Klatskin Tumor in the Light of ICD-O-3: A Population-Based Clinical Outcome Study Involving 1,144 Patients from the Surveillance, Epidemiology, and End Result (SEER) Database (2001-2012)

Abstract

IntroductionKlatskin tumors (KTs) occur at the confluence of the right and left extrahepatic ducts and are classified based on their anatomical and histological codes in the International Classification of Diseases for Oncology (ICD-O). The second edition of the ICD-O (ICD-O-2) allocated a distinctive histological code to KT, which also included intrahepatic cholangiocarcinoma (CC). This unclear coding may result in ambiguous reporting of the demographic and clinical features of KT. The current study aimed to investigate the demographic, clinical, and pathological factors affecting the prognosis and survival of KT in the light of the updated third edition of ICD-O, Ninth Revision (ICD-O-3). MethodsData of 1,144 patients with KT from the Surveillance, Epidemiology, and End Result (SEER) database (2001)(2002)(2003)(2004)(2005)(2006)(2007)(2008)(2009)(2010)(2011)(2012) were extracted. Patients with KT were analyzed for age, sex, race, stage, treatment, and long-term survival. The data were analyzed using chi-square tests, t-tests, and univariate and multivariate analyses. The Kaplan-Meier analysis was used to compare long-term survival between KT and subgroups of all biliary CCs. ResultsOf all biliary CCs, KT comprised 9.35%, with a mean age of diagnosis of 73±13 years, and was more common in men (54.8%) and Caucasian patients (69.5%). Histologically, moderately differentiated tumors were the most common (38.9%) followed by poorly differentiated (35.7%), well-differentiated (23.3%), and undifferentiated tumors (2.2%) (p<0.001). Most tumors in the KT group were 2-4 cm in size (41.5%), while fewer were >4 cm (29.7%) and <2 cm (28.8%) (p<0.001). ICD-O-3 defined most KTs in extrahepatic location (53.5%), while the remainder were in other biliary locations (46.5%) (p<0.001). Most KT patients received no treatment (73%), and for those who were treated, the most frequent modality was radiation (52.7%), followed by surgery (28.1%), and both surgery and radiation (19.2%) (p<0.001). Mean survival time for KT patients treated with surgery was inferior to all CCs of the biliary tree (1.72±2.61 vs. 1.87±2.18 years) (p=0.047). Multivariate analysis identified regional metastasis (OR=2.8; 95% CI=2.6-3.0), distant metastasis (OR=2.1; 95% CI=1.9-2.4), lymph node positivity (OR=1.6; 95% CI=1.4-1.8), Caucasian race (OR=2.0; 95% CI=1.8-2.2), and male sex (OR=1.2; 95% CI=1.1-1.3) were independently associated with increased mortality for KT (p<0.001). ConclusionThe ICD-O-3 has permitted a greater understanding of KT. KT is a rare and lethal biliary malignancy that presents most often in Caucasian men in their seventh decade of life with moderately differentiated histology. Surgical resection does not provide any survival advantage compared to similarly treated biliary CCs. In addition, the combination of surgery and radiation appeared to provide no added survival benefits compared to other treatment modalities for KT.

Role of sex in liver tumor occurrence and clinical outcomes: A comprehensive review

“…[120,121,124,125,[128][129][130][131][132][133][134][135][136] The mortality rate was higher among males than among females in nine of these studies. [120,121,129,[131][132][133][134][135][136] Gender had no impact on mortality in three studies. [124,128,130] Al Mahjoub et al [125] analyzed survival according to the eCCA subtype and found that it was significantly better among males than among females with perihilar CCA and did not differ between males and females with distal CCA.…”

“…The influence of gender on the mortality of patients with eCCA has been examined in 13 studies 120,121,124,125,128–136 . The mortality rate was higher among males than among females in nine of these studies 120,121,129,131–136 .…”

“…The influence of gender on the mortality of patients with eCCA has been examined in 13 studies 120,121,124,125,128–136 . The mortality rate was higher among males than among females in nine of these studies 120,121,129,131–136 . Gender had no impact on mortality in three studies 124,128,130 .…”

Intraductal photodynamic therapy and its combination with intra-arterial chemoinfusion in the treatment of inoperable patients with Klatskin tumor

“…По данным многоцентрового исследования 1144 пациентов с внепеченочной холангиокарциномой J. Khan с соавт. [9] отметили, что специфическая терапия не предлагалась подавляющему большинству (n=835, 73%; р <0,001). В остальных случаях проводили лучевую терапию (n=148, 52,7%; р <0,001), радикальную операцию (n=79, 28,1%; р ≤0,001) и комбинацию облучения с хирургическим методом (n=54, 19,2%; р <0,001).…”

Changing Epidemiological Trends of Hepatobiliary Carcinomas in Austria 2010–2018

“…Adaptions of the International Classification of Diseases for Oncology (ICD-0) over the last decades, resulted in a significant bias concerning incidences of cholangiocarcinomas. In the first ICD-0 version there was no specific coding for Klatskin tumors (perihilar cholangiocarcinoma), in the second version a unique code was given and Klatskin tumors were classified as intrahepatic tumors, leading to an overestimation of iCC and underestimation of eCC [40]. In the ICD-0-3 version, it was cross-referenced to both iCC and eCC.…”