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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

Microscopic findings of the cystic tumor. The tumor is composed of atypical epithelial cells showing nuclear enlargement, clear nucleoli, and disordered polarity (hematoxylin and eosin, magnification 400×)
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Fig6: Microscopic findings of the cystic tumor. The tumor is composed of atypical epithelial cells showing nuclear enlargement, clear nucleoli, and disordered polarity (hematoxylin and eosin, magnification 400×)

Mentions: A pancreatography via the major and minor duodenal papillae on the surgical specimen revealed no connection between the ventral and dorsal pancreatic duct systems (Fig. 5). Macroscopically, a multilocular cystic tumor, 40 × 35 × 25 mm in size, with abundant accumulation of mucin was identified in the ventral pancreas. Microscopically, the tumor was composed of atypical epithelial cells showing nuclear enlargement, clear nucleoli, and disordered polarity (Fig. 6). They formed prominent papillary structures. The Mib-1 index was up to 80 %. The tumor cells slightly progressed into the main pancreatic duct. Finally, the tumor was determined to be a mixed type IPMC (well-differentiated adenocarcinoma) with partial microinvasion. The pancreas bearing the tumor was drained by the pancreatic duct, which opened into the major papilla, suggesting that it was the ventral pancreas. The region surrounding the IPMC was mainly composed of fibrous tissue, which was clearly distinguished from the normal pancreas (Fig. 7). The normal pancreas was relatively rich in adipose tissue and the islets of Langerhans were typically oval in shape, features consistent with the dorsal pancreas. Furthermore, the pancreatic duct in the region of the normal pancreas was linked to the minor papilla, also suggesting a dorsal pancreas origin.Fig. 5


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)

Microscopic findings of the cystic tumor. The tumor is composed of atypical epithelial cells showing nuclear enlargement, clear nucleoli, and disordered polarity (hematoxylin and eosin, magnification 400×)
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Fig6: Microscopic findings of the cystic tumor. The tumor is composed of atypical epithelial cells showing nuclear enlargement, clear nucleoli, and disordered polarity (hematoxylin and eosin, magnification 400×)
Mentions: A pancreatography via the major and minor duodenal papillae on the surgical specimen revealed no connection between the ventral and dorsal pancreatic duct systems (Fig. 5). Macroscopically, a multilocular cystic tumor, 40 × 35 × 25 mm in size, with abundant accumulation of mucin was identified in the ventral pancreas. Microscopically, the tumor was composed of atypical epithelial cells showing nuclear enlargement, clear nucleoli, and disordered polarity (Fig. 6). They formed prominent papillary structures. The Mib-1 index was up to 80 %. The tumor cells slightly progressed into the main pancreatic duct. Finally, the tumor was determined to be a mixed type IPMC (well-differentiated adenocarcinoma) with partial microinvasion. The pancreas bearing the tumor was drained by the pancreatic duct, which opened into the major papilla, suggesting that it was the ventral pancreas. The region surrounding the IPMC was mainly composed of fibrous tissue, which was clearly distinguished from the normal pancreas (Fig. 7). The normal pancreas was relatively rich in adipose tissue and the islets of Langerhans were typically oval in shape, features consistent with the dorsal pancreas. Furthermore, the pancreatic duct in the region of the normal pancreas was linked to the minor papilla, also suggesting a dorsal pancreas origin.Fig. 5

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