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Klatskin Tumor

Hilar Cholangiocarcinoma

Klatskin tumors are cholangiocarcinomas that develop at the point where the left and right hepatic ducts join to form the common hepatic duct. They are malignant tumors that were first described in 1965.


Presentation

Cholangiocarcinomas (CCCs) are malignant tumors of the biliary tree that arise from the bile ducts or the liver. Klatskin tumors (KTs) are also referred to as hilar or perihilar cholangiocarcinomas [1]. A large proportion of all cholangiocarcinomas are Klatskin tumors. They are usually undifferentiated and grow rapidly. The majority of occurrences of KTs are sporadic, however, the prevalence is still unknown.

Klatskin tumors are more common in men and are typically reported in older individuals in their 6th decade of life [2]. KTs normally do not produce symptoms until they have progressed to a late stage. This contributes to their poor prognosis. When tumors grow large enough, they may cause obstructive jaundice [3]. KTs are the most commonly reported cause of proximal biliary obstruction, accounting for approximately 80% of such cases [4]. Other signs of disease are non-specific and include abdominal pain, loss of weight, malaise, and fatigue. Cholangitis is less frequently encountered.

KTs are known to metastasize to regional lymph nodes in the abdomen. Metastases can also occur to the head of the pancreas and can spread distally via the systemic or portal circulation.

Certain factors predispose individuals to the development of KTs, the most prominent being primary sclerosing cholangitis (PSC). It is thought to be responsible for up to 40% of KTs. Other risk factors include chronic inflammation of the biliary tree, parasitic infections, namely ascariasis and schistosomiasis, chemical substances exemplified by thorotrast (X-ray contrast) and asbestos, some congenital diseases of the biliary tree, liver cirrhosis, and cholelithiasis [5]. Many patients have comorbidities such as inflammatory bowel disease and viral hepatitis [3] [6].

Lymphadenopathy
  • Apart from altered liver function, her lactate dehydrogenase was significantly elevated, and imaging studies showed prominent lesion close to the liver bed with localized lymphadenopathy.[ncbi.nlm.nih.gov]
  • It is the most common type of cholangiocarcinoma, accounting for more than half of all cases. 0000952 30%-79% of people have these symptoms Hepatomegaly Enlarged liver 0002240 Lymphadenopathy Swollen lymph nodes 0002716 5%-29% of people have these symptoms[rarediseases.info.nih.gov]
  • Physical examination revealed a jaundiced patient with right upper quadrant tenderness but no overt abdominal masses or lymphadenopathy.[consultant360.com]
  • Diagnosis: Klatskin tumour with biliary obstruction and para-aortic lymphadenopathy. Her blood test results are as below.[cancercaremalaysia.com]
  • There was no supraclavicular lymphadenopathy. Pruritus marks were present over her body.[tropicalgastro.com]
Asymptomatic
  • In this manuscript, we share our experience with an asymptomatic patient with elevated liver enzymes, who was found to have a Klatskin tumor, extending into the left hepatic duct. The patient's diagnosis, management, and prognosis are discussed.[ncbi.nlm.nih.gov]
  • الصفحة 489 - Executive Committee for the Asymptomatic Carotid Atherosclerosis Study. Endarterectomy for asymptomatic carotid artery stenosis. ‏ الصفحة 245 - III. Tears of the anterior cruciate ligament and menisci of the knee: MR imaging evaluation.[books.google.com]
  • Patients are usually asymptomatic until advanced stages of the disease where jaundice is the principle manifestation. Abdominal pain, weight loss and malaise are other manifestations experienced by some patients.[orpha.net]
  • Brown, Asymptomatic Liver Mass, Gastroenterology, 131, 2, (619), (2006). Thomas Gerhardt, C-reactive protein is a prognostic indicator in patients with perihilar cholangiocarcinoma, World Journal of Gastroenterology, 12, 34, (5495), (2006).[dx.doi.org]
Soft Tissue Mass
  • Abdominal computed tomography (CT) showed intrahepatic and extrahepatic bile duct dilatation and a soft tissue mass in the hepatic hilum.[ncbi.nlm.nih.gov]
Collapse
  • Findings on either study suggestive of Klatskin tumor include a dilated intrahepatic biliary tree, a normal or collapsed gallbladder and extrahepatic biliary tree, and a normal pancreas.[ncbi.nlm.nih.gov]
Upper Abdominal Pain
  • We report the case of a 65-year old male patient who initially presented with recurrent episodes of upper abdominal pain, lack of appetite and weight loss.[ncbi.nlm.nih.gov]
Right Upper Quadrant Pain
  • A patient presented with obstructive jaundice along with right upper quadrant pain, weight loss, and decreased appetite.[ncbi.nlm.nih.gov]
Cyanosis
  • There was no pallor, cyanosis, clubbing or edema. There was no supraclavicular lymphadenopathy. Pruritus marks were present over her body.[tropicalgastro.com]
Pruritus
  • These include jaundice (yellowing of the skin), clay-colored stools (because bile normally darkens the stool, but in this case is not draining into the bowel), bilirubinuria (dark urine), pruritus (itchy skin), weight loss, and abdominal pain.[oncolink.org]
  • Pruritus marks were present over her body. Abdominal examination revealed fullness present in right upper abdomen and a right paramedian scar, which was 12 cm 10 cm incisional hernia with large defect present around the scar. Vitals were normal.[tropicalgastro.com]
Testicular Pain
  • A 38-year-old man presented with right scrotal enlargement and chronic testicular pain.[ncbi.nlm.nih.gov]

Workup

There are various clinical, laboratory and imaging studies that are needed in order to make the diagnosis of Klatskin tumor. Imaging techniques include:

  • Ultrasound: This may show evidence of a hyperechoic mass that may cause obstruction at the bifurcation of the common hepatic duct [7]. Endoscopic ultrasound (EUS) is an accurate technique that can be used to facilitate fine needle aspiration (FNA) for cytology [8].
  • Computerized tomography (CT): Direct visualization of the tumor via CT scanning is usually not possible, however, biliary tract dilatation, metastases and affected lymph nodes can be seen.
  • Magnetic resonance cholangiopancreatography (MRCP): This is the preferred technique, and the gold standard when it comes to diagnosis of KTs, as it is the most accurate in determining the location and extension of the tumor.
  • Magnetic resonance imaging (MRI): This reveals similar findings to CT scanning.
  • Positron emission (PET): PET scans can be utilized to detect small tumors, as well as distant metastases [9].

Staging of the tumor is necessary for planning treatment, as advanced stages of KT are inoperable. Using the Bismuth-Corlette classification, there are four types of hilar CCCs, of which type IV is the most advanced, involving both hepatic ducts.

The resultant cholestasis from KTs can be detected by measuring the levels of tumor markers such as carbohydrate antigen 19-9 (CA 19-9) and carcinoembryonic antigen (CEA) [10]. Levels of over 100 units per milliliter are a good diagnostic indicator for CCC in patients with PSC [11]. Additionally, liver function tests (LFTs) may show elevated levels of parameters such as alkaline phosphate (ALP) and gamma-glutamyl transpeptidase (GGT).

Pancreatic Necrosis
  • In the 3 patients who died in the postoperative period (hospital mortality 7%; table 1 ), liver failure was initiated by local complications (portal vein thrombosis, loss of segmental hepatic artery, pancreatic necrosis) rather than insufficient remnant[karger.com]

Treatment

  • In the context of inter-disciplinary treatment concepts, this radiotherapy technique is a promising choice of treatment for patients with hilar CCC.[ncbi.nlm.nih.gov]
  • Despite emerging adjuvant treatment regimens, survival outcomes remain poor. Surgery is currently the standard of care and the only curative treatment modality available.[ncbi.nlm.nih.gov]
  • This technique was feasible and could be considered for the treatment of patients with Bismuth type IV tumors.[ncbi.nlm.nih.gov]
  • Changes in the clinical symptoms after the PTBD treatment, the radiotherapy effect, and the survival time were recorded and analyzed. RESULTS: The clinical symptoms of the 2 groups gradually decreased after the PTBD treatment.[ncbi.nlm.nih.gov]
  • MRI could be useful to differentiate such presentation of peripheral cholangiocracinoma from Klatskin tumor and orientate the surgical treatment.[ncbi.nlm.nih.gov]

Prognosis

  • PcBCL with spindle-cell morphology has been described as a rare variant of pcFCL but the prognosis data of this variant is sparse.[ncbi.nlm.nih.gov]
  • The patient's diagnosis, management, and prognosis are discussed. The case highlights the importance of screening for hepato-biliary malignancies in elderly patients with abnormal liver function markers.[ncbi.nlm.nih.gov]
  • Treatment consists of surgical excision after which prognosis is favorable. Copyright 2000 S. Karger AG, Basel[ncbi.nlm.nih.gov]
  • In view of the poor prognosis of patients with cholangiocarcinoma (CCC), there is a need for new therapeutic strategies. Inter-disciplinary therapy seems to be most promising.[ncbi.nlm.nih.gov]
  • The prognosis of patients undergoing OLT is encouraging.[ncbi.nlm.nih.gov]

Etiology

  • Etiology In 90% of cases Klatskin tumors occur sporadically but certain risk factors have been associated with the disease.[orpha.net]
  • An unusual etiology of biliary hilar obstruction and the potential role of acidic fibroblast growth factor in the development of a biliary neuroma. Am Surgeon. 1999;65:47–51. Zeff R, Pfeffer R, Adams P, Ruoff M.[tropicalgastro.com]
  • Part 1: epidemiology and etiology. J Gastroenterol Hepatol 17: 1049-55 3 Patel T (2001) Increasing incidence and mortality of primary intrahepatic cholangiocarcinoma in the United States. Hepatology 33: 1353-7 .[codes.iarc.fr]
  • Part 1: epidemiology and etiology. J Gastroenterol Hepatol, 2002, 17(10): 1049–1055 CrossRef PubMed Google Scholar 30. Wu T T, Levy M, Correa A M, Rosen C B, Abraham S C.[doi.org]

Epidemiology

  • […] of Cancer Epidemiology and Genetics, National Cancer Institute, EPS 7084, MSC 7234 6120 Executive Blvd., Bethesda, MD 20892 (e-mail: welzelt@mail.nih.gov ).[academic.oup.com]
  • Neurology and Neurosurgery Czech and Slovak Ophthalmology Czech and Slovak Psychiatry Czech Geriatric Review Czech Gynaecology Czech Rheumatology Czech Dental Journal Czech Urology Czech-Slovak Dermatology Czecho-Slovak Pathology Czech-Slovak Pediatrics Epidemiology[prolekare.cz]
  • Part 1: epidemiology and etiology. J Gastroenterol Hepatol, 2002, 17(10): 1049–1055 CrossRef PubMed Google Scholar 30. Wu T T, Levy M, Correa A M, Rosen C B, Abraham S C.[doi.org]
Sex distribution
Age distribution

Pathophysiology

  • In: Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology, Diagnosis, Management. 10th ed. Philadelphia, Pa.: Saunders Elsevier; 2016. . Accessed Feb. 22, 2016. Rizvi S, et al.[mayoclinic.org]
  • Unlike others on this topic, this text demonstrates how the endoscope incorporates pathophysiology, diagnostic, and treatment modalities into endoscopic practice.[books.google.com]
  • […] without involvement of the secondary intrahepatic ducts Type IIIa - Extends into the right secondary intrahepatic duct Type IIIb - Extends into the left secondary intrahepatic duct Type IV - Involvement of the secondary intrahepatic ducts on both sides Pathophysiology[emedicine.com]

Prevention

  • CONCLUSION: The air contrast cholangiography is a safe and effective method, and it appears justified as a routine procedure to prevent or reduce the risk of post-ERCP cholangitis, specially in Klatskin patients (p 0.005).[ncbi.nlm.nih.gov]
  • Preoperative work-up included staging laparoscopy, preoperative biliary drainage, assessment of volume/function of future remnant liver and radiation therapy to prevent seeding metastases.[ncbi.nlm.nih.gov]
  • Preoperative workup included staging laparoscopy, preoperative biliary drainage, assessment of volume/function of future remnant liver and radiation therapy to prevent seeding metastases.[ncbi.nlm.nih.gov]
  • Preventive measures For the prevention of neoplastic diseases of the liver and bile ducts, it is necessary to adhere to proper nutrition, not to abuse alcohol and to prevent the infection with parasites.[acikgunluk.net]
  • Special chapters, such as the Designing Clinical Research in Gastrointestinal Endoscopy, The Prevention of Infection Following Gastrointestinal Endoscopy, Complications of Peptic Ulcer Disease, and Credentialing for Gastrointestinal Endoscopy, help make[books.google.com]

References

Article

  1. Banales JM, Cardinale V, Carpino G, et al. Expert consensus document: Cholangiocarcinoma: current knowledge and future perspectives consensus statement from the European Network for the Study of Cholangiocarcinoma (ENS-CCA). Nat Rev Gastroenterol Hepatol. 2016;13(5):261-280.
  2. Cannon RM, Brock G, Buell JF. Surgical resection for hilar cholangiocarcinoma: experience improves resectability. HPB (Oxford). 2012;14(2):142–149.
  3. Saxena A, Chua TC, Chu FC, Morris DL. Improved outcomes after aggressive surgical resection of hilar cholangiocarcinoma: a critical analysis of recurrence and survival. Am J Surg. 2011;202(3):310-320.
  4. Are C, Gonen M, D’Angelica M, et al. Differential diagnosis of proximal biliary obstruction. Surgery. 2006;140(5):756-63.
  5. Suarez-Munoz MA, Fernandez-Aguilar JL, Sanchez-Perez B, et al. Risk factors and classifications of hilar cholangiocarcinoma. World J Gastrointest Oncol. 2013;5(7):132–138.
  6. Zheng-Rong L, Hai-Bo Y, Xin C, et al. Resection and drainage of hilar cholangiocarcinoma: an 11-year experience of a single center in mainland China. Am Surg. 2011;77(5):627-633.
  7. Yeung EY, Mccarthy P, Gompertz RH, Benjamin S, Gibson RN, Dawson P. The ultrasonographic appearances of hilar cholangiocarcinoma (Klatskin tumours). Br J Radiol. 1988;61(731):991-995.
  8. Fritscher-Ravens A, Broering DC, Knoefel WT, et al. EUS-guided fine-needle aspiration of suspected hilar cholangiocarcinoma in potentially operable patients with negative brush cytology. Am J Gastroenterol. 2004 Jan;99(1):45-51.
  9. Petrowsky H, Wildbrett P, Husarik DB, et al. Impact of Integrated PET and CT on staging and management of glabladder cancer and cholangiocarcinoma. J Hepatol. 2006;45(1):43-50.
  10. Berardi R, Mocchegiani F, Pierantoni C, et al. Resected biliary tract cancers: a novel clinical-pathological score correlates with global outcome. Dig Liver Dis. 2013;45(1):70-74.
  11. Chalasani N, Baluyut A, Ismail A, et al. Cholangiocarcinoma in patients with primary sclerosing cholangitis: a multicenter case-control study. Hepatology. 2000;31(1):7-11.

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Last updated: 2019-07-11 22:03