cancer of the bile duct

胆管癌

WordNet

1. a special situation; "this thing has got to end"; "it is a remarkable thing"
2. a separate and self-contained entity
3. an action; "how could you do such a thing?"
4. an artifact; "how does this thing work?"
5. an entity that is not named specifically; "I couldnt tell what the thing was"
6. any attribute or quality considered as having its own existence; "the thing I like about her is ..."
7. a persistent illogical feeling of desire or aversion; "he has a thing about seafood"; "she has a thing about him"
8. a special abstraction; "a thing of the spirit"; "things of the heart"
9. a special objective; "the thing is to stay in bounds"
10. a statement regarded as an object; "to say the same thing in other terms"; "how can you say such a thing?"
11. an event; "a funny thing happened on the way to the..."
12. a continuous tube formed by a row of elongated cells lacking intervening end walls
13. an enclosed conduit for a fluid
14. a bodily passage or tube lined with epithelial cells and conveying a secretion or other substance; "the tear duct was obstructed"; "the alimentary canal"; "poison is released through a channel in the snakes fangs" (同)epithelial duct, canal, channel
15. type genus of the family Cancridae (同)genus Cancer
16. the fourth sign of the zodiac; the sun is in this sign from about June 21 to July 22 (同)Cancer the Crab, Crab
17. (astrology) a person who is born while the sun is in Cancer (同)Crab
18. a small zodiacal constellation in the northern hemisphere; between Leo and Gemini

PrepTutorEJDIC

1. 〈C〉(形のある)『物』,物体;(生物に対しての)『無生物』 / 〈C〉(形のない)『もの』,事,事柄,でき事 / 《複数形で》事情,事態,状勢,状況 / 《複数形で》(すべての)事物,事柄 / 《通例 the~》(努力・活動などの)目的,目標 / 〈C〉衣服,衣類・《one's things》持ち物,所持品,身の回り品 / 《複数形で》道具,用具・〈C〉《親愛・哀れみ・軽べつなどの感情をこめて》人,者,やつ / 《the~》《話》(…の)流行《+in+名》
2. 《所有・所属》…『の』,…のものである,…に属する・《材料・要素》…『でできた』,から成る・《部分》…『の』[『中の』] ・《数量・単位・種類を表す名詞に付いて》…の・《原因・動機》…『で』,のために(because of) ・《主格関係》…『の』,による,によって・《目的格関係》…『を』,の・《同格関係》…『という』・《関係・関連》…『についての』[『の』],の点で・《抽象名詞などと共に》…の[性質をもつ] ・《『It is』+『形』+『of』+『名』+『to』 doの形で,ofの後の名詞を意味上の主語として》・《分離》…『から』・《起原・出所》…『から』[『の』](out of) ・《『名』+『of』+『a』(『an』)+『名』の形で》…のような・《『名』+『of』+『mine』(『yours, his』など独立所有格)の形で》…の…・《時》(1)《副詞句を作って》…に《形容詞句を作って》…の・《時刻》《米》…前(to,《米》before)
3. 送水管;(建物・船などの)通気管;(地下ケーブルなどの)ダクト / (体液を運ぶ)導管,(植物が水・空気を運ぶ)導管
4. 《前述の名詞または文脈でそれと分かる名詞に付けて》『その』,あの,名の / 《唯一の事物に付けて》 / 《方角・自然現象に付けて》 / 《用具・楽器・特定の病気などに付けて》 / 《修飾吾[句]を伴う名詞に付けて》 / 《単数普通名詞に付けて》(1)《種族全体》…『というもの』 / 《複数名詞または集合名詞に付けて》 / 《単位》『…につき;…単位[で]』 / 《身体の一部に付けて,所有格に代わる》 / 《形容詞に付けて》 / 《固有名詞に付けて》 / 《強意用法に》『随一の』,典型的な / 《「the+比較級,the+比較級」の形で》『…すればするほどそれだけ』 / 《比較級の前に》(…につれて,のために)『それだけ』,ますます

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2013/10/17 03:59:46」(JST)

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Cholangiocarcinoma
Classification and external resources
Micrograph of an intrahepatic cholangiocarcinoma (right of image) adjacent to benign hepatocytes (left of image). H&E stain.
ICD-10	C22.1
ICD-9	155.1, 156.1
ICD-O:	M8160/3
DiseasesDB	2505
MedlinePlus	000291
eMedicine	med/343 radio/153
MeSH	D018281

Digestive system diagram showing bile duct location.

Cholangiocarcinoma is a medical term denoting a form of cancer that is composed of mutated epithelial cells (or cells showing characteristics of epithelial differentiation) that originate in the bile ducts which drain bile from the liver into the small intestine. Other biliary tract cancers include pancreatic cancer, gallbladder cancer, and cancer of the ampulla of Vater.

Cholangiocarcinoma is a relatively rare neoplasm that is classified as an adenocarcinoma (a cancer that forms glands or secretes significant amounts of mucins). It has an annual incidence rate of 1–2 cases per 100,000 in the Western world, but rates of cholangiocarcinoma have been rising worldwide over the past several decades.

Prominent signs and symptoms of cholangiocarcinoma include abnormal liver function tests, abdominal pain, jaundice, and weight loss. generalized itching, fever, and changes in color of stool or urine may also occur. The disease is diagnosed through a combination of blood tests, imaging, endoscopy, and sometimes surgical exploration, with confirmation obtained after a pathologist examines cells from the tumor under a microscope. Known risk factors for cholangiocarcinoma include primary sclerosing cholangitis (an inflammatory disease of the bile ducts), congenital liver malformations, infection with the parasitic liver flukes Opisthorchis viverrini or Clonorchis sinensis, and exposure to Thorotrast (thorium dioxide), a chemical formerly used in medical imaging. However, most patients with cholangiocarcinoma have no identifiable specific risk factors.

Cholangiocarcinoma is considered to be an incurable and rapidly lethal malignancy unless both the primary tumor and any metastases can be fully resected (removed surgically). No potentially curative treatment yet exists except surgery, but most patients have advanced stage disease at presentation and are inoperable at the time of diagnosis. Patients with cholangiocarcinoma are generally managed - though never cured - with chemotherapy, radiation therapy, and other palliative care measures. These are also used as adjuvant therapies (i.e. post-surgically) in cases where resection has apparently been successful (or nearly so). Some areas of ongoing medical research in cholangiocarcinoma include the use of newer targeted therapies, (such as erlotinib) or photodynamic therapy for treatment, and the techniques to measure the concentration of byproducts of cancer stromal cell formation in the blood for diagnostic purposes.

Staging

Although there are at least three staging systems for cholangiocarcinoma (e.g. those of Bismuth, Blumgart, and the American Joint Committee on Cancer), none have been shown to be useful in predicting survival. The most important staging issue is whether the tumor can be surgically removed, or whether it is too advanced for surgical treatment to be successful. Often, this determination can only be made at the time of surgery.

General guidelines for operability include:

• Absence of lymph node or liver metastases
• Absence of involvement of the portal vein
• Absence of direct invasion of adjacent organs
• Absence of widespread metastatic disease

Signs and symptoms

Yellowing of the skin (jaundice) and eyes (scleral icterus)

The most common physical indications of cholangiocarcinoma are abnormal liver function tests, jaundice (yellowing of the eyes and skin occurring when bile ducts are blocked by tumor), abdominal pain (30%–50%), generalized itching (66%), weight loss (30%–50%), fever (up to 20%), and changes in stool or urine color. To some extent, the symptoms depend upon the location of the tumor: patients with cholangiocarcinoma in the extrahepatic bile ducts (outside the liver) are more likely to have jaundice, while those with tumors of the bile ducts within the liver more often have pain without jaundice.

Blood tests of liver function in patients with cholangiocarcinoma often reveal a so-called "obstructive picture," with elevated bilirubin, alkaline phosphatase, and gamma glutamyl transferase levels, and relatively normal transaminase levels. Such laboratory findings suggest obstruction of the bile ducts, rather than inflammation or infection of the liver parenchyma, as the primary cause of the jaundice. CA19-9 is elevated in most cases of cholangiocarcinoma.

Risk factors

Life cycle of Clonorchis sinensis, a liver fluke associated with cholangiocarcinoma

Although most patients present without any known risk factors evident, a number of risk factors for the development of cholangiocarcinoma have been described. In the Western world, the most common of these is primary sclerosing cholangitis (PSC), an inflammatory disease of the bile ducts which is itself closely associated with ulcerative colitis (UC). Epidemiologic studies have suggested that the lifetime risk of developing cholangiocarcinoma for a person with PSC is on the order of 10%–15%, although autopsy series have found rates as high as 30% in this population. The mechanism by which PSC increases the risk of cholangiocarcinoma is not well understood.

Certain parasitic liver diseases may be risk factors as well. Colonization with the liver flukes Opisthorchis viverrini (found in Thailand, Laos, and Malaysia) or Clonorchis sinensis (found in Japan, Korea, and Vietnam) has been associated with the development of cholangiocarcinoma. Patients with chronic liver disease, whether in the form of viral hepatitis (e.g. hepatitis B or hepatitis C), alcoholic liver disease, or cirrhosis of the liver due to other causes, are at significantly increased risk of cholangiocarcinoma. HIV infection was also identified in one study as a potential risk factor for cholangiocarcinoma, although it was unclear whether HIV itself or other correlated and confounding factors (e.g. hepatitis C infection) were responsible for the association.

Congenital liver abnormalities, such as Caroli's syndrome or choledochal cysts, have been associated with an approximately 15% lifetime risk of developing cholangiocarcinoma. The rare inherited disorders Lynch syndrome II and biliary papillomatosis have also been found to be associated with cholangiocarcinoma. The presence of gallstones (cholelithiasis) is not clearly associated with cholangiocarcinoma. However, intrahepatic stones (called hepatolithiasis), which are rare in the West but common in parts of Asia, have been strongly associated with cholangiocarcinoma. Exposure to Thorotrast, a form of thorium dioxide which was used as a radiologic contrast medium, has been linked to the development of cholangiocarcinoma as late as 30–40 years after exposure; Thorotrast was banned in the United States in the 1950s due to its carcinogenicity.

Pathophysiology

Cholangiocarcinoma can affect any area of the bile ducts, either within or outside the liver. Tumors occurring in the bile ducts within the liver are referred to as intrahepatic, those occurring in the ducts outside the liver are extrahepatic, and tumors occurring at the site where the bile ducts exit the liver may be referred to as perihilar. A cholangiocarcinoma occurring at the junction where the left and right hepatic ducts meet to form the common bile duct may be referred to eponymously as a Klatskin tumor.

Although cholangiocarcinoma is known have the histological and molecular features of an adenocarcinoma of epithelial cells lining the biliary tract, the actual cell of origin is unknown. Recent evidence has suggested that the initial transformed cell that generates the primary tumor may arise from a pluripotent hepatic stem cell. Cholangiocarcinoma is thought to develop through a series of stages - from early hyperplasia and metaplasia, through dysplasia, to the development of frank carcinoma - in a process similar to that seen in the development of colon cancer. Chronic inflammation and obstruction of the bile ducts, and the resulting impaired bile flow, are thought to play a role in this progression.

Histologically, cholangiocarcinomas may vary from undifferentiated to well-differentiated. They are often surrounded by a brisk fibrotic or desmoplastic tissue response; in the presence of extensive fibrosis, it can be difficult to distinguish well-differentiated cholangiocarcinoma from normal reactive epithelium. There is no entirely specific immunohistochemical stain that can distinguish malignant from benign biliary ductal tissue, although staining for cytokeratins, carcinoembryonic antigen, and mucins may aid in diagnosis. Most tumors (>90%) are adenocarcinomas.

Diagnosis

Micrograph of an intrahepatic, i.e. in the liver, cholangiocarcinoma (right of image); benign hepatocytes are seen (left of image). Histologically, this is a cholangiocarcinoma as (1) atypical bile duct-like cells (left of image) extend from the tumor in an interlobular septum (the normal anatomical location of bile ducts), and (2) the tumor has the abundant desmoplastic stroma often seen in cholangiocarcinomas. A portal triad (upper-left of image) has a histologically normal bile duct. H&E stain.

Cholangiocarcinoma is definitively diagnosed from tissue, i.e. it is proven by biopsy or examination of the tissue excised at surgery. It may be suspected in a patient with obstructive jaundice. Considering it as the working diagnosis may be challenging in patients with primary sclerosing cholangitis (PSC); such patients are at high risk of developing cholangiocarcinoma, but the symptoms may be difficult to distinguish from those of PSC. Furthermore, in patients with PSC, such diagnostic clues as a visible mass on imaging or biliary ductal dilatation may not be evident.

Blood tests

There are no specific blood tests that can diagnose cholangiocarcinoma by themselves. Serum levels of carcinoembryonic antigen (CEA) and CA19-9 are often elevated, but are not sensitive or specific enough to be used as a general screening tool. However, they may be useful in conjunction with imaging methods in supporting a suspected diagnosis of cholangiocarcinoma.

Abdominal imaging

CT scan showing cholangiocarcinoma

Ultrasound of the liver and biliary tree is often used as the initial imaging modality in patients with suspected obstructive jaundice. Ultrasound can identify obstruction and ductal dilatation and, in some cases, may be sufficient to diagnose cholangiocarcinoma. Computed tomography (CT) scanning may also play an important role in the diagnosis of cholangiocarcinoma.

Imaging of the biliary tree

ERCP image of cholangiocarcinoma, showing common bile duct stricture and dilation of the proximal common bile duct

While abdominal imaging can be useful in the diagnosis of cholangiocarcinoma, direct imaging of the bile ducts is often necessary. Endoscopic retrograde cholangiopancreatography (ERCP), an endoscopic procedure performed by a gastroenterologist or specially trained surgeon, has been widely used for this purpose. Although ERCP is an invasive procedure with attendant risks, its advantages include the ability to obtain biopsies and to place stents or perform other interventions to relieve biliary obstruction. Endoscopic ultrasound can also be performed at the time of ERCP and may increase the accuracy of the biopsy and yield information on lymph node invasion and operability. As an alternative to ERCP, percutaneous transhepatic cholangiography (PTC) may be utilized. Magnetic resonance cholangiopancreatography (MRCP) is a non-invasive alternative to ERCP. Some authors have suggested that MRCP should supplant ERCP in the diagnosis of biliary cancers, as it may more accurately define the tumor and avoids the risks of ERCP.

Surgery

Photograph of cholangiocarcinoma in human liver.

Surgical exploration may be necessary to obtain a suitable biopsy and to accurately stage a patient with cholangiocarcinoma. Laparoscopy can be used for staging purposes and may avoid the need for a more invasive surgical procedure, such as laparotomy, in some patients. Surgery is also the only curative option for cholangiocarcinoma, although it is limited to patients with early-stage disease.

Pathology

Histologically, cholangiocarcinomas are classically well to moderately differentiated adenocarcinomas. Immunohistochemistry is useful in the diagnosis and may be used to help differentiate a cholangiocarcinoma from, hepatocellular carcinoma and metastasis of other gastrointestinal tumors. Cytological scrapings are often nondiagnostic, as these tumors typically have a desmoplastic stroma and, therefore, do not release diagnostic tumor cells with scrapings.

Treatment

Cholangiocarcinoma is considered to be an incurable and rapidly lethal disease unless all the tumors can be fully resected (that is, cut out surgically). Since the operability of the tumor can only be assessed during surgery in most cases, a majority of patients undergo exploratory surgery unless there is already a clear indication that the tumor is inoperable.

Adjuvant therapy followed by liver transplantation may have a role in treatment of certain unresectable cases.

Adjuvant chemotherapy and radiation therapy

If the tumor can be removed surgically, patients may receive adjuvant chemotherapy or radiation therapy after the operation to improve the chances of cure. If the tissue margins are negative (i.e. the tumor has been totally excised), adjuvant therapy is of uncertain benefit. Both positive and negative results have been reported with adjuvant radiation therapy in this setting, and no prospective randomized controlled trials have been conducted as of March 2007. Adjuvant chemotherapy appears to be ineffective in patients with completely resected tumors. The role of combined chemoradiotherapy in this setting is unclear. However, if the tumor tissue margins are positive, indicating that the tumor was not completely removed via surgery, then adjuvant therapy with radiation and possibly chemotherapy is generally recommended based on the available data.

Treatment of advanced disease

The majority of cases of cholangiocarcinoma present as inoperable (unresectable) disease in which case patients are generally treated with palliative chemotherapy, with or without radiotherapy. Chemotherapy has been shown in a randomized controlled trial to improve quality of life and extend survival in patients with inoperable cholangiocarcinoma. There is no single chemotherapy regimen which is universally used, and enrollment in clinical trials is often recommended when possible. Chemotherapy agents used to treat cholangiocarcinoma include 5-fluorouracil with leucovorin, gemcitabine as a single agent, or gemcitabine plus cisplatin, irinotecan, or capecitabine. A small pilot study suggested possible benefit from the tyrosine kinase inhibitor erlotinib in patients with advanced cholangiocarcinoma.

Photodynamic therapy, an experimental approach in which patients are injected with a light-sensitizing agent and light is then applied endoscopically directly to the tumor, has shown promising results compared to supportive care in two small randomized controlled trials. However, its ultimate role in the management of cholangiocarcinoma is unclear at present. Photodynamic Therapy has been shown to improve survival and quality of life

Prognosis

Surgical resection offers the only potential chance of cure in cholangiocarcinoma. For non-resectable cases, the 5-year survival rate is 0% where the disease is inoperable because distal lymph nodes show metastases, and less than 5% in general. Overall median duration of survival is less than 6 months in inoperable, untreated, otherwise healthy patients with tumors involving the liver by way of the intrahepatic bile ducts and hepatic portal vein.

For surgical cases, the odds of cure vary depending on the tumor location and whether the tumor can be completely, or only partially, removed. Distal cholangiocarcinomas (those arising from the common bile duct) are generally treated surgically with a Whipple procedure; long-term survival rates range from 15%–25%, although one series reported a five-year survival of 54% for patients with no involvement of the lymph nodes. Intrahepatic cholangiocarcinomas (those arising from the bile ducts within the liver) are usually treated with partial hepatectomy. Various series have reported survival estimates after surgery ranging from 22%–66%; the outcome may depend on involvement of lymph nodes and completeness of the surgery. Perihilar cholangiocarcinomas (those occurring near where the bile ducts exit the liver) are least likely to be operable. When surgery is possible, they are generally treated with an aggressive approach often including removal of the gallbladder and potentially part of the liver. In patients with operable perihilar tumors, reported 5-year survival rates range from 20%–50%.

The prognosis may be worse for patients with primary sclerosing cholangitis who develop cholangiocarcinoma, likely because the cancer is not detected until it is advanced. Some evidence suggests that outcomes may be improving with more aggressive surgical approaches and adjuvant therapy.

Epidemiology

Cholangiocarcinoma is an adenocarcinoma of the biliary tract, along with pancreatic cancer (which occurs about 20 times more frequently), gall bladder cancer (which occurs twice as often), and cancer of the ampulla of Vater. Treatments and clinical trials for pancreatic cancer, being far more prevalent, are often taken as a starting point for managing cholangiocarcinoma, even though the biologies are different enough that chemotherapies can put pancreatic cancer into permanent remission whereas there are no reports in the literature of long-term survival due to chemotherapy or radiation applied to an inoperable cholangiocarcinoma case.

Age-standardized mortality rates from intrahepatic (IC) and extrahepatic (EC) cholangiocarcinoma for men and women, by country. Source: Khan et al., 2002.

Country	IC (men/women)	EC (men/women)
U.S.A.	0.60 / 0.43	0.70 / 0.87
Japan	0.23 / 0.10	5.87 / 5.20
Australia	0.70 / 0.53	0.90 / 1.23
England/Wales	0.83 / 0.63	0.43 / 0.60
Scotland	1.17 / 1.00	0.60 / 0.73
France	0.27 / 0.20	1.20 / 1.37
Italy	0.13 / 0.13	2.10 / 2.60

Cholangiocarcinoma is a relatively rare form of cancer; each year, approximately 2,000 to 3,000 new cases are diagnosed in the United States, translating into an annual incidence of 1–2 cases per 100,000 people. Autopsy series have reported a prevalence of 0.01% to 0.46%. There is a higher prevalence of cholangiocarcinoma in Asia, which has been attributed to endemic chronic parasitic infestation. The incidence of cholangiocarcinoma increases with age, and the disease is slightly more common in men than in women (possibly due to the higher rate of primary sclerosing cholangitis, a major risk factor, in men). The prevalence of cholangiocarcinoma in patients with primary sclerosing cholangitis may be as high as 30%, based on autopsy studies.

Multiple studies have documented a steady increase in the incidence of intrahepatic cholangiocarcinoma over the past several decades; increases have been seen in North America, Europe, Asia, and Australia. The reasons for the increasing occurrence of cholangiocarcinoma are unclear; improved diagnostic methods may be partially responsible, but the prevalence of potential risk factors for cholangiocarcinoma, such as HIV infection, has also been increasing during this time frame.

Notable Victims

Ray Manzarek, keyboard player of The Doors, died from bile duct cancer.

Walter Payton, Legendary running back, Chicago Bears

Notes

External links

• American Cancer Society Detailed Guide to Bile Duct Cancer.
• Patient information on extrahepatic bile duct tumors, from the National Cancer Institute.
• Cancer.Net: Bile Duct Cancer
• The Cholangiocarcinoma Foundation: a resource for patients, friends, caregivers and loved ones of those affected by bile duct cancer.
• The Alan Morement Memorial Fund, the UK's only charity dedicated to the disease
• Macmillan/Cancerbackup page on Cholangiocarcinoma

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

• 1. 胆管切除術および再建術 bile duct resection and reconstruction
• 2. Overview of the complications of chronic pancreatitis
• 3. 肝切除の概要 overview of hepatic resection
• 4. 胆道鏡検査および膵管鏡検査 cholangioscopy and pancreatoscopy
• 5. 膵胆管系の管腔内超音波検査 intraductal ultrasound of the pancreaticobiliary ductal system

English Journal

• Hepatic fibrosis and angiogenesis after bile duct ligation are endogenously expressed vasohibin-1 independent.

• Furutani Y1, Shiozaki-Sato Y1, Hara M1, Sato Y2, Kojima S3.
• Biochemical and biophysical research communications.Biochem Biophys Res Commun.2015 Jul 31;463(3):384-8. doi: 10.1016/j.bbrc.2015.05.074. Epub 2015 May 27.
• Liver fibrosis is linked to VEGF-induced angiogenesis. Overexpression of exogenous vasohibin-1, a feedback inhibitor of angiogenesis, has been reported to reduce liver fibrosis after bile duct ligation (BDL). To uncover the function of endogenous vasohibin-1, we performed BDL using vasohibin-1-defic
• PMID 26025651

• Intraductal Ultrasonography without Radiocontrast Cholangiogramin Patients with Extrahepatic Biliary Disease.

• Lim SU1, Park CH1, Kee WJ1, Lee JH1, Rew SJ2, Park SY1, Kim HS1, Choi SK1, Rew JS1.
• Gut and liver.Gut Liver.2015 Jul 23;9(4):540-6. doi: 10.5009/gnl14200.
• BACKGROUND/AIMS: Intraductal ultrasonography (IDUS) has been performed as an adjunct to endoscopic retrograde cholangiography (ERC) during radiocontrast cholangiography (RC). Radiation exposure during RC poses a health risk to both patients and examiners. We evaluated the feasibility of IDUS without
• PMID 25963077

• Curative Resection After Gemcitabine, Cisplatin and S-1 Chemotherapy for Initially Unresectable Biliary Duct Cancer: A Case Report.

• Furukawa K1, Uwagawa T2, Sakamoto T2, Shiba H2, Tsutsumi J3, Yanaga K2.
• Anticancer research.Anticancer Res.2015 Jul;35(7):4203-6.
• A 68-year-old woman was diagnosed with unresectable upper bile duct cancer with suspected invasion of the right hepatic artery and para-aortic lymph node metastasis (T4N3M0, stage IVb). She underwent plastic stent placement for obstructive jaundice and enrolled in our phase I study for unresectable
• PMID 26124379

Japanese Journal

• 症例報告 検診にて発見された胆管内乳頭状腫瘍(IPNB)の1例

• 肱岡 範,丹羽 康正,田中 努 [他]
• 日本消化器がん検診学会雑誌 = Journal of
• NAID 40020030404

• IgG4関連硬化性胆管炎診断における内視鏡の役割

• 内藤 格,大原 弘隆,中沢 貴宏,林 香月,城 卓志
• 日本消化器内視鏡学会雑誌 56(3), 433-442, 2014
• IgG4関連硬化性胆管炎（IgG4-SC）は，血中IgG4値の上昇，病変局所の線維化とIgG4陽性形質細胞の著しい浸潤，ステロイドに対する良好な反応性などを特徴とする原因不明の硬化性胆管炎である．近年，IgG4関連性疾患とともに，その疾患概念は定着しつつあり，本邦においてもIgG4-SC臨床診断基準2012が作成された．診断基準2012は1）胆管の特徴的な画像所見，2）高IgG4血症，3）胆管外の …
• NAID 130003395968

• Incidence of Malignancies in Patients with IgG4-related Disease

• Hirano Kenji,Tada Minoru,Sasahira Naoki,Isayama Hiroyuki,Mizuno Suguru,Takagi Kaoru,Watanabe Takeo,Saito Tomotaka,Kawahata Shuhei,Uchino Rie,Hamada Tsuyoshi,Miyabayashi Koji,Mohri Dai,Sasaki Takashi,Kogure Hirofumi,Yamamoto Natsuyo,Nakai Yousuke,Yoshida Haruhiko,Ito Yukiko,Akiyama Dai,Toda Nobuo,Arizumi Toshihiko,Yagioka Hiroshi,Takahara Naminatsu,Matsubara Saburo,Yashima Yoko,Koike Kazuhiko
• Internal Medicine 53(3), 171-176, 2014
• … however, the issue has not been clarified. … Methods We analyzed 113 patients with IgG4-RD in whom malignancy was not diagnosed at the time of IgG4-RD onset and the follow-up period was longer than six months. … A total of 95 patients had AIP. … The mean follow-up period was 73 months. …
• NAID 130003392645

Related Pictures

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★リンクテーブル★

リンク元	「胆管癌」「胆管がん」「bile duct neoplasm」
関連記事	「duct」「thing」「cancer」

「胆管癌」

　　[★]

英

cholangiocarcinoma CCC, bile duct cancer, bile duct carcinoma, cancer of the bile duct

同

胆管細胞癌 cholangiocellular carcinoma, primary cholangiocellular carcinoma

ラ

cholangioma

関

肝内胆管癌、胆道癌

概念

• 胆管に発生する癌腫の総称
• 胆管癌とは通常肝外胆管癌のことであり、肝内胆管細胞癌とは区別される。

部位による分類

• 肝内胆管癌
• 肝外胆管癌

• 肝門部胆管癌
• 上部胆管癌：肝外胆管中枢側と下部胆管中枢側を二等分したときの、その中枢側？
• 中部胆管癌：肝外胆管中枢側と下部胆管中枢側を二等分したときの、その末梢側？
• 下部胆管癌：膵臓内の胆管？

早期癌

早期胆管癌：組織学的深達度が粘膜(m)・線維筋層(fm)にとどまるもの。リンパ節転移の有無は問わない。

疫学

• 高齢男性
• 男女比=2:1。50歳代、60歳代に好発し60歳代が最多　(⇔胆嚢癌)

リスク因子

• 膵胆管合流異常、原発性硬化性胆管炎、炎症性腸疾患、肝吸虫症

症状

• 初発症状は黄疸(31%)、腹痛(13%)、全身倦怠感(11%)、食思不振(9%)、暗色尿(8%)、発熱(5%)、掻痒感(4%)、背部痛(4%)、嘔気・嘔吐(3%)、体重減少(2%)

検査

• 内視鏡的逆行性胆管造影 ERCP

肝門部胆管癌

[show details]

治療

• 胆道癌の唯一の根治治療は外科切除

手術療法(ガイドラインより)

• 標準術式

• 肝門部・上部胆管：胆管切除＋肝切除
• 中下部胆管癌　　：膵頭十二指腸切除

• 切除不能例

胆道癌の肝，肺，腹膜転移，遠隔リンパ節（大動脈周囲リンパ節，腹腔外リンパ節など）転移は切除不能

ガイドライン

• http://minds.jcqhc.or.jp/stc/0058/1/0058_G0000159_GL.html

資料

• 胆道癌の診断

• http://www.cancertherapy.jp/biliary/2009_winter/pdf/2009winter_02_01.pdf

国試

• 105A031

「胆管がん」

　　[★]

英

cholangiocarcinoma、bile duct cancer、bile duct carcinoma、cancer of the bile duct

関

胆管癌、胆管腫瘍

「bile duct neoplasm」

　　[★]

胆管腫瘍、胆管新生物

関

bile duct cancer、cancer of the bile duct

「duct」

　　[★]

• n.

• 管、(植物)導管

関

canal、conduit、ductal、ductus、meatus、pipe、tubal、tube、vas、vessel

　 　 　 　

「thing」

　　[★] 　 　 　 　 　 　 　 　 　 　 　 　

「cancer」

　　[★]

同

癌腫

関

tumor