Hepatic Angiomyolipoma (HAML) is a rare mesenchymal liver tumour assumed to be predominantly benign, although incidental cases with malignant behaviour such as invasive growth, recurrence after resection and metastases have been reported. The aim of this systematic review was to assess the biological behaviour, estimate the risk of HAML related mortality and recommend on a justifiable management strategy. We performed a systematic literature search in Embase, Medline, Web-of-Science, Scopus, Pubmed Publisher, Cochrane and Google Scholar. We included all articles published from inception until March 2016 which reported on follow-up of various treatment strategies. We included 18 articles reporting on 292 patients. Male:female ratio was estimated at 1:3 with gender not reported in 31 cases. Of 292 patients 247 were treated with surgery, including one liver transplant, seven with chemotherapy or Sirolimus, three with embolization, and 35 conservatively. Recurrence after resection was described in 6/247 (2.4%) with pathologically proven HAML resulting in metastases and death in 2/247 (mortality rate 0.8%). Progression was described in 6/35 patients treated conservatively (21.4%). Two of 12 patients with malignant behaviour of HAML had an epithelioid-type HAML, of the remaining 10 histological subtype was undefined. With a risk estimate of 0.8% in surgically treated patients HAML related mortality is very low. Biopsy is indicated when imaging is inconclusive. In case of certain HAML diagnosis on imaging conservative management with annual imaging is justified. Resection should be considered in case of symptoms, inconclusive biopsy or growth in follow-up.

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Keywords Hepatic angiomyolipoma, Malignant, Management, Review
Persistent URL dx.doi.org/10.1111/liv.13381, hdl.handle.net/1765/98356
Journal Liver International
Klompenhouwer, A.J. (Anne J.), Verver, D, Janki, S, Bramer, W.M, Doukas, M, Dwarkasing, R.S, … IJzermans, J.N.M. (2017). Management of hepatic angiomyolipoma: A systematic review. Liver International. doi:10.1111/liv.13381