Klompenhouwer AJ, Dwarkasing RS, Doukas M, Pellegrino S, Vilgrain V, Paradis V, Soubrane O, Beane JD, Geller DA, Nalesnik MA, Tripke V, Lang H, Schmelzle M, Pratschke J, SchÃ¶ning W, Beal E, Sun S, Pawlik TM, de Man RA, Ijzermans JNM. Hepatic angiomyolipoma: an international multicenter analysis on diagnosis, management and outcome. HPB (Oxford). 2019 Oct 13. pii: S1365-182X(19)30716-6. doi: 10.1016/j.hpb.2019.09.004. PubMed PMID: 31619346.
BACKGROUND: Hepatic angiomyolipoma (HAML) may easily be misdiagnosed as a malignancy. The study aim was to assess diagnostic dilemmas, clinical management and outcome of this rare tumor.
METHODS: This retrospective international multicenter study included all patients with pathologically proven HAML diagnosed between 1997 and 2017. Data on patient characteristics, diagnostic work-up, management and follow-up were analyzed.
RESULTS: Thirty-eight patients were included, 32 female. Median age was 56yrs (i.q.r. 43-64) and median HAML-diameter was 57.5 mm (i.q.r. 38.5-95.3). Thirty patients had undergone CT and 27/38 MRI of the liver, diagnostic biopsy was performed in 19/38. Initial diagnosis was incorrect in 15/38 patients, of which 13 were thought to have malignancy. In 84% biopsy resulted in a correct preoperative diagnosis. Twenty-nine patients were managed with surgical resection, 4/38 with surveillance and 3/38 with liver transplantation. Recurrence after resection occurred in two cases. No HAML related deaths or progression to malignancy were documented.
CONCLUSION: HAML diagnosis proved problematic even in hepatobiliary expertise centers. Biopsy is indicated and may provide valuable additional information when HAML diagnosis is considered on cross-sectional imaging, especially when surgical resection imposes a risk of complications. Conservative management with regular imaging follow-up might be justified when biopsy confirms (classic type) HAML.
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