Intraductal Papillary Mucinous Neoplasm of the Bile Ducts

Intraductal papillary mucinous neoplasms of the bile ducts (IPMNs-B) are uncommon lesions that are characterized by innumerable papillary fronds that contain fine vascular cores, enriched mucin production and bile duct dilatation. IPMNs-B are histologically similar to intraductal papillary mucinous neoplasms of the pancreas and they are occasionally associated with hepatolithiasis. IPMNs-B are considered to be relatively low-grade malignancy and they merit consideration for aggressive surgery. Thus, early and precise diagnosis is important to maximize patient survival. From July 2002 to March 2006, we identified four patients with IPMNs-B at our hospital. In three patients, intrahepatic or extrahepatic bile duct stones were associated with their condition. Computed tomography and magnetic resonance cholangiography were done in all four cases and this demonstrated marked dilatation of the biliary tree. Endoscopic retrograde cholangiography was done in two cases and a large amount of mucin that was draining from the patulous orifice of the duodenal papilla was seen on endoscopy. However, two cases were initially misdiagnosed as intrahepatic cholangiocarcinoma or choledochal cyst with intrahepatic bile duct stones. All the cases underwent ipsilateral hemihepatectomy with caudate lobectomy. Histologically, one case showed to be adenoma, one case was borderline and two cases were invasive adenocarcinoma.

Papillary Neoplasm of the Endolymphatic Sac: A report of two cases

Papillary tumor of the temporal bone or middle ear has been recognized as an aggressive neoplasm because of its invasive growth pattern. The site of origin is controversial so that most cases have been reported under various diagnostic terms. Recently, Heffner(1989) suggested that the endolymphatic sac is a possible site of origin, because the tumor resembles the endolymphatic sac in several aspects. We report two such cases. One patient was a 34-year-old female presenting with tinnitus and hearing difficulty for 1 year. Temporal bone CT revealed extensive bone destruction by the tumor which was located in the posterolateral aspect of temporal bone. The other patient was a 56-year-old female who complained of tinnitus, dizziness and otalgia for 2 years. Cranial MR imaging showed an irregularly marginated mass in the left jugular fossa with extension to the petrous bone. Histologically, both cases showed a papillary pattern and locally destructive growth that are typical of papillary tumor of the endolymphatic sac. The papillae were lined by a single layer of bland-looking cuboidal to low columnar cells. Immunohistochemically the lining cells expressed cytokeratin, epithelial membrane antigen, neuron specific antigen and in one case, S-100 protein, supporting the thesis that these neoplasms might be of endolymphatic sac origin.