ELECTRONIC CLINICAL CHALLENGES AND IMAGES IN GI Not Every Ampullary Mass Is Deadly Joseph D. Feuerstein,1 Eric U. Yee,2 and Douglas Pleskow1 1 Department of Medicine and Division of Gastroenterology, and 2Department of Pathology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts
Question: A 67-year-old woman with a history of an upper gastrointestinal bleed 7 years prior that was localized to the ampullary region and resolved without endoscopic intervention presented to our medical center for a second opinion after a recent gastrointestinal bleed. She initially presented to an outside medical center after 3 days of feeling lightheaded and having a syncopal event after standing up in the morning as well as melanotic stool for the past day. On admission, she was noted to have an hemoglobin of 6 g/dL and was transfused 4 U of packed red cells. An esophagogastroduodenoscopy was performed and showed a duodenal mass with ulceration around the ampulla. Attempts were made to clip and cauterize the area, but were unsuccessful even with a side-viewing scope. She improved and was discharged with an hemoglobin of 7.9 g/dL. The patient and her family were advised that a pancreatic malignancy was the most likely diagnosis. She presented to our medical center for further evaluation. She denied any weight loss, jaundiced skin, or smoking history. She had no family history of pancreatic cancer. Her physical examination was unremarkable. She underwent an esophagogastroduodenoscopy and endoscopic ultrasonography that showed a semipedunculated, 2-cm mass of malignant appearance at the ampulla (Figure A, B). Endoscopic ultrasonography further characterized the mass as 2.4 2.2 cm that was hypoechoic and heterogenous in echotexture (Figure C–E). The borders were regular and well-defined. There was no vascular or biliary involvement. The head of the pancreas, pancreatic duct, and common bile ducts all seemed to be normal. Multiple cold forceps biopsies were obtained. Pathology is as shown in Figure F–H. What is the most likely diagnosis? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.
Conflicts of interest The authors disclose no conflicts. © 2014 by the AGA Institute 0016-5085/$36.00 http://dx.doi.org/10.1053/j.gastro.2014.06.038
Gastroenterology 2014;147:e3–e4
ELECTRONIC CLINICAL CHALLENGES AND IMAGES IN GI Answer to the Clinical Challenges and Images in GI Question: Image 2: Gangliocytic Paraganglioma The lesion was composed of 3 cellular constituents. Figure F shows spindle cells intermixed with epithelioid cells (original magnification, 400). Figure G shows areas where epithelioid cells seemed to be arranged in nests (original magnification, 400). Figure H shows focal extension of the lesion into the mucosa. Additionally, there are occasional ganglion cells within the lesion (left and right of image; original magnification, 400). Immunohistochemical studies revealed that the lesional cells were positive for cytokeratin cocktail (AE1/AE3, CAM 5.2), synaptophysin, and chromogranin; occasional cells within the lesion showed S100 immunoreactivity. These findings were most consistent with a gangliocytic paraganglioma (GP). Later, computed tomography of the abdomen with contrast was obtained, but did not identify the mass or any suspicious lymph nodes or metastatic disease. She eventually underwent a transduodenal resection of the ampulla of Vater neoplasm with reimplantation of the common bile duct and pancreatic duct with a biliopancreatic ductal septoplasty. Pathologic examination of the resected specimen confirmed the diagnosis. The resected margins were negative and she has remained well postoperatively. GPs were first reported by Dahl et al in 1957.1 The exact etiology of GP is unclear. GP are usually considered a type of neuroendocrine tumor, but others have postulated that it is a hamartoma from embryonic pancreatic tissue.2,3 Okubo et al2 identified 192 patients with GP reported in the literature. The mean age of diagnosis was 52.3 years (range, 15–84), with a male predominance. Over 90% of the tumors were located in the duodenum. Less frequently, they were in the jejunum, esophagus, pancreas, appendix, lower spinal cord, or respiratory system. Duodenal lesions most commonly present with gastrointestinal bleeding, followed by abdominal pain, anemia, and rarely with biliary obstruction.2 Diagnosis is confirmed on pathology. The cellular make up of GP includes epithelioid cells, spindle cells, and ganglion cells.2,3 Immunohistochemical stains are most frequently positive for the following markers in the pattern below: Epithelioid cells: Keratin, synaptophysin, chromogranin A, pancreatic polypeptide, and somatostatin. Spindle cells: S-100 protein and neuron specific enolase. Ganglion-like cells: synaptophysin, chromogranin A and neuron specific enolase. Although many of the tumors are benign, the tumors do possess malignant and metastatic potential. Unfortunately, there are no tumor markers or histologic findings to determine if the tumor has malignant potential.2,3 As a result, current standard of care is operative resection of these tumors, although there are case reports of successful endoscopic resection.
References 1. 2. 3.
e4
Dahl EV, Waugh JM, Dahlin DC. Gastrointestinal ganglioneuromas: brief review with report of a duodenal ganglioneuroma*. Am J Pathol 1957;33:953. Okubo Y, Wakayama M, Nemoto T, et al. Literature survey on epidemiology and pathology of gangliocytic paraganglioma. BMC Cancer 2011;11:187. Witkiewicz A, Galler A, Yeo CJ, et al. Gangliocytic paraganglioma: case report and review of the literature. J Gastrointest Surg 2007;11:1351–1354.
Question: A 67-year-old woman with a history of an upper gastrointestinal bleed 7 years prior that was localized to the ampullary region and resolved without endoscopic intervention presented to our medical center for a second opinion after a recent gastrointestinal bleed. She initially presented to an outside medical center after 3 days of feeling lightheaded and having a syncopal event after standing up in the morning as well as melanotic stool for the past day. On admission, she was noted to have an hemoglobin of 6 g/dL and was transfused 4 U of packed red cells. An esophagogastroduodenoscopy was performed and showed a duodenal mass with ulceration around the ampulla. Attempts were made to clip and cauterize the area, but were unsuccessful even with a side-viewing scope. She improved and was discharged with an hemoglobin of 7.9 g/dL. The patient and her family were advised that a pancreatic malignancy was the most likely diagnosis. She presented to our medical center for further evaluation. She denied any weight loss, jaundiced skin, or smoking history. She had no family history of pancreatic cancer. Her physical examination was unremarkable. She underwent an esophagogastroduodenoscopy and endoscopic ultrasonography that showed a semipedunculated, 2-cm mass of malignant appearance at the ampulla (Figure A, B). Endoscopic ultrasonography further characterized the mass as 2.4 2.2 cm that was hypoechoic and heterogenous in echotexture (Figure C–E). The borders were regular and well-defined. There was no vascular or biliary involvement. The head of the pancreas, pancreatic duct, and common bile ducts all seemed to be normal. Multiple cold forceps biopsies were obtained. Pathology is as shown in Figure F–H. What is the most likely diagnosis? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI.
Conflicts of interest The authors disclose no conflicts. © 2014 by the AGA Institute 0016-5085/$36.00 http://dx.doi.org/10.1053/j.gastro.2014.06.038
Gastroenterology 2014;147:e3–e4
ELECTRONIC CLINICAL CHALLENGES AND IMAGES IN GI Answer to the Clinical Challenges and Images in GI Question: Image 2: Gangliocytic Paraganglioma The lesion was composed of 3 cellular constituents. Figure F shows spindle cells intermixed with epithelioid cells (original magnification, 400). Figure G shows areas where epithelioid cells seemed to be arranged in nests (original magnification, 400). Figure H shows focal extension of the lesion into the mucosa. Additionally, there are occasional ganglion cells within the lesion (left and right of image; original magnification, 400). Immunohistochemical studies revealed that the lesional cells were positive for cytokeratin cocktail (AE1/AE3, CAM 5.2), synaptophysin, and chromogranin; occasional cells within the lesion showed S100 immunoreactivity. These findings were most consistent with a gangliocytic paraganglioma (GP). Later, computed tomography of the abdomen with contrast was obtained, but did not identify the mass or any suspicious lymph nodes or metastatic disease. She eventually underwent a transduodenal resection of the ampulla of Vater neoplasm with reimplantation of the common bile duct and pancreatic duct with a biliopancreatic ductal septoplasty. Pathologic examination of the resected specimen confirmed the diagnosis. The resected margins were negative and she has remained well postoperatively. GPs were first reported by Dahl et al in 1957.1 The exact etiology of GP is unclear. GP are usually considered a type of neuroendocrine tumor, but others have postulated that it is a hamartoma from embryonic pancreatic tissue.2,3 Okubo et al2 identified 192 patients with GP reported in the literature. The mean age of diagnosis was 52.3 years (range, 15–84), with a male predominance. Over 90% of the tumors were located in the duodenum. Less frequently, they were in the jejunum, esophagus, pancreas, appendix, lower spinal cord, or respiratory system. Duodenal lesions most commonly present with gastrointestinal bleeding, followed by abdominal pain, anemia, and rarely with biliary obstruction.2 Diagnosis is confirmed on pathology. The cellular make up of GP includes epithelioid cells, spindle cells, and ganglion cells.2,3 Immunohistochemical stains are most frequently positive for the following markers in the pattern below: Epithelioid cells: Keratin, synaptophysin, chromogranin A, pancreatic polypeptide, and somatostatin. Spindle cells: S-100 protein and neuron specific enolase. Ganglion-like cells: synaptophysin, chromogranin A and neuron specific enolase. Although many of the tumors are benign, the tumors do possess malignant and metastatic potential. Unfortunately, there are no tumor markers or histologic findings to determine if the tumor has malignant potential.2,3 As a result, current standard of care is operative resection of these tumors, although there are case reports of successful endoscopic resection.
References 1. 2. 3.
e4
Dahl EV, Waugh JM, Dahlin DC. Gastrointestinal ganglioneuromas: brief review with report of a duodenal ganglioneuroma*. Am J Pathol 1957;33:953. Okubo Y, Wakayama M, Nemoto T, et al. Literature survey on epidemiology and pathology of gangliocytic paraganglioma. BMC Cancer 2011;11:187. Witkiewicz A, Galler A, Yeo CJ, et al. Gangliocytic paraganglioma: case report and review of the literature. J Gastrointest Surg 2007;11:1351–1354.
