Calcification in mucinous cholangiocellular carcinoma

Calcification is rarely seen in cholangiocellular carcinoma. We herein report the case of a 53 year-old man with calcification in a cholangiocellular carcinoma. Because imaging studies had revealed coarse calcified foci, hepatolithiasis was suspected pre-operatively. The patient underwent a laparotomy in which intra-operative cholangioscopy revealed no gallstones but did reveal an unsuspected tumor with abundant mucin. A left hepatic lobectomy with resection of the extrahepatic bile duct was performed. The tumor histology was mucinous adenocarcinoma with calcification. In the English language literature, we found 9 cases of cholangiocellular carcinoma with macroscopic calcification. Six of these cases were mucinous adenocarcinomas. Roentgenologic examination revealed coarse calcification in 7 cases and fine calcification in 2 cases. Clinicians should note that cholangiocellular carcinoma, especially the mucinous variant, may be accompanied by coarse calcification.     

“An imprisoned heart” arrested by pillory of calcified ring surrounding both ventricles: An extremely rare case of constrictive pericarditis

We report the case of a 63‐year‐old man who was admitted to our hospital due to progressive dyspnea and leg edema. Echocardiography and cardiac computed tomography revealed localized severe pericardial calcification surrounding the basal segments of both ventricles, which caused pericardial constriction. Right heart catheter examination confirmed typical hemodynamic findings of constrictive pericarditis and subsequently led to the definite diagnosis of constrictive pericarditis. We experienced a rare case of localized constrictive pericarditis caused by the thickened and calcified pericardial ring. Non‐invasive and invasive multimodality evaluations should be encouraged for accurate diagnosis and better management of these cases.     

Gastrointestinal stromal tumor presenting with prominent calcification

We present a rare case of a gastrointestinal stromal tumor (GIST) in the stomach with prominent calcification at presentation. A 61-year-old woman visited our hospital because of epigastric discomfort. A spherical calcified lesion with a diameter of about 30 mm was incidentally shown in the left upper quadrant on an abdominal X-ray. Computed tomography demonstrated that the tumor was growing from the upper gastric body, with calcification in the peripheral ring area. A laparoscopic partial gastrectomy was performed, and the resected specimen revealed a well-circumscribed tumor with exophytic growth from the gastric muscularis propria. Microscopic examination revealed spindle-shaped tumor cells with calcification and hemorrhage. Additionally, positive immunoreactivity of the tumor to KIT and CD34 and a low mitotic index resulted in the diagnosis of very low risk GIST. There are a few case reports of heavily calcified GIST, although solitary or punctate calcification of primary GIST has been reported in several case series. Dystrophic calcification of necrotic or degenerative tissue is the supposed cause of primary calcified GISTs. In contrast, appearance of calcification after administration of imatinib mesylate, which may be one indicator of disease response, is possibly caused by a different mechanism.     

Unusual appearance of mitral annular calcification mimicking intracardiac tumor prompting early surgery

Although mitral annular calcification (MAC) is usually easy to diagnose by transthoracic echocardiography, we experienced a rare case with MAC which looked like an intracardiac tumor. The patient who had been on chronic hemodialysis for 20 years was admitted to our hospital because of dyspnea. Transthoracic echocardiography showed a mass with severe calcification on the anterior mitral annulus and mean mitral gradient of 20 mm Hg. Because of the suspicion of the intracardiac calcified tumor that restricted mitral valve motion causing mitral obstruction, she underwent resection of the mass and mitral valve replacement. Pathological findings showed that the mass had a calcified envelope containing liquefied necrotic eosinophilic material with lympocytic infiltrate inside consistent with MAC. We should consider a possibility of MAC when we see a severe calcified mass attached to the mitral annulus in a patient on long-term hemodialysis.     

A case of follicular cholangitis mimicking hilar cholangiocarcinoma

Follicular cholangitis is a sclerosing cholangitis with hilar biliary stricture that must be differentiated from both immunoglobulin G4-related sclerosing cholangitis and primary sclerosing cholangitis. This disorder is extremely rare and difficult to distinguish from hilar biliary cholangiocarcinoma. We report here a case of a Japanese female patient in her 60s with this disease. The patient visited a family doctor for itching and general fatigue. Blood examination showed elevated hepatobiliary enzyme levels. Various imaging studies showed dilation of the bilateral intrahepatic bile duct and wide stenosis from the proximal bile duct to the right and left hepatic duct. They also showed the enlargement of multiple lymph nodes in the hepatoduodenal ligament, periaorta, and mesocolon. Based on endoscopic retrograde cholangiopancreatography-directed brush cytology, we diagnosed this patient with hilar cholangiocarcinoma and performed left trisegmentectomy of the liver. The pathology results showed that the wall from the bilateral hepatic duct to the proximal bile duct had thickened irregularly with dense fibrosis and a marked formation of lymph follicles. The mucosal epithelia did not have malignant findings. The diagnosis was follicular cholangitis. This case indicates that follicular cholangitis should be considered as a differential diagnosis of hilar biliary stricture.     

132例胆管癌的临床诊治比较分析

目的比较肝门胆管癌和肝内胆管癌的临床特点及手术疗效。方法回顾性分析2001年1月—2008年12月我院132例胆管细胞癌患者（肝门胆管癌47例,肝内胆管癌85例）的临床资料。结果两组患者在年龄、性别以及ALT、AST、AFP等指标上均无明显差异,但肝门胆管癌患者具有较高的胆红素水平[（115±38）对（55±31）μmol/L,（P〈0.01）]。肝门胆管癌组中HBV携带者占42.35%（36/85）,明显高于肝内胆管癌组29.79%（14/47）,差异具有统计学意义（P〈0.01）。临床表现方面,肝门胆管癌的首发症状多为黄疸者占72.34%（34/47）,而肝内胆管癌组首发症状为腹痛的占57.65%（49/85）;CA19-9在肝门胆管癌高于肝内胆管癌组（P=0.02）。132患者中106例行根治性手术切除,肝门胆管癌和肝内胆管癌的手术切除率分别为57.4%（27/47）和92.9%（79/85）。肝门胆管癌行根治性手术的1、3、5年生存率分别为77.78%、44.44%、25.93%,明显低于肝内胆管癌行根治性手术的89.87%、59.49%、36.71%。结论 HBV感染为胆管癌的主要危险因素,肝门胆管癌组中HBV携带者明显高于肝内胆管癌组。CA19-9对肝门胆管癌的诊断更具参考价值。积极的手术治疗是提高肝门胆管癌和肝内胆管癌生存率的主要手段。     

Long-term survival of intrahepatic cholangiocarcinoma with hilar lymph node metastasis and portal vein involvement

We report the case of a very rare 6-year disease-free survivor of intrahepatic cholangiocarcinoma with hilar lymph node metastasis and portal vein involvement. A 76-year-old female with liver dysfunction was referred to our institution. Contrast-enhanced computed tomography showed a 5-cm low-density tumor with irregular marginal enhancement in the left and caudate lobes of the liver. Cholangiography revealed complete obstruction of the left hepatic bile duct. Angiography showed obstruction of the left branch of the portal vein. Metastasis to the hilar lymph nodes was disclosed at surgery. The patient underwent left hepatectomy with caudate lobectomy, resection of the extrahepatic bile duct, and lymphadenectomy. The total vascular exclusion of the liver was used for hepatectomy and reconstruction of the portal vein. Microscopically, the tumor was a poorly differentiated adenocarcinoma with many infiltrating lymphocytes, and extensive necrosis was present within the tumor. The experience gained in the present case suggests that aggressive surgery may be a potential approach to provide a hope of long-term survival for patients with intrahepatic cholangiocarcinoma despite the presence of regional lymph node metastasis and vascular invasion.     

Solitary recurrence of hilar cholangiocarcinoma in a mediastinal lymph node two years after curative resection

We recently encountered an unusual case of hilar cholangiocarcinoma in which a solitary recurrence in a mediastinal lymph node occurred two years after curative resection of the primary tumor. A 64-year old woman was admitted to our hospital with a complaint of right hypochondrial discomfort. After imaging studies demonstrated a hilar cholangiocarcinoma in the left hepatic duct, a curative resection of the tumor was performed, consisting of a left hepatic lobectomy along with caudate lobectomy, regional lymph node dissection, and resection of the extrahepatic bile duct. No nodal metastasis was observed histologically. Two years after surgery, the patient was found to have a nodule in the posterior mediastinum, which was thoracoscopically resected. No other swollen lymph nodes, local recurrence, or distant metastasis were noted. Histologically, the nodule proved to be a metastatic lymph node, and adjuvant chemoradiation therapy was initiated. The patient remained well for the four years following her first operation and had no evidence of disease recurrence 28 mo after her second operation. To our knowledge, this case is the first report of solitary recurrence in a mediastinal lymph node after curative resection of hilar cholangiocarcinoma.     

Solitary recurrence of hilar cholangiocarcinoma in a mediastinai lymph node two years after curative resection

We recently encountered an unusual case of hilar cholangiocarcinoma in which a solitary recurrence in a mediastinal lymph node occurred two years after curative resection of the primary tumor. A 64-year old woman was admitted to our hospital with a complaint of right hypochondrial discomfort. After imaging studies demonstrated a hilar cholangiocarcinoma in the left hepatic duct, a curative resection of the tumor was performed, consisting of a left hepatic lobectomy along with caudate lobectomy, regional lymph node dissection, and resection of the extrahepatic bile duct. No nodal metastasis was observed histologically. Two years after surgery, the patient was found to have a nodule in the posterior mediastinum, which was thoracoscopically resected. No other swollen lymph nodes, local recurrence, or distant metastasis were noted. Histologically, the nodule proved to be a metastatic lymph node, and adjuvant chemoradiation therapy was initiated. The patient remained well for the four years following her first operation and had no evidence of disease recurrence 28 mo after her second operation. To our knowledge, this case is the first report of solitary recurrence in a mediastinal lymph node after curative resection of hilar cholangiocarcinoma.     

Colonic metastasis from primary carcinoma of the lung: report of a case and review of Japanese literature

We report a rare case of colonic metastasis from primary carcinoma of the lung. A 59-year-old man who underwent pulmonary surgery for lung cancer was referred to our hospital in June 2007. The patient complained of abdominal pain, and barium enema examination at another hospital had demonstrated a descending colon tumor. Postoperative histopathological and immunohistochemical findings indicated that the tumor was a colonic metastasis of lung cancer. Three months postoperatively, the cancer had metastasized to the brain, and the patient underwent radiotherapy. He survived for more than 1 year after colonic surgery. Clinically apparent metastases from lung cancer to the colon are rare, and in the 50 Japanese cases retrospectively investigated here, the prognosis was poor.     

Endoscopic ultrasound-guided biliary drainage:Bilateral systems drainage via left duct approach

Endoscopic ultrasound(EUS)-guided biliary drainage is accepted as a less invasive,alternative treatment for patients in whom endoscopic retrograde cholangiopancreatography has failed. Most patients with malignant hilar obstruction undergo EUSguided hepaticogastrostomy. The authors present the case of a 77-year-old man with advanced hilar cholangiocarcinoma who had undergone a rouxen-Y hepaticojejunostomy several months prior. He developed progressive jaundice and a low-grade fever that persisted for one week. The enteroscopic-assisted endoscopic retrograde cholangiopancreatography failed,thus the patient was scheduled for EUS-guided biliary drainage. In order to obtain adequate drainage,both intrahepatic systems were drained. This report describes the technique used for bilateral drainage via a transgastric approach. Currently,only a few different techniques for EUS-guided right system drainage have been reported in the literature. This case demonstrates that bilateral EUS-guided biliary drainage is feasible and effective in patients with hilar cholangiocarcinoma,and thus can be used as an alternative to percutaneous biliary drainage.     

Perihilar cholangiocarcinoma arising in hepatitis C virus-related liver cirrhosis with hepatocellular carcinoma

Liver cirrhosis is reportedly one of the conditions preceding peripheral-type intrahepatic cholangiocarcinoma but not hilar/perihilar cholangiocarcinoma. Herein, we report a case of perihilar cholangiocarcinoma arising in a hepatitis C virus-related cirrhotic liver. The patient was a 69-year-old man. He was diagnosed with hepatitis C virus-related chronic hepatitis at the age of 56 years, and 9 years later, multiple hepatocellular carcinomas were detected by imaging modalities. Despite treatments, including chemotherapy, he died of hepatic failure at the age of 69 years. At autopsy, in addition to multiple nodules of hepatocellular carcinoma, we found a white mucinous and fibrous tumor spreading from the hepatic hilum to the periphery along the left lateral segmental bile ducts in the advanced cirrhotic liver. This tumor was histologically a cholangiocarcinoma that involved mainly the peribiliary glands and showed variable cystic dilation, suggesting that it might have been derived from these peribiliary glands. Immunohistochemically, the cholangiocarcinoma cells were positive for cytokeratin 7 and mucin core protein 1, and negative for cytokeratin 20 and mucin core protein 2. Hilar/perihilar cholangiocarcinoma arising in hepatitis C virus-related liver cirrhosis has rarely been reported. This case warrants further studies to clarify the possible involvement of hepatitis C virus in tumorigenesis of hilar/perihilar cholangiocarcinoma.     

Cholangiocarcinoma in hepatolithiasis

During the period of 1979-1982, 10 cases of cholangiocarcinoma associated with hepatolithiasis were seen. We report the clinical features of 10 cases of cholangiocarcinoma in association with hepatolithiasis, along with detailed histopathology from the four resected specimens. Our observations suggest that whenever intrahepatic stones are encountered in patients over 50 years of age with a long history of recurrent cholangitis and intractable pain, further examination including echo-guided aspiration cytology, liver scanning, and peritoneoscopy should be performed to rule out a coexisting cholangiocarcinoma.     

A case of groove pancreatitis observed from initial stage

We report a case of groove pancreatitis. A 60-year-old man was admitted to our hospital because of nausea and vomitting. CT showed a mass lesion in the groove between the pancreas head, duodenum and common bile duct. He was given a diagnosis of the groove pancreatitis, and underwent endoscopic balloon dilation. Groove pancreatitis is rare, and we discuss this case with references.     

Surgical Treatment and Prognostic Analysis of 93 Cases of Hilar Cholangiocarcinoma

ObjectiveTo explore the surgical treatment and prognosis of hilar cholangiocarcinoma.MethodsThis was a retrospective study of 93 cases of hilar cholangiocarcinoma that were treated surgically at our hospital from June 1999 to June 2005. Prognostic factors were also analyzed.ResultsFifty-two cases were treated with curative resection, 21 with palliative resection, and 9 with nonoperative drainage. Eleven cases underwent palliative drainage operations. The median survive time was 31 months in the curative resection group, 13.7 months in the palliative resection group, and 11 months in the nonoperative drainage group. Patient age, serum total bilirubin, clinical type of Bismuth-Corlette, tumor differentiation, and lymph node metastases were important factors for predicting the prognosis of hilar cholangiocarcinoma.ConclusionsResection was the main treatment for hilar cholangiocarcinoma, and curative resection was the best way to obtain better prognosis. Age, preoperative serum total bilirubin, bismuth clinical type, tumor histopathological grading, and lymph node metastases were considered to have a significant effect on prognosis.     

Lymphoepithelioma-like cholangiocarcinoma:A mimic of hepatocellular carcinoma on imaging features

Primary lymphoepithelioma-like carcinoma in the liver is extremely rare. A few cases of lymphoepithelioma-like cholangiocarcinoma have been reported, but few radiologic features were described. We reviewed 23 cases of lymphoepithelioma-like cholangiocarcinoma reported between 1996 and 2014 and describe a rare case of a 35-year-old woman in our hospital who was diagnosed with lymphoepithelioma-like cholangiocarcinoma of the liver and was a hepatitis B carrier. The tumor(1.6 cm) in our patient appeared to be hypoechoic in sonographic images and hypodense in computed tomography(CT) images. In addition, it was homogeneous hypointense in T1-weighted magnetic resonance(MR) images(MRI) and hyperintense in T2-weighted MRI. Dynamic gadolinium-enhanced MRI showed typical image pattern of hepatocellular carcinoma(HCC). The patient underwent a laparoscopic left hepatic lobectomy, and the resected tumor consisted of well-differentiated glandular cells with extensive lymphocytic infiltration that were immunoreactive to CK(AE1/AE3), CD3, and CD20. In addition, the tumor was positive for Epstein-Barr virus-encoded RNA in situ hybridization. Finally, lymphoepithelioma-like cholangiocarcinoma was diagnosed. In previous studies, the incidence is highest among middle-aged people. Most tumors appeared to be hypodense with either hypovascular or hypervascular patterns in CT images. This case report is the first study to address sonography, CT, and MRI observations and delineate pathologic correlations. We suggest that the imaging pattern of lymphoepithelioma-like cholangiocarcinoma, either the typical cholangiocarcinoma pattern or a mimic of HCC, should be considered in the differential lists for HCC.     

Use of profound hypothermia and circulatory arrest for the calcified aorta

This case report discusses one method of dealing with the extensively calcified aorta in patients undergoing open heart surgery. Profound hypothermia and circulatory arrest was used in a patient undergoing aortic valve replacement with severe calcification of the ascending aorta and transverse arch. This patient recovered from surgery and was discharged from the hospital with no neurologic deficits.     

Successful diagnosis and resection of icteric type hepatocellular carcinoma

We report a rare case of icteric type hepatocellular carcinoma with successful preoperative diagnosis and curative resection. A 50-year-old man was admitted to our hospital with fever elevation and jaundice. Endoscopic retrograde cholangiography revealed a tumor at the hepatic hilum, which caused obstruction of the right and left hepatic duct. The tumor was suspected of being a hilar cholangiocarcinoma, but it was diagnosed as being a hepatocellular carcinoma by endoscopic retrograde bile duct biopsy. After percutaneous transhepatic biliary drainage a right hemihepatectomy was performed. Subsequent pathological finding showed the tumor to be moderately-differentiated hepatocellular carcinoma (Edmondson III) which invaded into the right hepatic duct. The patient survived for three years without recurrence. Although patients with icteric type hepatocellular carcinoma have been reported to have a poor prognosis, earlier diagnosis and curative resection may occasionally bring them an improved prognosis.     

Intrahepatic cholangiocarcinoma and hepatolithiasis: an unusual association in Western countries

Hepatolithiasis is uncommon in Western countries and the relationship with cholangiocarcinoma is unusual. We report the association of hepatolithiasis and a cholangiocarcinoma in a Caucasian patient with a 17-year history of recurrent pancreatitis associated with hepatolithiasis. We discuss work-up and surgical treatment, and stress the need to keep in mind the possible association between hepatolithiasis and cholangiocarcinoma even in Western countries.     

Spontaneous cutaneous biliary fistula: a rare complication of cholangiocarcinoma

A rare case of intrahepatic-cutaneous biliary fistula resulted from obstruction of the biliary tree by cholangiocarcinoma in the hilar area. The diagnosis was made clinically by the presence of a constant pus discharge through the fistula opening and confirmed by sonogram, computed tomogram (CT), and surgery. To our knowledge, there have been no previous reports of such a fistula as the presenting symptom of cholangiocarcinoma.