title: Primary hepatic epithelioid angiomyolipoma: A malignant potential tumor which should be recognized.
abstract: OBJECTIVE
  To improve the clinical diagnosis and recognition of hepatic epithelioid angiomyolipoma (HEAML).
  
  
  METHODS
  Four cases of primary HEAML were confirmed based on the pathology archive system in our hospital from January 2009 to November 2015. The general state, clinical symptoms, imaging manifestations, histological results and immunohistochemistry of these patients were retrospectively reviewed and analyzed. Studies of HEAML published in the last 15 years were collected from PubMed and MEDLINE to summarize the clinical symptoms, imaging characteristics, pathological features and management of HEAML.
  
  
  RESULTS
  Four cases of primary HEAML were retrieved from our archives. These included three female patients and one male patient, with a mean age of 41.8 ± 11.5 years (ranging from 31 to 56 years). The mean tumor size was 7.3 ± 5.5 cm (ranging from 3.0 to 15 cm). In the contrast-enhanced imaging, the tumor was obviously enhanced in the arterial phase, but enhanced continuously or exhibited a slow-density masse during the venous and delayed phases. Histologically, the tumors mainly consisted of epithelioid cells that comprised approximately 95% of the total neoplastic mass. Although no metastases occurred in our patients, pathological studies revealed necrosis, mitotic figures and liver invasion in two patients, which indicates aggressive behavior. Immunohistochemical staining revealed that human melanoma black 45 (HMB-45) and Melan-A were positive in 4 cases. We only identified 81 cases with primary HEAML, including our present patients, from 26 articles available from PubMed and MEDLINE. The majority of the papers were published as case reports. Only 5 (5/75, 6%) cases were associated with tuberous sclerosis complex (TSC). More than half (35/66) were discovered incidentally upon physical examination. Approximately 65% (22/34) of the patients were misdiagnosed with HCC or other tumors before surgery. Approximately 10% (8/81) of the patients with HEAML had recurrence or metastasis after surgery, which was a very high and alarming rate.
  
  
  CONCLUSIONS
  HEAML is a very rare primary hepatic tumor that is often misdiagnosed before surgery. Patients should be followed closely after surgery because of its malignant potential.
authors.affiliation: Jie Liu, Cheng-Wu Zhang, De-Fei Hong, Ran Tao, Min-Jie Shang, Yu-Hua Zhang, Department of Hepatopancreatobiliary Surgery and Minimally Invasive Surgery, Zhejiang Provincial People's Hospital, Hangzhou 310014, Zhejiang Province, China.
authors.forename: Jie
authors.initials: J
authors.lastname: Liu
authors.affiliation: Jie Liu, Cheng-Wu Zhang, De-Fei Hong, Ran Tao, Min-Jie Shang, Yu-Hua Zhang, Department of Hepatopancreatobiliary Surgery and Minimally Invasive Surgery, Zhejiang Provincial People's Hospital, Hangzhou 310014, Zhejiang Province, China.
authors.forename: Cheng-Wu
authors.initials: CW
authors.lastname: Zhang
authors.affiliation: Jie Liu, Cheng-Wu Zhang, De-Fei Hong, Ran Tao, Min-Jie Shang, Yu-Hua Zhang, Department of Hepatopancreatobiliary Surgery and Minimally Invasive Surgery, Zhejiang Provincial People's Hospital, Hangzhou 310014, Zhejiang Province, China.
authors.forename: De-Fei
authors.initials: DF
authors.lastname: Hong
authors.affiliation: Jie Liu, Cheng-Wu Zhang, De-Fei Hong, Ran Tao, Min-Jie Shang, Yu-Hua Zhang, Department of Hepatopancreatobiliary Surgery and Minimally Invasive Surgery, Zhejiang Provincial People's Hospital, Hangzhou 310014, Zhejiang Province, China.
authors.forename: Ran
authors.initials: R
authors.lastname: Tao
authors.affiliation: Jie Liu, Cheng-Wu Zhang, De-Fei Hong, Ran Tao, Min-Jie Shang, Yu-Hua Zhang, Department of Hepatopancreatobiliary Surgery and Minimally Invasive Surgery, Zhejiang Provincial People's Hospital, Hangzhou 310014, Zhejiang Province, China.
authors.forename: Yuan
authors.initials: Y
authors.lastname: Chen
authors.affiliation: Jie Liu, Cheng-Wu Zhang, De-Fei Hong, Ran Tao, Min-Jie Shang, Yu-Hua Zhang, Department of Hepatopancreatobiliary Surgery and Minimally Invasive Surgery, Zhejiang Provincial People's Hospital, Hangzhou 310014, Zhejiang Province, China.
authors.forename: Min-Jie
authors.initials: MJ
authors.lastname: Shang
authors.affiliation: Jie Liu, Cheng-Wu Zhang, De-Fei Hong, Ran Tao, Min-Jie Shang, Yu-Hua Zhang, Department of Hepatopancreatobiliary Surgery and Minimally Invasive Surgery, Zhejiang Provincial People's Hospital, Hangzhou 310014, Zhejiang Province, China.
authors.forename: Yu-Hua
authors.initials: YH
authors.lastname: Zhang
mesh_terms: D000328:Adult; D000368:Aged; D018206:Angiolipoma / Q000737:chemistry / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D014408:Biomarkers, Tumor / Q000032:analysis; D001706:Biopsy; D003951:Diagnostic Errors; D018450:Disease Progression; D015622:Epithelioid Cells / Q000737:chemistry / Q000473:pathology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008113:Liver Neoplasms / Q000737:chemistry / Q000175:diagnosis* / Q000473:pathology / Q000601:surgery; D058965:MART-1 Antigen / Q000032:analysis; D008279:Magnetic Resonance Imaging; D008297:Male; D058950:Melanoma-Specific Antigens / Q000032:analysis; D008875:Middle Aged; D009362:Neoplasm Metastasis; D009364:Neoplasm Recurrence, Local; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D047368:Tumor Burden; D058951:gp100 Melanoma Antigen
chemical_list: D014408:Biomarkers, Tumor; D058965:MART-1 Antigen; C548667:MLANA protein, human; D058950:Melanoma-Specific Antigens; C548637:PMEL protein, human; D058951:gp100 Melanoma Antigen
keywords: Epithelioid angiomyolipoma; Human melanoma black 45; Imaging; Immunohistochemical staining; Liver