부산대학교 도서관

Anastasia Murtha-Lemekhova,1,2 Juri Fuchs,1,2 Erik Schulz,1,2 Anthe Suzan Sterkenburg,1 Philipp Mayer,2,3 Jan Pfeiffenberger,2,4 Katrin Hoffmann1,2 1Department of General, Visceral, and Transplantation Surgery, Heidelberg University Hospital, Heidelberg, Germany; 2RELIVE Initiative, Heidelberg University Hospital, Heidelberg, Germany; 3Department of Diagnostic and Interventional Radiology, Heidelberg University Hospital, Heidelberg, Germany; 4Department of Gastroenterology and Hepatology, Heidelberg University Hospital, Heidelberg, GermanyCorrespondence: Katrin HoffmannDepartment of General, Visceral, and Transplantation Surgery, Heidelberg University Hospital, Im Neuenheimer Feld 420, Heidelberg, 69120, GermanyTel +49 6221 56 6110Fax +49 6221 56 5450Email katrin.hoffmann@med.uni-heidelberg.deBackground: Aberrant subtypes of hepatocellular carcinoma (HCC) account for 20– 30% of all HCCs and habitually present a challenge in diagnosis and treatment. Scirrhous hepatocellular carcinoma (s-HCC) is often misdiagnosed as cholangiocarcinoma, fibrolamellar hepatocellular carcinoma, or metastasis.Methods: Electronic databases (PubMed, Web of Knowledge, Google Scholar, Cochrane Library, and WHO International Clinical Trials Registry Platform) were searched for publications on scirrhous hepatocellular carcinoma without date or language restrictions. Quality assessment was performed using a tool proposed by Murad et al for case reports and series. For observational studies, MINORS quality assessment tool was used. This study was registered at PROSPERO (CRD42020212323).Results: S-HCC arises in patients with chronic hepatitis (hepatitis B in 60% and hepatitis C in 21%). S-HCC primarily affects men with a mean age of 55.8 years. Serum AFP is elevated above 20IU/mL in 66.7% of the patients. On ultrasound, s-HCC presents as hypoechoic or mosaic pattern lesions (47.6% each) and causes a retraction of the liver surface (70%) when near the capsule. Delayed enhancement of the tumor is evident in 87.0%. On MRI, 65.0% of s-HCCs show a target appearance. Histopathologic pattern is mostly irregular (97.6%). Lesions show a bulging appearance (100%), septae (85.6%) and a central scar (63.5%), and usually lack central necrosis (75%). Immunohistochemistry shows HepPar 1 positivity in 64.6%, CK7 in 40.7%, and EMA in 41.9%. The 5-year overall survival rate estimates 45.2% and 45.5% of the patients experience a recurrence after hepatectomy.Conclusion: S-HCC is a rare subtype of HCC primarily arising in hepatitis- or cirrhosis-afflicted livers and incorporates atypical radiological and histopathological HCC features. Despite lower recurrence rates, overall survival of patients with s-HCC is poorer than generally for HCC, underlining the need for individualized treatment. Patients with atypical lesions of the liver should be referred to tertiary hospitals for interdisciplinary assessment and treatment.Keywords: scirrhous HCC, s-HCC, rare liver tumors, RELIVE initiative