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.     

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.     

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.     

Primary gastrointestinal stromal tumor of the liver： A case report

We report a case of primary gastrointestinal stromal tumor （GIST） of the liver. A 17-year-old man with a solid mass in the anterior segment of the right liver was asymptomatic with negative laboratory examinations with the exception of positive HBV. Contrast-enhanced ultrasound （CEUS） revealed a hypervascular lesion in the arterial phase and hypoechoic features during the portal and late phases. However, enhanced spiral computed tomography （CT） showed hypoattenuation in all three phases. Following biopsy, immunohistochemical evaluation demonstrated positive CDl17. Different imaging features of primary GISTs of the liver are due to pathological properties and different working systems between CEUS and enhanced spiral CT.     

A rare case of periampullary carcinoma with ectopic ending of Vater＇s ampulla

A 71-year-old woman was referred to our department complaining of painless progressive jaundice for the last 3 too. Magnetic resonance imaging and magnetic resonance cholangiopancreatography （MRCP） showed the ectopic hepatopancreatic ampulla draining into the fourth part of the duodenum adjacent to the duodenojejunal flexure; the irregular morphology of the duodenojejunal flexure likely due to a soft tissue mass. Laparotomy confirmed the presence of the abnormal ampulla of Vater located at the fourth part of the duodenum and a soft tissue tumor about 6 cm × 5 cm×5 cm with a peduncle adjoining the ampulla. Resection of the tumor, including some peripheral tissue, and a Roux-Y loop anastomosis choledochojejunostomy were performed. Pathological examination indicated an intestinal villous adenoma accompanied by severe dysplasia and focal canceration. Periampullary carcinoma with ectopic ending of the Vater＇s ampulla into the fourth part of the duodenum is rather rare. The embryonic genetic background of this anomaly has not yet been fully explained. It is worth mentioning that MRCP is useful for demonstrating anomalies and anatomic variants of the biliary tract system and pancreatic duct.     

Bone and brain metastases from ampullary adenocarcinoma

Ampullary carcinoma is the second most common cancer of the peri-ampullary area after pancreatic carcinoma and metastasizes mostly intra-abdominally and to the liver. Extra-abdominal metastases are less frequent. In this report we describe the case of a patient with resected adenocarcinoma of the ampulla of Vater who developed skeletal metastases in the lower extremity and brain metastases. We briefly discuss aspects of this comparatively rare gastrointestinal malignancy.     

Delayed gastric emptying is associated with pylorus-preserving but not classical Whipple pancreaticoduodenectomy： A review of the literature and critical reappraisal of the implicated pathomechanism

INTRODUCTION The introduction of partial pancreaticoduodenectomy for the treatment of carcinoma of the ampulla of Vater dates back to almost a century ago and is credited to Kausch, a German surgeon from Berlin[1]. Then, in 1935 Whipple and associates red…     

Biliary phytobezoar resulting in intestinal obstruction

Phytobezoar is the most common type of bezoar.It is composed of indigestible vegetable matter and is usually found in the stomach.Biliary phytobezoar is extremely rare and difficult to diagnose preoperatively.The pathogenesis is not clear,and there have been only a few reports of biliary bezoars associated with sphincteric impairmentat the ampulla of Vater.Here,we present a report of biliary bezoar that resulted in jejunal obstruction.We were unable to identifythe bezoar in the extrahepatic bile duct until it obstructed the small bowel lumen.To our knowledge,this is the first report of small bowel obstruction resulting frommigration of a biliary bezoar.     

Composite neuroendocrine and adenomatous carcinoma of the papilla of Vater

Malignant tumors of papilla are usually adenocarcinomas. We present a 67-year-old female who became icteric as result of a malignant tumor infiltrating the papilla of Vater. Histopathological assessment of surgically excised tumor showed both neuroendocrine and adenocarcinomatous features. To our knowledge, this is the seventh report of this rare neoplastic association in the duodenal periampullary region.     

KL-6 mucin expression in carcinoma of the ampulla of Vater： Association with cancer progression

AIM: To assess histochemical expression of KL-6 and its clinicopathological significance in carcinoma of the ampulla of Vater. METHODS: Ampullary carcinoma tissues were collected from 38 patients who underwent pancreatoduodenectomy or local resection. Tissues were subjected to immunohistochemical analysis using KL-6 antibody. RESULTS: Positive staining of ampullary carcinoma cells was observed in 26 (68.4%) cases. Staining was not found in the surrounding non-cancer regions of the ampullary tissues. Remarkable KL-6 expression was observed in invasive carcinoma cells in pancreatic and duodenal tissues and in metastatic carcinoma cells in lymph nodes. Positive KL-6 expression was related to lymph node metastasis (P=0.020), pancreatic invasion (P=0.016), duodenal invasion (P= 0.034), and advanced stage of TNM clinical classification (P=0.010). Survival analysis showed that positive expression of KL-6 was related to a poorer prognosis (P=0.029). CONCLUSION: The aberrant expression of KL-6 mucin is significantly related to unfavorable behaviors of carcinoma of the ampulla of Vater.     

Signet ring cell carcinoma of the Ampulla of Vater: outcomes of patients in the United States

BackgroundSignet ring cell carcinoma (SRCC) of the ampulla of Vater is poorly understood, with approximately 22 reported cases. Our study sought to create a comprehensive review of cases in the United States.MethodsWe used the Surveillance, Epidemiology, and End Results Program to collect all cases of ampullary adenocarcinoma diagnosed between 2010 and 2015.ResultsThe age-adjusted incidence rate of SRCC of the ampulla of Vater was 1.2 cases per 10,000,000 persons per year, with 50% more cases in males than females. We identified 3448 cases of adenocarcinoma of the ampulla of Vater, 81 of which were SRCC (2.3%). SRCC tended to present a later stage than other ampullary cancers, with median survival times of 17 vs. 25 months, (p = 0.07). Survival was significantly worse for SRCC when accounting for other clinical features (HR 1.46, p = 0.01). Factors portending worse prognosis in SRCC of the ampulla of Vater were advanced age, late stage and lack of surgical intervention.ConclusionOur study represents the largest study of SRCC of the ampulla of Vater to date. SRCC has a poorer prognosis compared with other ampullary cancers. Optimal treatment regimen is the most important future area of study.     

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.     

Signet ring cell carcinoma of the ampulla of Vater

Introduction

Signet ring cell carcinoma of the ampulla of Vater is a rare entity and less than 20 cases have been described in the literature. We report the cases of two patients with this disease and provide a literature review of previous studies.

Case report

We describe two patients with obstructive jaundice. Abdominal ultrasonography and abdominal computed tomography showed dilatation of the intrahepatic and common bile duct. Duodenoscopy indicated a protruding mass on the ampulla of Vater. Histopathological examination showed round cells and their nuclei were located on one side with prominent signet-ring features. One patient underwent a cephalic pancreatoduodenectomy with lymphadenectomy and the other a total pancreatectomy.

Discussion

Signet ring cell carcinoma of the ampulla of Vater has only been described in isolated cases in the literature. Therefore, the clinicopathological features and prognosis of this disease have not yet been well defined.     

Histamine-producing neuroendocrine tumor of the ampulla of Vater

Neuroendocrine tumors of the ampulla of Vater are rare (less than 100 cases reported). We report here a new case characterized by histamine secretion, a hitherto unreported feature. Clinical presentation is similar to that of other tumors of the ampulla of Vater. In our observation, the patient had noticed urticaria on the right forearm for several months. Tumor of the ampulla was confirmed by endoscopic ultrasonography, while neuroendocrine characterization was assessed on biopsies after endoscopic sphincterotomy. Histamine concentration in blood was the only elevated neuroendocrine marker and returned to normal after surgical resection. Histology showed a neuroendocrine tumor with extension to lymph nodes. On immunohistochemical analysis, production of histamine was confirmed, and the diagnosis of mastocytoma was eliminated. In view of the literature, neuroendocrine tumors of the ampulla of Vater are associated with a good prognosis (5 year-survival rate: 90%) despite early lymph node involvement.     

Expression of hedgehog proteins in periampullary cancer]

BACKGROUND/AIMS: Hedgehog protein is an essential molecule for gastrointestinal tract development, and disruption of hedgehog signaling pathway is linked to some gastrointestinal tumorigenesis. Here, we performed hedgehog immunostaining in periampullary cancer to evaluate the differences according to the location type of cancer and the differentiation of adenocarcinoma. METHODS: We retrieved surgical specimens from 43 periampullary cancer patients (15 ampulla of Vater cancer, 12 distal common bile duct cancer, 13 pancreatic head cancer, and 3 combined ampulla of Vater/bile duct cancer). Immunohistochemical stain was performed in both normal and cancerous tissue portions of each case using Sonic hedgehog (H-160) rabbit polyclonal antibody. Immunohistochemical stain results were grouped into three groups according to the percentage of positive cytoplasmic stain in tumor volume (unstained: <5%, weakly stained: 5-50%, and strongly stained: >50%). RESULTS: All of the normal tissue revealed negative immunohistochemical stain while cancerous tissue revealed positivity in 95.3% (41/43 cases). Strongly stained cases were more frequently seen in ampulla of Vater cancers (13/15) and in combined ampulla of Vater/bile duct cancers (3/3) than in distal common bile duct cancers (4/12) and in pancreatic head cancers (3/13) (p=0.002). In addition, strongly stained cases were more frequently seen in well-differentiated adenocarcinoma than the others (p<0.001). CONCLUSIONS: Most of the periampullary cancers show hedgehog protein expression. In addition, hedgehog protein immunostainings shows stronger expression in ampulla of Vater cancers and in well-differentiated adenocarcinoma.     

Transduodenal ampullectomy in the treatment of villous adenomas and adenocarcinomas of the Vater's ampulla.

INTRODUCTION: Adenomas are the most frequent tumors of the Vater s ampulla. Their capacity for malignant transformation following the adenoma-carcinoma sequence is well known. It is because of this that resection after diagnosis is required. The identification of the appropriate technique according to tumor features would require that patients not be undertreated or overtreated, which would give rise to serious consequences derived from their location. PATIENTS AND METHODS: Villous adenomas and adenocarcinomas of the Vater s ampulla candidates for local resection were revised from January 1st, 1998 through June 30th, 2003. We describe the methods of diagnosis and ampulectomy techniques we performed. RESULTS: We performed an ampulectomy by first intention in all 8 patients included in this study. However, pancreatoduodenectomy was necessary in two patients because of the closeness of resection margins. We had no mortality in this series, and morbidity was limited to two episodes of digestive bleeding that were controlled by electrocoagulation and embolization. The mean follow-up was 28.5 months (range, 6-72 months). CONCLUSIONS: The difficulty of precise preoperatory diagnosis in adenomas of the Vater s ampulla demands resection after identification. Ampulectomy is the treatment of choice for villous adenomas and T1 adenocarcinomas, with 1 cm of resection margin to avoid local recurrence.     

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.     

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.