ABSTRACT
Background: The heterogeneity, high rate of mortality and lack of comprehensive diagnostic methods have categorized primary sarcomas of the thorax as a malignancy with dismal outcomes and unknown etiology. Given the fundamental role of epidemiological analysis in establishing management strategies, we designed a study with focus on the epidemiological characteristics of primary thoracic sarcomas in Iran. Methods: This national population-based cancer study was conducted on patients with histologically confirmed sarcoma of the thorax referred to the Iranian National Cancer Registry between 2009 and 2014. The incidence was calculated as number of cases per 100,000 person-years and was age-adjusted by the direct method using the weight of the 1960 world standard population. Results: Over a 6-year period, 1477 cases with pathologically confirmed thoracic sarcomas were registered in Iran, of which 896 were male and 581 were female. Khuzestan Province had the highest incidence of thoracic sarcomas as compared to other provinces. Malignant mesothelioma was the most common histological subtype (20.85%). Moreover, the age-standardized incidence rate (ASR) of the disease was 1.94 per 100,000 which was more common in males than females with the highest incidence rate in men aged more than 65 years. Conclusion: Our study provided valuable epidemiologic data on characteristics of thoracic sarcomas. This data can be used for strategizing preventive measures.
ABSTRACT
BACKGROUND: Autoimmune hepatitis-primary sclerosing cholangitis overlap syndrome is a form of autoimmune hepatitis with cholestatic features and is characterized by negative anti-mitochondrial antibody and cholangiographic changes on magnetic resonance cholangiopancreatography or endoscopic retrograde cholangiopancreatography. Peripheral blood hypereosinophilia in conjunction with autoimmune hepatitis-primary sclerosing cholangitis overlap syndrome has not been reported yet. Here we present a case of autoimmune hepatitis-primary sclerosing cholangitis overlap syndrome with hypereosinophilia. CASE PRESENTATION: A 33-year-old Iranian man with the fatigue, jaundice, elevated liver enzymes and alkaline phosphatase, and hypereosinophilia was referred to our hospital. Viral and autoimmune hepatitis were excluded, and secondary workups for hypereosinophilia were all negative. Magnetic resonance cholangiopancreatography showed beaded appearance of intra- and extrahepatic biliary tree, and liver biopsy revealed interface hepatitis. Therefore, the diagnosis of autoimmune hepatitis-primary sclerosing cholangitis overlap syndrome was made, and prednisolone, azathioprine, and ursodeoxycholic acid was initiated. His jaundice and peripheral blood eosinophilia resolved after 2 weeks, and he became completely asymptomatic. CONCLUSION: Eosinophils might contribute to the clinical presentation and disease complications.
