[The rational diagnostic of cholangiocarcinoma]. / Racionální diagnostika cholangiokarcinomu.

Cholangiocarcinoma (CC) is a rare malignant tumour arising from cholangiocytes, and its prognosis is usually unfavourable, mostly as a result of late diagnosis of the tumour. The current incidence of cholangiocarcinoma in the Czech Republic is 1.4/100,000 inhabitants per year; in less than 30 % of patients with CC, one of the known risk factors can be identified, most frequently, primary sclerosing cholangitis. Only patients with early diagnosed and surgically amenable cholangiocarcinoma are likely to have a longer survival time; in their case, survival for more than five years has been achieved in 20 % to 40 %. From the perspective of the need for early diagnosis of CC, a significant part is played by imaging and histopathologic evaluation; the early diagnostic significance of oncomarkers is limited. The rational early diagnosis of CC consists in effective use of differentiated advantages of different imaging modalities - MRI with DSA appears to be the optimal method, endosonography is a sensitive method for the identification of malignancy in the hepatic hilum or distal common bile duct, MRCP (magnetic resonance cholangiopancreatography) is used to display pathological changes in the biliary tree, ERCP (endoscopic retrograde cholangiopancreatography) allows material removal for histopathological examination. Other new approaches are also beneficial, such as IDUS - intraductal ultrasonography of biliary tract or SPY-GLASS, enabling examination of the bile ducts by direct view with the possibility of taking targeted biopsies. Sensitivity and specificity of histology and cytology can be increased by using the molecular cytogenetic FISH method, i.e. fluorescence in situ by hybridization, with a specificity of 97 %.

Clinical stages in patients with primary and subsequent cancers based on the czech cancer registry 1976-2005.

Of 1,486,984 new cancers registered in the Czech Cancer Registry in 1976-2005, 290,312 (19.5%) were multiple malignant neoplasms (MMNs), of which there were 65,292 primary and 89,796 subsequent cases in men and 59,970 primary and 75,254 subsequent cases in women. The duplicities were higher in women, and the triplicities and others (3-6 MMNs) were higher in men. The most frequent diagnoses were the primary cancers of skin, gastrointestinal and urinary tract, male genital organs, respiratory tract in men, and cancers of skin, breast, female genital organs, and gastrointestinal tract in women. The analysis of the early and advanced clinical stages shows that the number of subsequent advanced stages increased after primary advanced stages. Their time-age-space distributions visualized maps of MMNs in 14 Czech regions. These results support the improvement of algorithms of dispensary care for the early detection of the subsequent neoplasms.

Immunoglobulin G4-related cholangitis: a variant of IgG4-related systemic disease.

IgG4-related sclerosing cholangitis as part of IgG4 systemic-related diseases is commonly associated with autoimmune pancreatitis. Major clinical manifestations of IgG4-related sclerosing diseases are apparent in the organs in which tissue fibrosis with obstructive phlebitis is pathologically induced. IgG4-related sclerosing cholangitis is included within the heterogeneous group of 'sclerosing cholangitis'. Sclerosing cholangitis may be associated with choledocholithiasis, infection or biliary malignancies. Sclerosing cholangitis of unknown etiology is called primary sclerosing cholangitis (PSC). Conservative therapy of PSC is usually unsuccessful, the disease involves extra- and/or intrahepatic biliary tree, and the end point of this disease is liver cirrhosis. Typically, PSC is identified at the age of 30 to 40 years, and the disease is frequently associated with inflammatory bowel diseases. On the other hand, IgG4-related sclerosing cholangitis is not associated with inflammatory bowel diseases. In patients with IgG4-related sclerosing cholangitis, a first symptom can be obstructive jaundice, whereas obstructive jaundice is rarely present in PSC. Clinically, patients with IgG4-related sclerosing cholangitis are older at diagnosis compared to patients with PSC. A typical diagnostic feature of IgG4-related sclerosing cholangitis is elevation of serum immunoglobulin G4. In patients with IgG4-related sclerosing cholangitis, response to steroid therapy is high; in patients with PSC corticosteroid therapy is unsuccessful. Histochemically abundant infiltration of IgG4-positive plasma cells is detected in the biliary duct wall. Histologically, we can identify dense lymphoplasmacytic infiltration of the bile duct wall, transmural fibrosis, lymphoplasmacytic infiltration and fibrosis in the periportal area of the liver - a typically obliterative phlebitis. The biliary epithelium is usually intact in contrast to PSC, where mucosal erosion is often present. Steroids are the first-choice therapy of IgG4-related sclerosing cholangitis. In the literature, cholangiocarcinoma in patients with IgG4- related sclerosing cholangitis was not described, whereas cholangiocarcinoma develops in up to 10-30% of patients with PSC.

[International health statistics databases and available data]. / Mezinárodní databáze zdravotních statistik a jejich dostupné údaje.

The relevant data and information is necessary for the correct decision making. It applies also in case of health system management. Nowadays, many international databases of health data are available. The first part of this paper describes databases of the most important health data providers. Databases of WHO, WHO/Europe, OECD, IARC and Eurostat are searched and compared. However, combining statistics from various databases can be dangerous because the data are mutually incomparable. This is caused by different standard population, varying definitions of similar attributes, etc. used for comparison, which complicate international data comparison. Available indicators also do not allow adequate assessment of the quality of national health systems. The last part is dedicated to projects that compare international health statistics, the quality indicators and health care expenditures in order to achieve sufficient capacity and productivity of national health systems.

Trends of primary and subsequent cancers of the gastrointestinal tract in the Czech population, 1976-2005.

A total of 355,624 new gastrointestinal (GI) cancers were registered in 1976-2005 in the Czech Cancer Registry. Of these, there were 14,744 (4.1%) primary and 26,790 (7.5%) subsequent cases, of which all were GI multiple cancers (12.1% males and 11.1% females). The primary GI cancers were followed by 16,362 other neoplasms (60.7% males, 39.3% females); the subsequent GI cancers were preceded by 31,519 other neoplasms (55.8% males, 44.2% females). Double neoplasms were higher in females, and multiple cases were higher in males. The number of primary cases peaked in 1997, the number of subsequent cases increased until 2005. Almost half of the cases were registered in the age group of 50-69 years. The average interval between primary GI cancers and subsequent neoplasms was 6.1 years; the ratio of synchronous to metachronous cases was 1:3.6 in males and 1:5 in females. The most frequent synchronous cases in males were cancers of other GI, urinary, genital and respiratory tract; in females these were cancers of other GI, genital and urinary tract and the breast. The most frequent cancers preceding the next subsequent GI cancer included primary cancers of the skin, other GI, genital, urinary and respiratory tract for males, and those of the skin, genital and other GI tract and breast cancer for females. The 23,462 subsequent GI cancers reported as a 2nd cancer included early stages in 29.6% of males and 27.9% of females, advanced stages in 31.2% males and 31.3% females, and unknown stages in 39.3% males and 40.8% females. Of 3,562 primary neoplasms of advanced stages before subsequent GI cancers, 2,093 were cases at stage III (51.4% males, 48.6% females) and 1,469 cases were at stage IV (60.2% males, 39.8% females); the most frequent in males were primary cancers of other GI, respiratory and genital tract, and cancers of other GI, breast and genital cancers in females. Of 9,568 primary neoplasms before subsequent GI cancers at advantage stages, 3,325 cases were registered before stage III (53.9% males, 46.1% females) and 6,243 cases before stage IV (57.7% males, 42.3% females). The most frequent in males were primary cancers of skin, other GI, genital and urinary tract; for women, the most frequent were those of the skin, genital cancers, breast and other GI tract. Up to October 2007, 78.2% males died and 21.8% survived primary GI cancers; for women, 76.6% of died and 23.4% survived. Of the subsequent GI cancers, 86.7% males died and 13.3% survived; for women, 85.7% died and 14.3% survived.

[Cancer multiplicities in the Czech population]. / Nádorové multiplicity u ceské populace.

During 1976-2005 the Czech Cancer Registry registered 1.486,984 neoplasms of categories ICD-10: C00-D48, of which were notified 290,312 (19.5%) multiple cases. There were 65,292 primary diseases in men and 59,970 in women, 89,796 subsequent neoplasms in men and 75,254 in women. The duplicities were higher in men and multiplicities higher in women. The multiple cases there were 19.5% of total new diagnosed neoplasms in men and 18.5% in women, without the skin cancers there were 13.7% in men and 13.6% in women. The most frequent were primary cancers of skin 46%, digestive tract 13.5%, urinary tract 9.6%, genital organs 8.1%, respiratory and intrathoracic organs 7.8% in men and cancers of skin 39.4%, breast 17.3%, genital organs 14.6%, and digestive tract 9.8% in women. The highest percentage of multiplicities in new diagnosed cases were cancers of skin 56.4%, melanoma 22.7%, urinary tract 19.9%, oral cavity 17.8%, genital organs 16.4%, endocrine glands 15.8% and haemopoietic tissues 13.9% in men, the cancers of skin 43.6%, oral cavity 19.8%, melanoma 18.6%, breast 17.7%, urinary tract 17.7%, genital organs 13.5% and endocrine glands 13% in women. The most multiplicities were registered in region Northern Moravia 38,547 (13.3%), Southern Moravia 34,219 (11.8%) and in Prague 34,218 (11.8%). From 125,262 patients, 18,887 (15.1%) men and 22,274 (17.8%) women survived, number of deceased persons was 46,405 (37%) men and 37,696 (30.1%) women. In the view of multiple cancers the broad education of lifestyle appears not to be sufficient for both the healthy population and for patients with 595 thousand cancers, expected in 2010.