. We report a case of a small pure somatostatin-producing neuroendocrine tumor of ampulla of Vater in a 54-year-old woman with neither neurofibromatosis nor somatostatinoma syndrome, “incidentally” discovered during an abdominal computed tomography. The patient initially refused other adjunctive exams but after 2 years she was admitted, presenting with itch, night sweats, severe fatigue, and unintentional weight loss. The size of the tumor (1.5 cm) and the other radiologic findings had not changed since the abdominal CT scan 2 years before. The somatostatin, gastrin, glucagons, serotonin, vasoactive intestinal peptide, dopamine, norepinephrine, epinephrine, and calcitonin plasma levels were normal. ERCP-obtained biopsies revealed a neuroendocrine tumor with psammoma bodies; immunohistochemical profile was positive for chromogranin and somatostatin. The patient underwent surgery; intraoperative histologic examination of lymph nodes sampling of perihepatic and periduodenal lymph nodes was negative for metastasis. We performed, therefore, a transduodenal ampullectomy. The patient continues to do well at 3 years’ follow-up with no evidence of local or distance recurrence of disease.     

A case report: endoscopic enucleation of gastrointestinal stromal tumor of the ampulla of Vater

Objective

To report a case of endoscopic enucleation of a gastrointestinal stromal tumor of the ampulla of Vater.

Design

The tumor resection was performed by needle knife endoscopic submucosal dissection.

Results

The tumor was resected completely without serious complications and the patient recovered rapidly.

Conclusion

Endoscopic enucleation may be an alternative treatment for gastrointestinal stromal tumors of the ampulla of Vater for patients who fulfill certain requirements.     

Coexistence of small cell neuroendocrine carcinoma and villous adenoma in the ampulla of Vater

Small cell neuroendocrine carcinoma of the ampulla of Vater is extremely rare and different from the common ampullary adenocarcinoma. The ampullary adenoma is also a rare neoplasm and has the potential to develop an adenocarcinoma. Their coexistence has been rarely reported in the literature. We herein describe an unusual case of a small cell neuroendocrine carcinoma associated with a villous adenoma in the ampulla of Vater with emphasis on computed tomography （CT） and histopathological findings. We also discuss their clinical, histopathological and radiological features as well as possible histogenesis.     

Endoscopic sphincterotomy in patients with stenosis of ampulla of Vater： Three-year follow-up of exocrine pancreatic function and clinical symptoms

AIM: To investigate retrospectively the long-term effect of endoscopic sphincterotomy (ES) including exocrine pancreatic function in patients with stenosis of ampulla of Vater. METHODS: After diagnostic endoscopic retrograde cholangiopancreatography (ERCP) and ES because of stenosis of the ampulla of Vater (SOD Type Ⅰ), follow-up examinations were performed in 60 patients (mean follow-up time 37.7 mo). Patients were asked about clini-cal signs and symptoms at present and before interven-tion using a standard questionnaire. Before and after ES exocrine pancreatic function was assessed by determina-tion of immunoreactive fecal elastase 1. Serum enzymes indicating cholestasis as well as serum lipase and amy-lase were measured. RESULTS: Eighty percent of patients reported an im-provement in their general condition after ES. The fecal elastase 1 concentrations (FEC) in all patients increased significantly after ES. This effect was even more marked in patients with pathologically low concentrations (< 200 μg/g) of fecal elastase prior to ES. The levels of serum lipase and amylase as well as serum alcaline phosphatase (AP) and gamma-glutamyltranspeptidase (GGT) decreased signifi-cantly after ES. CONCLUSION: The results of this study demonstrate that patients with stenosis of the ampulla of Vater can be successfully treated with endoscopic sphincterotomy. The positive effect is not only indicated by sustained improvement of clinical symptoms and cholestasis but also by improvement of exocrine pancreatic function.     

Mixed adenoneuroendocrine carcinoma of the ampulla:Two case reports

Mixed adenoneuroendocrine carcinoma(MANEC)is a malignant tumor with adenocarcinoma and neuroendocrine components,with≥30%of each component required.MANEC of the ampulla is rare.To the best of our knowledge,only 15 cases of MANEC of the ampulla have been reported in the English-language literature.Here,we report two cases of MANEC of the ampulla in two womenaged 43 and 60 years,which was confirmed by histology after pancreaticoduodenectomy.These tumors contained neuroendocrine and adenocarcinoma components.The neuroendocrine components were positive for chromogranin A(Cg A),synaptophysin(Syn)and CD56 by immunostaining.The adenocarcinoma components were negative for Cg A,Syn and CD56.Both cases were T3N0M0(StageⅢA).They survived for 15 and 20 mo after surgery,respectively.A brief discussion about the histopathological features,clinical behavior and treatment of MANEC of ampulla,and review of the relevant literature are presented.     

Unusual manifestation of an ampullary tumor presenting with severe upper gastrointestinal bleeding.

Ampullary tumors can occasionally ulcerate and present as frank gastrointestinal bleeding. The most common clinical presentation is jaundice like in other tumors of the biliary tree. We report on a 68-year-old man who presented with severe upper gastrointestinal hemorrhage secondary to an asymptomatic mass of the ampulla of Vater. An endoscopic biopsy specimen revealed a villous adenoma with moderate dysplasia. A curative resection was performed, and pathological work-up revealed the presence of an infiltrating, moderately differentiated ampullary adenocarcinoma.