ONCOLOGY LETTERS 11: 3669-3675, 2016
Hepatic epithelioid angiomyolipoma is a rare and potentially severe but treatable tumor: A report of three cases and review of the literature WENTAO LIU1, ZIHUI MENG1, HONGYU LIU1, WEI LI1, QIONG WU2, XUEWEN ZHANG1 and CHANGYONG E1 Departments of 1Hepatobiliary and Pancreatic Surgery and 2Pathology, China‑Japan Union Hospital of Jilin University, Changchun, Jilin 130033, P.R. China Received March 29, 2015; Accepted March 1, 2016 DOI: 10.3892/ol.2016.4443 Abstract. Hepatic epithelioid angiomyolipoma (EAML) is a rare type of hepatic tumor. Due to a lack of adequate understanding about this tumor, hepatic EAML is often misdiagnosed as other diseases with similar clinical characteristics such as hepatic cancer. In the present study, 3 cases of hepatic EAML are reported, and the main clinicopathological features of this disease are presented, based on a literature search that included articles published in English between February 2000 and September 2014. A total of 24 hepatic EAML cases were considered, of which, 17 were females and 4 presented multiple liver lesions. Among the patients with single lesions, 2 underwent surgery and relapsed after 5 months and 9 years, respectively. Immunohistochemical staining was positive for human melanoma black‑45 in the present 3 cases. The aim of the present study was to focus the attention of clinicians on this type of hepatic tumor in order to improve its diagnosis and treatment. Introduction Epithelioid angiomyolipoma (EAML) is a rare mesenchymal neoplasia (1). At present, EAML is considered a member of the perivascular epithelioid cell (PEC) tumors known as PEComas (2), which are a type of epithelioid tumors adjacent to vessels and different from hamartomas (3). EAML is generally considered benign and the majority of patients with EAML usually have a good prognosis. However, EAML possesses malignant potential, which may lead to a poor
Correspondence to: Dr Changyong E or Professor Xuewen Zhang,
Department of Hepatobiliary and Pancreatic Surgery, China‑Japan Union Hospital of Jilin University, 126 Xiantai Street, Changchun, Jilin 130033, P.R. China E‑mail:
[email protected] E‑mail:
[email protected]
Key words: epithelioid angiomyolipoma, liver, hepatic cancer
prognosis (4,5). The treatment for patients with single lesion hepatic EAML is surgical resection (1,6-14). Multiple lesion hepatic EAML is usually metastatic, which indicates a poor prognosis of the patients. For these patients, no good treatments can be conducted. Therefore, early diagnosis of EAML is very important (15-17). The majority of EAMLs originate in the kidneys, and primary hepatic EAML appears to be much less common than renal EAML (1). In the present study, 3 cases of hepatic EAML are presented, and a review of the relevant English literature is conducted. Case report Clinical data and literature review. Clinical data of the 3 EAML cases described in the present study were obtained from the records of the China‑Japan Union Hospital of Jilin University (Changchun, China). The current study was approved by the ethics committee of the China‑Japan Union Hospital of Jilin University. For the literature review, different keyword combinations, i ncludi ng ‘l iver a nd EA M L’, ‘l iver a nd epithelioid angiomyolipoma’, ‘hepatic monotypic epithelioid angiomyolipoma’ and ‘atypical angiomyolipoma’, were used for searching studies on hepatic EAML published in PubMed (www.ncbi.nlm.nih.gov/pubmed), MEDLINE (www.proquest.com/products‑services/medline_ft.html) and Google Scholar (http://scholar.google.com). Articles were selected when full‑text versions were available and contained adequate patient information for comparison. Literature reviews and duplicate reports were excluded. Table I lists the collected information, including author names and year of publication, as well as patient's age, gender, medical history, presence of single or multiple tumors, tumor site, tumor size, symptoms, treatment, results of immunohistochemical staining and follow‑up (6-18). A total of 17 publications met the selection criteria, which corresponded to 24 patients, including the 3 present cases. Demographic and clinical data of the 24 patients are presented in Table I. The mean age of the patients was 47±15 years (range, 23‑80 years). Of the 24 patients, 17 were females, and 4 exhibited multiple hepatic EAML, all patients had a history of renal EAML, 3 of which
Medical history
Tumor Tumor size, number Tumor site cm Symptoms
Treatment
IHC
Follow‑up
Refs.
Dalle et al, 2000 1 70 F Breast Single Right ф15.0 Fever, abdominal Partial HMB‑45+, NKI/C3+, Recurrence (6) cancer lobe pain, dyspnoea hepatectomy CK‑, CEA‑, VIM‑ after 5 months Yamasaki et al, 2 30 F Nil Single Right ф3.0 Nil Partial HMB‑45+, S‑100+, 12 months aw (1) 2000 lobe hepatectomy VIM+‑desmin+‑, SMA+‑, EMA‑,CK‑ Mai et al, 2001 3 51 F Renal Multiple Whole ф5.0 Lumbar pain, N/A HMB‑45+, N/A (15) EAML liver (max) weight loss, SMA+, PAS+, low‑grade fever CK‑, AFP‑, AE1/AE3‑, Hino et al, 2002 4 34 M TSC, Multiple Whole N/A Nil Partial HMB‑45+, SMA+, N/A (18) renal liver hepatectomy VIM+, S‑100‑, EAML Ki‑67 1.6% Tryggvason et al, 5 42 F Nil Single Left ф7.0 Abdominal pain, Partial HMB‑45+, melan A+, N/A (7) 2004 lobe change in bowel hepatectomy CEA‑, EMA‑, CD117‑, habits, weight loss SMA‑, S‑100‑, AE1/AE3‑ Parfitt et al, 2006 6 60 F Nil Single Right 14.0 Abdominal pain Right hepatic HMB‑45+, melan A+, Recurrence (8) lobe x11.0 lobectomy SMA+, S100‑, after 9 years AE1/AE3‑, VIM‑, MSA‑, CD31‑, CD34‑ Alatassi and 7 23 F Bilateral Multiple Whole ф4.0 Abdominal pain N/A HMB‑45+, SMA+, N/A (16) Sahoo, 2009 renal liver ‑11.0 CK‑, S‑100‑ AML, TSC Xie et al, 8 32 F Bilateral renal Multiple Whole ф4.0 Progressive N/A HMB‑45+, melan A+, N/A (17) 2012 AMLs, TSC, liver (max) dyspnea, SMA+, MSA+, seizures, cardiac cough, fever CD34+, desmin‑, rhabdomyomas, EMA‑, TTF1‑ cutaneous angiofibromas, multiple giant cell astrocytomas
Author, Age, year Case years Gender
Table I. Demographic and clinical data of hepatic EAML reports.
3670 LIU et al: A REPORT OF THREE CASES OF HEPATIC EPITHELIOID ANGIOMYOLIPOMA
Tumor size, cm Symptoms
Treatment IHC
Follow‑up
Refs.
Occhionorelli 9 25 F Nil Single Left ф9.0 Abdominal Left‑liver HMB‑45+, melan A+, N/A (9) et al, 2013 lobe pain, lobectomy S‑100‑, actin‑, CK‑, hypotension CK7‑, desmin‑, Ki‑67 2% Zhou et al, 10 34 F Nil Single Left 30.0x25.0 Abdominal Left‑liver HMB‑45+, melan A+, 71 months aw (10) 2014 lobe x15.0 discomfort lobectomy S‑100+,CD10‑, CD34‑, CD117‑, CK‑, AE1/AE3‑, EMA‑, AFP‑, Ki‑67
