Two Consecutive Cases of Ampulla of Vater Cancer Combined with Annular Pancreas and Unusual Anatomic Variation

Annular pancreas is a rare congenital anomaly that consists of a ring of pancreatic tissue partially or completely encircling the descending portion of the duodenum. Coexisting ampullary carcinoma in annular pancreas combined with anomaly of hepatic artery or bile duct are thought to be extremely rare. Two consecutive cases of ampullary carcinoma in annular pancreas with bile duct or hepatic artery anomaly are described. In addition, English literature reports of coexisting ampullary carcinoma in annular pancreas are summarized. Clinical symptoms of the two patients were jaundice and abdominal discomfort. The two ampullary cancers were early adenocarcinomas in the ampulla of Vater that were curatively treated by pylorus preserving pancreaticoduodenectomy. Ampullary carcinoma associated with annular pancreas is rare. Its combination with an additional biliary or hepatic artery anomaly make our cases extremely unique. Certain aberrant events in the overall stages of the development of the liver, bile duct, and pancreas may have occurred in these patients. Surgeons need to note preoperatively these possible associated anatomic variations.

A Case of Signet-ring Cell Carcinoma of the Ampulla of Vater / 대한소화기내시경학회지

Signet-ring cell carcinoma of the ampulla of Vater is so rare that only 16 cases have been reported. Here we report a case of a 47-year-old man with signet-ring cell carcinoma in the ampulla of Vater. The patient was hospitalized for right upper quadrant abdominal pain and jaundice which had developed 10 days prior. Computed tomography (CT) and ultrasonography (US) revealed dilatation of the common bile duct and the intrahepatic bile duct. On duodenoscopy, a bulging mass with an irregular margin in the major papilla was noted. Histologic findings showed a signet-ring cell carcinoma. Surgical resection was not done because lymph node metastasis was noted during the operation. Hence, conservative treatment including chemotherapy and bile drainage was done.

Concurrent occurrence of adenocarcinoma and neuroendocrine type small cell carcinoma in the ampulla of Vater / 대한내과학회지

Small cell carcinoma in the ampulla of Vater is rare, and the concurrent occurrence of adenocarcinoma and small cell carcinoma is extremely rare. We report the case of a double primary tumor, adenocarcinoma and small cell carcinoma, in the ampulla of Vater.

Ki-67 and VEGF as Possible Prognostic Factors in Radically-resected Ampulla of Vater Cancer

PURPOSE: Ampulla of Vater cancer has a more favorable prognosis and survival than other malignant periampullary tumors. The pathologic staging of ampulla of Vater carcinoma is a key determinant of the patient's prognosis. However, we have often encountered patients in whom the course of their disease differed substantially from what would be predicted based on their clinical staging, which highlights the need to consider additional predictive factors. We applied an immunohistochemical technique to examine the expression of Ki-67 and VEGF in radicallyresected ampulla of Vater carcinomas, and then compared the status of expression with several clinicopathologic factors. METHODS: Sixty-four patients who underwent curative resection for ampulla of Vater cancer between January 1992 and December 2006 at the Yonsei University College of Medicine were reviewed. The relationships between the expression of molecular markers and clinicopathologic factors were determined. RESULTS: There was no relationship between the clinicopathologic characteristics and the expression of molecular markers in patients with ampulla of Vater cancer. Among the clinicopathologic characteristics, lymph node metastasis was identified as an independent factor of survival after curative resection for ampulla of Vater carcinoma. CONCLUSION: Measurement of Ki-67 and VEGF in patients with ampulla of Vater carcinoma may have an important role in identifying the poor prognostic group.

The Differential Diagnosis and Prognosis of an Ampulla of Vater Cancer with a Grossly Normal Appearance / 대한소화기내시경학회지

BACKGROUND/AIMS: The purpose of this study was to determine the clinical parameters for a differential diagnosis between a malignant and benign stricture of the ampulla of Vater with a grossly normal appearance and to evaluate the diagnostic accuracy of the use of an endoscopic biopsy for the prognosis of ampulla of Vater cancers. METHODS: Medical records and cholangiograms were retrospectively reviewed. In order to determine the clinical parameters useful for a differential diagnosis, clinical manifestations, laboratory findings and the common bile duct diameter were compared between malignant and benign strictures. The diagnostic accuracy of the use of an endoscopic biopsy and the clinical features of patients with an ampulla of Vater cancer were analyzed. The survival rate after management was also evaluated. RESULTS: Nine patients with a benign stricture and 15 patients with a malignant stricture were included in this study. The levels of serum bilirubin, alkaline phosphatase and alanine aminotransferase were significantly higher in patients with a malignant stricture than in patients with a benign stricture (p<0.05). Of the patients who underwent surgical management, 93% had stage I or II disease. All of the patients were alive up to a mean follow-up period of 50 months. CONCLUSIONS: Liver function tests and the use of an endoscopic biopsy are useful in the differential diagnosis of a stricture of the ampulla of vater with a grossly normal appearance. Cancer patients typically present with an early stage and the prognosis is relatively good.

Photodynamic Therapy for Superficial Gastrointestinal Tumors / 대한소화기내시경학회지

BACKGROUND/AIMS: Although photodynamic therapy (PDT) has been used for the endoscopic treatment of digestive cancer, its curative efficacy remains uncertain. This study evaluated the curative role of PDT in superficial gastrointestinal cancer. METHODS: Fifteen lesions in 14 patients with a histologically proven carcinoma (early esophageal cancer 6, early gastric cancer 8, ampulla of Vater cancer 1) were injected with an intravenous hematoporphyrin derivative (2 mg/kg), and PDT was performed 48 hours later. The response to treatment was assessed by gastroscopy with biopsies. RESULTS: The median follow-up time was 273 days (42~1,030 days). According to the TNM stage of endoscopic ultrasonography, there were 14 T1 cases and 1 T2 case. Complete remission was observed in 13 cases after the initial and consecutive PDT. There were 2 cases of failure. The recurrence rate was 15.4% (2/13), and the median time from the initial PDT to recurrence was 349 days. CONCLUSIONS: PDT using a hematoporphyrin derivative as a photosensitizer is a safe and efficient method for treating early cancer. However, a long-term follow up period using a large population sample will be needed for confirmation.

Recurrence Patterns and Risk Factors of Early Recurrence after Curative Resection of Ampulla of Vater Cancer

PURPOSE: Carcinomas of the ampulla of Vater have a higher resection rate, lower recurrence rate and more favorable prognosis than other malignant tumors of the periampullary region. This is because they usually presents with symptoms at an early stage due to their special anatomic location. Because of their relative low incidence, there have been few reports on the patterns and risk factors of a recurrence after a curative resection. The aim of this study was to evaluate the patterns and risk factors of a recurrence after a curative resection of ampulla of Vater cancer. METHODS: The medical records of 87 regularly followed-up patients from a total of 102 patients with a carcinoma of the ampulla of Vater, who had undergone a curative surgery between January 1992 and December 2002, were retrospectively reviewed. Of these patients, 37 were diagnosed as recurrent, with the patterns and clinicopathological risk factors of the recurrence analyzed. RESULTS: The average time to recurrence after a curative resection was 29.3+/-35.3 months, with liver metastasis being the most frequent (40.5%). The mean age of the patients in which a recurrence occurred was 54.9+/-10.1 years. There were 18 male and 19 female patients. The average tumor size in the recurred group was 2.5+/-2.0 cm. The recurrences were classified as early and late if they occurred within 18 months and after 18 months, respectively. The risk factors affecting an early recurrence were the gross morphology of tumor(ulcer formation) and cell differentiation (poorly differentiated). A univariate analysis showed lymph node metastasis to be a statistically significant risk factor, but age, gender, degree of tumor invasion depth and cell differentiation were not statistically significant. CONCLUSION: Lymph node metastasis is the most important risk factor affecting a recurrence after a curative resection of ampulla of Vater cancer, with the tumor gross morphology and cell differentiation important factors in an early recurrence. Further study on postoperative chemotherapy or radiotherapy in patients with lymph node metastasis, an ulcerative tumor type or poorly differentiated cell type, with a long-term follow-up on many patients will be needed.

Transduodenal Local Resection for Low Risk Group Ampulla of Vater Cancer Patients

PURPOSE: A carcinoma of the ampulla of Vater has more favorable prognosis than other malignant tumors of the periampullary region, because it is symptomatic at an early stage. However, local resection of an ampullary carcinoma remains controversial. The aim of this study was to evaluate the treatment results of ampulla of Vater carcinomas, according to operation type in low risk group patients. METHODS: The records of 17 low risk group patients, among 120 patients with ampulla of Vater cancer, who underwent curative surgery beyween 1992 and 2002, were reviewed. All specimens were critically reviewed by a single expert pathologist. The relationship between surgical outcomes and operation type were assessed. RESULTS: There were 10 men and 7 women, with a median age of 57.8 years. 13 of the 17 patients underwent the Whipples operation or a PPPD, and 4 underwent a transduodenal local resection (TDLR). The operation time was shorter in the TDLR group, and was statistically significant. Among the 17 patients, only one had a recurrence in the inguinal area 33 months after the PD. CONCLUSION: Transduodenal local resection is a recommendable operation for low risk Ampulla of Vater cancer patients. During the operation, it is essential to accurately evaluate the depth of invasion, cell differentiation and positivity of the resection margin using frozen sections.