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Intraductal papillary mucinous carcinoma of the pancreas associated with pancreas divisum: a case report and review of the literature.

Nishi T, Kawabata Y, Ishikawa N, Araki A, Yano S, Maruyama R, Tajima Y - BMC Gastroenterol (2015)

Bottom Line: Endoscopic retrograde pancreatography via the major duodenal papilla revealed a cystic tumor and a slightly dilated main pancreatic duct with an abrupt interruption at the head of the pancreas.A pancreatography via the major and minor duodenal papillae on the surgical specimen revealed that the ventral and dorsal pancreatic ducts were not connected, and the tumor originated in the ventral duct, i.e., the Wirsung's duct.Microscopically, the tumor was diagnosed as intraductal papillary mucinous carcinoma with microinvasion.

View Article: PubMed Central - PubMed

Affiliation: Deparment of Surgery, Matsue Red Cross Hospital, 200 Horo-machi, Matsue, Shimane, 690-8506, Japan. nishiken1027@gmail.com.

ABSTRACT

Background: Pancreas divisum, the most common congenital anomaly of the pancreas, is caused by failure of the fusion of the ventral and dorsal pancreatic duct systems during embryological development. Although various pancreatic tumors can occur in patients with pancreas divisum, intraductal papillary mucinous neoplasm is rare.

Case presentation: A 77-year-old woman was referred to our hospital because she was incidentally found to have a cystic tumor in her pancreas at a regular health checkup. Contrast-enhanced abdominal computed tomography images demonstrated a cystic tumor in the head of the pancreas measuring 40 mm in diameter with slightly enhancing mural nodules within the cyst. Endoscopic retrograde pancreatography via the major duodenal papilla revealed a cystic tumor and a slightly dilated main pancreatic duct with an abrupt interruption at the head of the pancreas. The orifice of the major duodenal papilla was remarkably dilated and filled with an abundant extrusion of mucin, and the diagnosis based on pancreatic juice cytology was "highly suspicious for adenocarcinoma". Magnetic resonance cholangiopancreatography depicted a normal, non-dilated dorsal pancreatic duct throughout the pancreas. The patient underwent a pylorus-preserving pancreaticoduodenectomy under the diagnosis of intraductal papillary mucinous neoplasm with suspicion of malignancy arising in the ventral part of the pancreas divisum. A pancreatography via the major and minor duodenal papillae on the surgical specimen revealed that the ventral and dorsal pancreatic ducts were not connected, and the tumor originated in the ventral duct, i.e., the Wirsung's duct. Microscopically, the tumor was diagnosed as intraductal papillary mucinous carcinoma with microinvasion. In addition, marked fibrosis with acinar cell depletion was evident in the ventral pancreas, whereas no fibrotic change was noted in the dorsal pancreas.

Conclusion: Invasive ductal carcinomas of the pancreas associated with pancreas divisum usually arise from the dorsal pancreas, in which the occurrence of pancreatic cancer may link to underlying longstanding chronic pancreatitis in the dorsal pancreas; however, the histopathogenesis of intraductal papillary mucinous neoplasm in this anomaly is a critical issue that warrants further investigation in future.

No MeSH data available.


Related in: MedlinePlus

Endoscopic findings of the major duodenal papilla. The major duodenal papilla is enlarged and the orifice is filled with abundant mucin
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Fig4: Endoscopic findings of the major duodenal papilla. The major duodenal papilla is enlarged and the orifice is filled with abundant mucin

Mentions: A 77-year-old woman underwent an annual medical checkup and was diagnosed with a cystic tumor in the pancreas by computed tomography (CT) of the abdomen. The patient was referred to our hospital for further examination. Her medical history included a radical parotidectomy for a parotid gland tumor and a total knee replacement for the right leg. In addition, she was receiving treatment for hypertension and osteoporosis. On admission, she had no clinical symptoms. Her height was 154 cm and her body weight was 61 kg. There was no superficial lymphadenopathy or palpable mass in the abdomen. Her serum amylase level was 211 U/L (normal range; 30–120 U/L), and other biochemical data, including tumor marker levels, fasting plasma glucose, and hemoglobin A1c, were within normal ranges. An upper gastrointestinal endoscopy showed esophageal hiatal hernia and short-segment Barrett’s esophagus. Colonoscopy showed diverticula in the sigmoid colon. Contrast-enhanced abdominal CT scanning demonstrated a cystic tumor in the head of the pancreas measuring 40 mm in diameter with slightly enhancing mural nodules within the cyst (Fig. 1). Magnetic resonance cholangiopancreatography (MRCP) revealed a cystic tumor in the head of the pancreas along with a normal, non-dilated dorsal pancreatic duct throughout the pancreas (Fig. 2). The presence of a connection between the cystic lesion and the main pancreatic duct was unclear. Endoscopic retrograde pancreatography (ERP) via the major duodenal papilla showed a cystic tumor and a slightly dilated main pancreatic duct, but the main pancreatic duct was abruptly interrupted at the head of the pancreas (Fig. 3). The major duodenal papilla was enlarged and the orifice was filled with abundant mucin (Fig. 4). The minor duodenal papilla was normal in size and ERP via the minor papilla was not possible. The diagnosis based on pancreatic juice cytology was “highly suspicious for adenocarcinoma,” suggestive of an intraductal papillary mucinous carcinoma (IPMC) arising in the ventral pancreas of pancreas divisum. The patient underwent a pylorus-preserving pancreaticoduodenectomy (PPPD) with regional lymphadenectomy. The postoperative course was uneventful, except for a Grade A pancreatic fistula (staged according to the International Study Group on Pancreatic Fistula clinical criteria [10]), and the patient was discharged on postoperative day 29.Fig. 1


Intraductal papillary mucinous carcinoma of the pancreas associated with pancreas divisum: a case report and review of the literature.

Nishi T, Kawabata Y, Ishikawa N, Araki A, Yano S, Maruyama R, Tajima Y - BMC Gastroenterol (2015)

Endoscopic findings of the major duodenal papilla. The major duodenal papilla is enlarged and the orifice is filled with abundant mucin
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4495851&req=5

Fig4: Endoscopic findings of the major duodenal papilla. The major duodenal papilla is enlarged and the orifice is filled with abundant mucin
Mentions: A 77-year-old woman underwent an annual medical checkup and was diagnosed with a cystic tumor in the pancreas by computed tomography (CT) of the abdomen. The patient was referred to our hospital for further examination. Her medical history included a radical parotidectomy for a parotid gland tumor and a total knee replacement for the right leg. In addition, she was receiving treatment for hypertension and osteoporosis. On admission, she had no clinical symptoms. Her height was 154 cm and her body weight was 61 kg. There was no superficial lymphadenopathy or palpable mass in the abdomen. Her serum amylase level was 211 U/L (normal range; 30–120 U/L), and other biochemical data, including tumor marker levels, fasting plasma glucose, and hemoglobin A1c, were within normal ranges. An upper gastrointestinal endoscopy showed esophageal hiatal hernia and short-segment Barrett’s esophagus. Colonoscopy showed diverticula in the sigmoid colon. Contrast-enhanced abdominal CT scanning demonstrated a cystic tumor in the head of the pancreas measuring 40 mm in diameter with slightly enhancing mural nodules within the cyst (Fig. 1). Magnetic resonance cholangiopancreatography (MRCP) revealed a cystic tumor in the head of the pancreas along with a normal, non-dilated dorsal pancreatic duct throughout the pancreas (Fig. 2). The presence of a connection between the cystic lesion and the main pancreatic duct was unclear. Endoscopic retrograde pancreatography (ERP) via the major duodenal papilla showed a cystic tumor and a slightly dilated main pancreatic duct, but the main pancreatic duct was abruptly interrupted at the head of the pancreas (Fig. 3). The major duodenal papilla was enlarged and the orifice was filled with abundant mucin (Fig. 4). The minor duodenal papilla was normal in size and ERP via the minor papilla was not possible. The diagnosis based on pancreatic juice cytology was “highly suspicious for adenocarcinoma,” suggestive of an intraductal papillary mucinous carcinoma (IPMC) arising in the ventral pancreas of pancreas divisum. The patient underwent a pylorus-preserving pancreaticoduodenectomy (PPPD) with regional lymphadenectomy. The postoperative course was uneventful, except for a Grade A pancreatic fistula (staged according to the International Study Group on Pancreatic Fistula clinical criteria [10]), and the patient was discharged on postoperative day 29.Fig. 1

Bottom Line: Endoscopic retrograde pancreatography via the major duodenal papilla revealed a cystic tumor and a slightly dilated main pancreatic duct with an abrupt interruption at the head of the pancreas.A pancreatography via the major and minor duodenal papillae on the surgical specimen revealed that the ventral and dorsal pancreatic ducts were not connected, and the tumor originated in the ventral duct, i.e., the Wirsung's duct.Microscopically, the tumor was diagnosed as intraductal papillary mucinous carcinoma with microinvasion.

View Article: PubMed Central - PubMed

Affiliation: Deparment of Surgery, Matsue Red Cross Hospital, 200 Horo-machi, Matsue, Shimane, 690-8506, Japan. nishiken1027@gmail.com.

ABSTRACT

Background: Pancreas divisum, the most common congenital anomaly of the pancreas, is caused by failure of the fusion of the ventral and dorsal pancreatic duct systems during embryological development. Although various pancreatic tumors can occur in patients with pancreas divisum, intraductal papillary mucinous neoplasm is rare.

Case presentation: A 77-year-old woman was referred to our hospital because she was incidentally found to have a cystic tumor in her pancreas at a regular health checkup. Contrast-enhanced abdominal computed tomography images demonstrated a cystic tumor in the head of the pancreas measuring 40 mm in diameter with slightly enhancing mural nodules within the cyst. Endoscopic retrograde pancreatography via the major duodenal papilla revealed a cystic tumor and a slightly dilated main pancreatic duct with an abrupt interruption at the head of the pancreas. The orifice of the major duodenal papilla was remarkably dilated and filled with an abundant extrusion of mucin, and the diagnosis based on pancreatic juice cytology was "highly suspicious for adenocarcinoma". Magnetic resonance cholangiopancreatography depicted a normal, non-dilated dorsal pancreatic duct throughout the pancreas. The patient underwent a pylorus-preserving pancreaticoduodenectomy under the diagnosis of intraductal papillary mucinous neoplasm with suspicion of malignancy arising in the ventral part of the pancreas divisum. A pancreatography via the major and minor duodenal papillae on the surgical specimen revealed that the ventral and dorsal pancreatic ducts were not connected, and the tumor originated in the ventral duct, i.e., the Wirsung's duct. Microscopically, the tumor was diagnosed as intraductal papillary mucinous carcinoma with microinvasion. In addition, marked fibrosis with acinar cell depletion was evident in the ventral pancreas, whereas no fibrotic change was noted in the dorsal pancreas.

Conclusion: Invasive ductal carcinomas of the pancreas associated with pancreas divisum usually arise from the dorsal pancreas, in which the occurrence of pancreatic cancer may link to underlying longstanding chronic pancreatitis in the dorsal pancreas; however, the histopathogenesis of intraductal papillary mucinous neoplasm in this anomaly is a critical issue that warrants further investigation in future.

No MeSH data available.


Related in: MedlinePlus