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A palpable pancreatic duct
ARTICLE IN PRESS
A palpable pancreatic duct Jonathan M. Hernandez, MD, Zachary J. Brown, DO, and Michael I. D’Angelica, MD, New York, NY
From the Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY
A 21-YEAR-OLD MAN presented with a 1-year history of intermittent abdominal pain associated with a 20pound weight loss as well as an enlarging painful right-sided abdominal mass throughout the past month. He had no known medical problems and had not undergone an operation previously. His family history was significant for a paternal aunt with pancreatic cancer. On physical examination, a firm tender mass occupied the right upper quadrant (Fig 1, A). Laboratory tests included an alkaline phosphatase of 421 U/L, aspartate aminotransferase 229 U/L, alanine transaminase 109 U/L, and total bilirubin 0.6 mg/dL. A computed tomography scan was performed and demonstrated a large fluid-filled structure occupying the right hemiabdomen (Fig 1, B). This cystic structure was in fact a massively dilated
pancreatic duct (star), which on physical examination was palpable, secondary to ampullary mass (black arrows) that extended into the duodenum (white arrows, Fig 1, B). The patient underwent an upper endoscopy and endoscopic ultrasound, which revealed a 4-cm mass in the duodenum arising from the ampulla. The patient underwent pancreaticoduodenectomy to remove the tumor (Fig 2, A). Gross inspection of the resected specimen revealed a bifid ampullary tumor extending into the duodenum through both the major and minor papilla (Fig 2, B). The resulting blockage of the pancreatic ductal system caused dilatation of the main duct to 17 cm in cross-sectional diameter with accompanying atrophy of the glandular tissue. Histopathologic review revealed invasive adenocarcinoma of a pancreato-
Fig 1. (A) Physical examination findings of palpable mass in the right upper quadrant of the abdomen (star). (B) Computed tomography scan with a large fluid-filled structure occupying the right hemiabdomen, which was found to be a massively dilated pancreatic duct (star), secondary to an ampullary mass (black arrows) that extended into the duodenum (white arrows). GB, Gallbladder.
Accepted for publication July 26, 2017.
0039-6060/$ - see front matter
Reprint requests: Michael I. D’Angelica, MD, 1275 York Ave, New York, NY 10065. E-mail: [email protected].
Ó 2017 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.surg.2017.07.018
Surgery 2017;j:j-j.
SURGERY 1
ARTICLE IN PRESS 2 Hernandez, Brown, and D’Angelica
Surgery j 2017
Fig 2. (A) A massively dilated pancreatic duct (star) with surrounding atrophic gland was encountered during pancreatic transection. (B) Gross pathology specimen with dilated pancreatic duct (star) and ampullary tumor, duodenum removed (box). (C) Histologic finding of an ampullary tumor of pancreatobiliary phenotype protruding into duodenum (white arrows). SMV, Superior mesenteric vein; IVC, inferior vena cava.
biliary phenotype, arising in an intra-ampullary papillary-tubular neoplasm (Fig 2, C). In addition, 1 of 13 lymph nodes were found to contain metastatic adenocarcinoma. DISCUSSION Periampullary cancers include tumors of the distal common bile duct and proximal pancreas, as well as the ampullary complex.1 Ampullary cancer is an uncommon malignancy accounting for z0.2% of all gastrointestinal malignancies and 6% of periampullary tumors.2 Cancers of the ampulla of Vater are thought to arise from either intestinal or biliary epithelia. Intestinal-type adenocarcinoma originates from the intestinal lining of
the ampulla and tends to progress through the adenoma-carcinoma sequence and is associated with a better prognosis as compared to the pancreaticobiliary subtype.2 The pancreatobiliary subtype originates from the distal common bile duct or pancreatic duct and may be associated with pancreatic intraepithelial neoplasm lesions.2 Despite this ostensibly binary delineation, most tumors will be of mixed histology. Moreover, assignment of subtype is likely pathologist dependent, further complicating efforts to integrate ampullary carcinoma subtype into management algorithms. For example, in an evaluation of 232 cases of ampullary carcinoma reviewed by 5 independent pathologists, 40% of cases were classified as mixed
ARTICLE IN PRESS Surgery Volume j, Number j
histology and complete agreement by all observers was present in less than a quarter of the cases.3 Irrespective of the ampullary carcinoma subtype, resection is the preferred treatment modality for localized disease. Although not adequately studied in any meaningful way, it is worth noting that neoadjuvant chemoradiation resulted in a downstaging (67%) and complete pathologic response (23%) in a very small reported cohort of patients (n = 18).4 The application of criteria for receipt of neoadjuvant therapy for patients with pancreatic carcinoma can probably be applied to patients with ampullary carcinoma. The decision for adjuvant therapy is based more firmly on data. The ESPAC-3 trial demonstrated a survival advantage in the ampullary cancer subgroup of 70.8 months versus 40.6 months in patients who received adjuvant gemcitabine s compared with the observation.5 However, the question regarding the most appropriate chemotherapeutics (or combination) to use after resection remains unanswered.
Hernandez, Brown, and D’Angelica 3
REFERENCES 1. Ahn DH, Bekaii-Saab T. Ampullary cancer: an overview. American Society of Clinical Oncology educational book American Society of Clinical Oncology Meeting. 2014: 112-115. 2. Okano K, Oshima M, Yachida S, Kushida Y, Kato K, Kamada H, et al. Factors predicting survival and pathological subtype in patients with ampullary adenocarcinoma. J Surg Oncol 2014;110:156-62. 3. Reid MD, Balci S, Ohike N, Xue Y, Kim GE, Tajiri T, et al. Ampullary carcinoma is often of mixed or hybrid histologic type: an analysis of reproducibility and clinical relevance of classification as pancreatobiliary versus intestinal in 232 cases. Mod Pathol 2016;29: 1575-85. 4. Palta M, Patel P, Broadwater G, Willett C, Pepek J, Tyler D, et al. Carcinoma of the ampulla of Vater: patterns of failure following resection and benefit of chemoradiotherapy. Ann Surg Oncol 2012;19:1535-40. 5. Neoptolemos JP, Moore MJ, Cox TF, Valle JW, Palmer DH, McDonald AC, et al. Effect of adjuvant chemotherapy with fluorouracil plus folinic acid or gemcitabine vs observation on survival in patients with resected periampullary adenocarcinoma: the ESPAC-3 periampullary cancer randomized trial. JAMA 2012;308:147-56.
A palpable pancreatic duct Jonathan M. Hernandez, MD, Zachary J. Brown, DO, and Michael I. D’Angelica, MD, New York, NY
From the Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY
A 21-YEAR-OLD MAN presented with a 1-year history of intermittent abdominal pain associated with a 20pound weight loss as well as an enlarging painful right-sided abdominal mass throughout the past month. He had no known medical problems and had not undergone an operation previously. His family history was significant for a paternal aunt with pancreatic cancer. On physical examination, a firm tender mass occupied the right upper quadrant (Fig 1, A). Laboratory tests included an alkaline phosphatase of 421 U/L, aspartate aminotransferase 229 U/L, alanine transaminase 109 U/L, and total bilirubin 0.6 mg/dL. A computed tomography scan was performed and demonstrated a large fluid-filled structure occupying the right hemiabdomen (Fig 1, B). This cystic structure was in fact a massively dilated
pancreatic duct (star), which on physical examination was palpable, secondary to ampullary mass (black arrows) that extended into the duodenum (white arrows, Fig 1, B). The patient underwent an upper endoscopy and endoscopic ultrasound, which revealed a 4-cm mass in the duodenum arising from the ampulla. The patient underwent pancreaticoduodenectomy to remove the tumor (Fig 2, A). Gross inspection of the resected specimen revealed a bifid ampullary tumor extending into the duodenum through both the major and minor papilla (Fig 2, B). The resulting blockage of the pancreatic ductal system caused dilatation of the main duct to 17 cm in cross-sectional diameter with accompanying atrophy of the glandular tissue. Histopathologic review revealed invasive adenocarcinoma of a pancreato-
Fig 1. (A) Physical examination findings of palpable mass in the right upper quadrant of the abdomen (star). (B) Computed tomography scan with a large fluid-filled structure occupying the right hemiabdomen, which was found to be a massively dilated pancreatic duct (star), secondary to an ampullary mass (black arrows) that extended into the duodenum (white arrows). GB, Gallbladder.
Accepted for publication July 26, 2017.
0039-6060/$ - see front matter
Reprint requests: Michael I. D’Angelica, MD, 1275 York Ave, New York, NY 10065. E-mail: [email protected].
Ó 2017 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.surg.2017.07.018
Surgery 2017;j:j-j.
SURGERY 1
ARTICLE IN PRESS 2 Hernandez, Brown, and D’Angelica
Surgery j 2017
Fig 2. (A) A massively dilated pancreatic duct (star) with surrounding atrophic gland was encountered during pancreatic transection. (B) Gross pathology specimen with dilated pancreatic duct (star) and ampullary tumor, duodenum removed (box). (C) Histologic finding of an ampullary tumor of pancreatobiliary phenotype protruding into duodenum (white arrows). SMV, Superior mesenteric vein; IVC, inferior vena cava.
biliary phenotype, arising in an intra-ampullary papillary-tubular neoplasm (Fig 2, C). In addition, 1 of 13 lymph nodes were found to contain metastatic adenocarcinoma. DISCUSSION Periampullary cancers include tumors of the distal common bile duct and proximal pancreas, as well as the ampullary complex.1 Ampullary cancer is an uncommon malignancy accounting for z0.2% of all gastrointestinal malignancies and 6% of periampullary tumors.2 Cancers of the ampulla of Vater are thought to arise from either intestinal or biliary epithelia. Intestinal-type adenocarcinoma originates from the intestinal lining of
the ampulla and tends to progress through the adenoma-carcinoma sequence and is associated with a better prognosis as compared to the pancreaticobiliary subtype.2 The pancreatobiliary subtype originates from the distal common bile duct or pancreatic duct and may be associated with pancreatic intraepithelial neoplasm lesions.2 Despite this ostensibly binary delineation, most tumors will be of mixed histology. Moreover, assignment of subtype is likely pathologist dependent, further complicating efforts to integrate ampullary carcinoma subtype into management algorithms. For example, in an evaluation of 232 cases of ampullary carcinoma reviewed by 5 independent pathologists, 40% of cases were classified as mixed
ARTICLE IN PRESS Surgery Volume j, Number j
histology and complete agreement by all observers was present in less than a quarter of the cases.3 Irrespective of the ampullary carcinoma subtype, resection is the preferred treatment modality for localized disease. Although not adequately studied in any meaningful way, it is worth noting that neoadjuvant chemoradiation resulted in a downstaging (67%) and complete pathologic response (23%) in a very small reported cohort of patients (n = 18).4 The application of criteria for receipt of neoadjuvant therapy for patients with pancreatic carcinoma can probably be applied to patients with ampullary carcinoma. The decision for adjuvant therapy is based more firmly on data. The ESPAC-3 trial demonstrated a survival advantage in the ampullary cancer subgroup of 70.8 months versus 40.6 months in patients who received adjuvant gemcitabine s compared with the observation.5 However, the question regarding the most appropriate chemotherapeutics (or combination) to use after resection remains unanswered.
Hernandez, Brown, and D’Angelica 3
REFERENCES 1. Ahn DH, Bekaii-Saab T. Ampullary cancer: an overview. American Society of Clinical Oncology educational book American Society of Clinical Oncology Meeting. 2014: 112-115. 2. Okano K, Oshima M, Yachida S, Kushida Y, Kato K, Kamada H, et al. Factors predicting survival and pathological subtype in patients with ampullary adenocarcinoma. J Surg Oncol 2014;110:156-62. 3. Reid MD, Balci S, Ohike N, Xue Y, Kim GE, Tajiri T, et al. Ampullary carcinoma is often of mixed or hybrid histologic type: an analysis of reproducibility and clinical relevance of classification as pancreatobiliary versus intestinal in 232 cases. Mod Pathol 2016;29: 1575-85. 4. Palta M, Patel P, Broadwater G, Willett C, Pepek J, Tyler D, et al. Carcinoma of the ampulla of Vater: patterns of failure following resection and benefit of chemoradiotherapy. Ann Surg Oncol 2012;19:1535-40. 5. Neoptolemos JP, Moore MJ, Cox TF, Valle JW, Palmer DH, McDonald AC, et al. Effect of adjuvant chemotherapy with fluorouracil plus folinic acid or gemcitabine vs observation on survival in patients with resected periampullary adenocarcinoma: the ESPAC-3 periampullary cancer randomized trial. JAMA 2012;308:147-56.