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 | 7Â 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 14Â months after surgery |
Deng et al. [32] | 2008 | F/30 | 11 | Right lobe | Hypervascular | NP | Right lobectomy | Liver, pancreas, and lung | Adjuvant | Died 4Â months after recurrence |
Nguyen et al. [27] | 2008 | F/43 | 11 | Left lobe | Na | NA | Left hepatectomy | Liver, peritoneum, retroperitoneum, and omentum | NP | Died 3Â 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 3Â 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 25Â days after admission |
Ding et al. [35] | 2011 | F/31 | NA | Right lobe | NA | NA | Right lobectomy | Liver | NP | Died 1Â 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 3Â 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 12Â 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 |