Copyright ©The Author(s) 2021.
World J Gastroenterol. May 21, 2021; 27(19): 2299-2311
Published online May 21, 2021. doi: 10.3748/wjg.v27.i19.2299
Table 1 Cases of spontaneous rupture of hepatic angiomyolipoma
Age, yr
Abdominal radiological findings
Huber et al[25] 1996F22Hemorrhagic shock with clinical symptoms of acute abdomenCT scan: multiple tumors of the liver (the largest in segment III measured 8 cm) and both kidneys and a splenic lesion with a diameter of 4 cmSurgical resection of segments II and IIIPostoperative course was uneventful. Discharge from hospital 12 d later
Guidi et al[26] 1997M74Sudden onset of upper-quadrant painCT scan: liver tumor of 10 cm × 8 cm in the segments I and V and another small mass of 4 cm × 3 cm in segment IV. Fluid was present in the upper abdominal compartmentsSurgical resection of the hemorrhagic hepatic massPostoperative course was uneventful. Discharge from hospital 8 d later
Tsui et al[27] 1999F41Acute rupture of a subcapsular tumor9 cmSurgical resectionPatient in healthy condition 4 yr after surgery
Zhou et al[28] 2008NDNDHemorrhagic shockUltrasonography showed a 5-cm "cavernous hemangioma" in the right hepatic lobeEmergency laparotomy for hemostasisNo tumor recurrence or metastasis was found during follow-up of 2-3 yr
Ding et al[8] 2011F56NDA rupture of the tumor measuring 6 cm × 6 cm in segment VI was confirmed by emergent laparotomyLiver suture followed by segmentectomyNo serious morbidity in the postoperative course
Occhionorelli et al[29] 2013F25Sudden onset of abdominal upper-quadrant pain and hypotension, after two recent syncopal episodesCT scan showed a hepatic tumor in the left lobe (8.6 cm × 7.2 cm) with suspected peritoneal blood leakageHemorrhage initially managed by manual compression, followed by deep and pro-coagulant tissue adhesives. After 48 hours, the patient underwent left-liver lobectomyPostoperative course was uneventful. Discharge from hospital 9 d later
Aoki et al[30] 2014F70Sudden onset of back pain on the right sideCT scan: hepatic tumor in segment VII measuring 7 cm in diameter accompanied by subcapsular hematoma with extravasationTranscatheter arterial embolization. Right hepatic lobectomy was carried out 39 d laterFive days after surgery, she had thrombi in the left popliteal vein and the left pulmonary artery. Insertion of an IVC filter which was removed due to sepsis. She was discharged 24 d after surgery. There was no recurrence 42 mo following surgery
Tajima et al[31] 2014M38Upper abdominal painCT scan showed a tumor measuring 10.5 cm × 9.5 cm × 7 cm in the posterior segment of the right hepatic lobe that had ruptured into the space between the liver and the diaphragmTranscatheter arterial embolization was performed. The patient developed fever and the hematoma surrounding the liver was drained. No infection was confirmed but right lobectomy was performedND
Kai et al[32] 2015F77Sudden abdominal pain and transient loss of consciousnessCT scan: hemoperitoneum with subcapsular hematoma at the left lobe and a hepatic nodule measuring 2.3 cm in diameter in segment IIConservative initial treatment with periodic imaging studies. Transcatheter arterial chemoembolization was performed because a diagnosis of HCC was suggested. Surgical resection (laparoscopic left lateral segmentectomy) was performed 4 mo laterPostoperative course was uneventful. Discharge from hospital 7 d later No signs of recurrence at 3.5 yr after surgery
Kim et al[33] 2017M31Sudden onset severe abdominal pain in the right upper quadrant areaCT scan: Mass of approximately 12 cm in the right hepatic lobe with hemorrhage along the perihepatic spaceEmergent angiography with embolization.Hepatic resection was performed 15 d laterPostoperative course was uneventful
Table 2 Classification of perivascular epithelioid cell tumors according to their malignant potential[1,27]
BenignNo worrisome features: (1) Tumor size < 5 cm; (2) No infiltration; (3) Non-high nuclear grade and cellularity; (4) Mitotic activity ≤ 1/50 HPF; (5) No necrosis; and (6) No vascular invasion
Uncertain malignant potentialTumor with: (1) Pleomorphism/multinucleated giant cells only; or (2) Size > 5 cm only
Aggressive behaviorTwo or more worrisome features: (1) Size > 5 cm; (2) Peripheral infiltration; (3) High nuclear grade and cellularity; (4) Mitotic activity > 1/50 HPF; (5) Ischemic tumor necrosis for large tumor; and (6) Vascular invasion
According to the WHO classification of tumors[1]As with GISTs, the main predictors of a risk of metastatic behavior are marked nuclear atypia, diffuse pleomorphism and mitotic activity of more than 1 mitosis per 1 mm²
Table 3 Reported cases of hepatic angiomyolipoma with aggressive behavior
Age, yr
Duration of follow-up
Croquet et al[9] 2000F1619 cm × 12 cm × 8 cmEpithelioidSR6 yrRecurrence in the liver, associated with renal angiomyolipoma
Dalle et al[10] 2000F7015 cmEpithelioidSR5 moRecurrence in the liver with a lesion measuring 15 cm and presence of multiple metastases in the liver
Flemming et al[11] 2000F512 nodules: 0.5 cm and 15 cmEpithelioidSR3 yrRecurrence in the right hepatic lobe and presence of multiple metastases
McKinney et al[12] 2005F1411 cm × 7 cm × 8 cmNSSR, interferon α1 yrRecurrence with a hepatic lesion measuring 9 cm × 6 cm × 14 cm, appearance of lymph nodes and hepatic metastases. Death after disease progression
Parfitt et al[13] 2007F6014 cm × 11 cmEpithelioidSR9 yrRecurrence in the liver and appearance of metastases in the trapezius muscle, the left lung and the tail of the pancreas
Yang et al[14] 2007F3713 cm × 9 cm × 9 cmClassicSR14 moRecurrence in the right hepatic lobe 6 months after SR, appearance of pulmonary metastases 11 mo after SR and death occurred at 14 mo
Deng et al[15] 2008M3018 cm × 14 cmClassicSR, Chemotherapy3 yr and 4 moRecurrence with a hepatic lesion measuring 11 cm and metastases in pancreatic tail and portal vein thrombosis 3 yr after SR. Chemotherapy was initiated but 4 mo later pulmonary metastases appeared. Death occurred after disease progression
Nguyen et al[16] 2008F4311 cm × 7.5 cm × 7.5 cmClassicSR6 moRecurrence in the liver 6 mo after SR, together with metastases in the peritoneum, omentum, stomach and spleen. Death after disease progression
Xu et al[17] 2009F332 nodules: 1 cm and 6 cmEpithelioidSR1 yrRecurrence in the left hepatic lobe
Zeng et al[18] 2010NSNS6 cmNSSR9 yrRecurrence in the right hepatic lobe with a lesion measuring 6 cm
Butte et al[19] 2011F;M54; 41NS; 9 cmNS; EpithelioidSR; SR53 mo; 41 moRecurrence in the liver 53 mo after SR; Occurrence of pulmonary and retroperitoneal metastases 41 mo after SR
Hu et al[20] 2011FNSNSNSSR14 moAppearance of local and distant metastases 6 mo after SR. Death occurred 14 mo after SR
Ding et al[8] 2011F318 cm × 8 cmNSSR7 yrRecurrence in the right hepatic lobe 6 yr after SR and death occurred one year later
Wang et al[21] 2015F377 cm × 9 cmClassicSR3 yrRecurrence of two hepatic nodules in the right lobe (13 cm × 12 cm and 2.3 cm × 1.8 cm) 3 yr after SR. Arterial chemoembolization was performed, followed by liver transplantation
Fukuda et al[22] 2016M586.3 cmEpithelioidSR9 yrMetastases occurred in the right lung 7 yr after SR and were treated by pneumonectomy. No recurrence was observed after 2 yr of follow-up
Marcuzzi et al[23] 2018F473.8 cm × 4.6 cm × 4.7 cm+ 2 hepatic lesions measuring 6 mm and 5 mmEpithelioidSR8 yr and 8 moCT scan was performed 6 yr and 4 mo after the initial presentation: the hepatic lesion had grown in size to an estimated 10.9 cm × 9.7 cm × 11.2 cm and the adjacent lesions had grown to 1.9 cm and 2.4 cm with a new lesion on the kidney of 4.6 cm × 5.1 cm. 16 mo later, MRI showed an increase in size of the hepatic lesion (12 cm × 11 cm), and kidney lesion (6.2 cm × 5.6 cm). SR performed 2 mo later. 6 mo after SR, recurrence in the resection line and in the hepatic segment II
Yan et al[24] 2018NSNS15 cmEpithelioidSR9 yrRecurrence in the liver 9 yr after SR with invasion of the inferior vena cava and diaphragm, and appearance of pulmonary metastases