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Table 1 Review of 18 cases of malignant hepatic PEComa in adults reported in the literature

From: Malignant perivascular epithelioid cell neoplasm in the liver: report of a pediatric case

Author Year Sex/age Size (cm) Location CT finding MRI finding Surgical procedure Recurrence site Chemotherapy Prognosis
Dalle et al. [28] 2000 F/70 15.0 Right lobe Hypervascular NP Right trisegmentectomy Liver NP 7 months alive from surgery
Flemming et al. [29] 2000 F/51 10 and 0.5 left lobe NA NA Left lobectomy → 
Right lobectomy
Liver NP 1.5 years alive without disease
Mizuguchi et al. [30] 2004 F/49 NA Right lobe Hypervascular NA Right trisegmentectomy No NP NA
Parfitt et al. [5] 2006 F/60 14.0 Right lobe NA NP Right lobectomy Liver, trapezius, bladder, lung, and pancreas NP NA
Yang et al. [31] 2007 F/37 13 Left lobe NA NA Extended left lobectomy Liver, lung NA Died 14 months after surgery
Deng et al. [32] 2008 F/30 11 Right lobe Hypervascular NP Right lobectomy Liver, pancreas, and lung Adjuvant Died 4 months after recurrence
Nguyen et al. [27] 2008 F/43 11 Left lobe Na NA Left hepatectomy Liver, peritoneum, retroperitoneum, and omentum NP Died 3 months after surgery
Kamimura et al. [33] 2010 F/43 11.0 Left lobe Early enhancement, wash-out in delayed phase Low on T1I,
High on T2I
Left lobectomy No NP Disease free in 3 years
Selvaggi et al. [34] 2011 F/68 9.0 Right lobe Hypodense lesion, multiple lesions around triangular ligament, hemoperitoneum NP Omentectomy, toilette, hemostasis No NP Died 25 days after admission
Ding et al. [35] 2011 F/31 NA Right lobe NA NA Right lobectomy Liver NP Died 1 year after surgery
Kobayashi et al. [36] 2013 F/60 NA Right lobe Hypodense lesion, persistent enhancement Low on T1I
High on T2I
High on DWI
Partial hepatectomy No NP Disease free in 3 years
Bergamo et al. [23] 2014 M/30 18.0 Right lobe NP Hypervascular Partial hepatectomy No Sirolimus alive
Abhirup et al. [4] 2015 F/49 10 Right lobe Heterogeneously enhanced with central necrosis No Extended right hepatectomy Liver NP Died 12 months after recurrence
Wang et al. [20] 2015 F/37 9 Left lobe Early enhancement, wash-out in delayed phase Low on T1I
High on T2I
High on DWI
Hepatectomy → 
Liver transplantation
Liver NP Alive
Hao et al. [18] 2016 F/51 8.0 Right lobe Hypodense lesion, brightly enhance, slightly local hyperattenuating NP Hepatectomy NA NP Alive
Fukuda et al. [37] 2016 M/58 6.3 Right lobe Early enhancement, wash-out in delayed phase NP Anterior segmentectomy → 
Partial pneumonectomy
Lung NP Recurrence free in 2 years from surgery
Liu et al. [38] 2016 M/34 15 Left lobe Early enhancement, wash-out in delayed phase NA Left lobectomy No NP Alive
Liu et al. [38] 2016 M/31 3 Right lobe Early enhancement, wash-out in delayed phase NA Right lobectomy No NP Alive
Our case 2021 M/10 13.1 Central portion Weakly enhanced in the both arterial and portal phase Low on T1I
High on T2I
Relatively high on DWI
Central bisegmentectomy Thoracic vertebrae, lung and pelvis Ifosfamide, doxorubicin/everolimus Alive
  1. Eighteen cases were identified. Female predominance is obvious, and right lobe was more often affected. Surgical procedures most often performed were hepatic lobectomy, followed by segmentectomy, and partial hepatectomy. CT was performed for most patients, but MRI was limited in number. mTOR inhibitor was administered as neoadjuvant therapy to only one patient. F female, M male, NA information not available, NP not performed, T1I T1-weighted imaging, T2I T2-weighted imaging, DWI diffusion-weighted imaging