BACKGROUND: Liver angiomyolipomas (AML) are mesenchymal neoplasms with an uncertain clinical behavior. The spectrum of presentation, imaging and histologic features, and outcomes were analyzed in all patients treated at Memorial Sloan-Kettering Cancer Center. METHODS: Demographics, disease, pathologic, treatment, and outcome-related variables for consecutive patients were reviewed retrospectively. All imaging studies obtained at presentation were reexamined, categorized, and compared using Fisher and Wilcoxon tests. RESULTS: Between 1989 and 2010, 238 patients with AML were seen and 22 (9.3%) had liver involvement (exclusive = 17; combined with kidney = 5). The median age was 53 years; 18 were females, and 15 had symptoms. AML was not suspected at initial presentation in any patient. Fat-containing neoplasms on imaging were larger (P = .03), with more heterogeneous enhancement compared with fat-poor neoplasms (P = .001), but none of the imaging/histologic features correlated with outcome. Thirteen patients underwent resection (R0 = 9), 4 were observed, 2 received chemotherapy, 2 embolization, and 1 embolization plus intra-arterial chemotherapy. Two patients treated with R0-resection recurred and 2 treated with chemotherapy progressed, but no patient died of AML-related causes. At a median follow-up of 36 months, 7 patients were free of disease, 13 were alive with disease, 1 died of an unrelated cause, and another was lost to follow-up. CONCLUSION: AMLs are rare neoplasms with an indolent course in most patients. Subtypes based on fat content are recognized, but clinical behavior does not seem to be different. Recurrence after resection is not associated with disease-related mortality. Resection may be unnecessary in selected asymptomatic patients if the diagnosis can be established definitively.
BACKGROUND:Liver angiomyolipomas (AML) are mesenchymal neoplasms with an uncertain clinical behavior. The spectrum of presentation, imaging and histologic features, and outcomes were analyzed in all patients treated at Memorial Sloan-Kettering Cancer Center. METHODS: Demographics, disease, pathologic, treatment, and outcome-related variables for consecutive patients were reviewed retrospectively. All imaging studies obtained at presentation were reexamined, categorized, and compared using Fisher and Wilcoxon tests. RESULTS: Between 1989 and 2010, 238 patients with AML were seen and 22 (9.3%) had liver involvement (exclusive = 17; combined with kidney = 5). The median age was 53 years; 18 were females, and 15 had symptoms. AML was not suspected at initial presentation in any patient. Fat-containing neoplasms on imaging were larger (P = .03), with more heterogeneous enhancement compared with fat-poor neoplasms (P = .001), but none of the imaging/histologic features correlated with outcome. Thirteen patients underwent resection (R0 = 9), 4 were observed, 2 received chemotherapy, 2 embolization, and 1 embolization plus intra-arterial chemotherapy. Two patients treated with R0-resection recurred and 2 treated with chemotherapy progressed, but no patient died of AML-related causes. At a median follow-up of 36 months, 7 patients were free of disease, 13 were alive with disease, 1 died of an unrelated cause, and another was lost to follow-up. CONCLUSION: AMLs are rare neoplasms with an indolent course in most patients. Subtypes based on fat content are recognized, but clinical behavior does not seem to be different. Recurrence after resection is not associated with disease-related mortality. Resection may be unnecessary in selected asymptomatic patients if the diagnosis can be established definitively.
Authors: Paul Calame; Gaëlle Tyrode; Delphine Weil Verhoeven; Sophie Félix; Anne Julia Klompenhouwer; Vincent Di Martino; Eric Delabrousse; Thierry Thévenot Journal: World J Gastroenterol Date: 2021-05-21 Impact factor: 5.742