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Afzal A, Tahir MJ, Asghar A, Din IU, Naveed MA. Malignant epithelioid angiomyolipoma of the submandibular gland: A case report. Radiol Case Rep 2024; 19:6333-6338. [PMID: 39387025 PMCID: PMC11461934 DOI: 10.1016/j.radcr.2024.09.027] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/11/2024] [Revised: 09/02/2024] [Accepted: 09/04/2024] [Indexed: 10/12/2024] Open
Abstract
An epithelioid angiomyolipoma (a perivascular epithelioid cell tumor) is a rare mesenchymal neoplasm with distinctive cellular morphology and nonspecific imaging appearances. Mostly reported perivascular epithelioid cell tumors (PEComas) are benign; however, rarely, PEComas can be malignant with pulmonary, hepatic, nodal, and osseous metastases. We present a case of a 40-year-old man with malignant right submandibular salivary gland PEComa, metastasized to the bones, lungs, and liver. We are going to discuss the diagnosis and management options of the rare disease of metastatic PEComa of the submandibular salivary gland.
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Affiliation(s)
- Ayesha Afzal
- Department of Radiology, Shaukat Khanum Memorial Cancer Hospital and Research Center, Lahore, Punjab province, Pakistan
| | - Muhammad Junaid Tahir
- Department of Radiology, Shaukat Khanum Memorial Cancer Hospital and Research Center, Lahore, Punjab province, Pakistan
| | - Asma Asghar
- Department of Radiology, Shaukat Khanum Memorial Cancer Hospital and Research Center, Lahore, Punjab province, Pakistan
| | - Islah Ud Din
- Department of Radiology, Shaukat Khanum Memorial Cancer Hospital and Research Center, Lahore, Punjab province, Pakistan
| | - Muhammad Atif Naveed
- Department of Radiology, Shaukat Khanum Memorial Cancer Hospital and Research Center, Lahore, Punjab province, Pakistan
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Kinzel A, McArthur M, Gettle LM, Felker E, Patel M. PEComas: A review of imaging and clinical features. Clin Imaging 2024; 116:110332. [PMID: 39442258 DOI: 10.1016/j.clinimag.2024.110332] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/15/2024] [Revised: 10/08/2024] [Accepted: 10/13/2024] [Indexed: 10/25/2024]
Abstract
Perivascular epithelioid cell tumors (PEComas) are a group of rare mesenchymal tumors, which demonstrate varied imaging appearances and treatment options. These tumors may arise de novo or in the setting of systemic disorders, such as tuberous sclerosis. Some PEComas are benign and easily resected while others may represent systemic or metastatic disease with limited therapeutic options. The purpose of this review is to introduce the topic of perivascular epithelioid cell tumors and the most common tumors within the PEComa family as well as discuss the epidemiology, morphology, radiographic appearance, and treatment options of these rare tumors.
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Affiliation(s)
- Adam Kinzel
- Charlotte Radiology, 8514 McAlpine Park Dr., Suite 100, Charlotte, NC 28211, USA
| | - Mark McArthur
- University of California, Los Angeles, David Geffen School of Medicine at UCLA, Department of Radiological Sciences, 757 Westwood Plaza, Suite 1621, Los Angeles, CA 90095-7532, USA.
| | - Lori Mankowski Gettle
- Richard L. Roudebush VA Medical Center, 1481 W. 10(th) St., Indianapolis, IN 46202, USA
| | - Ely Felker
- University of California, Los Angeles, David Geffen School of Medicine at UCLA, Department of Radiological Sciences, 757 Westwood Plaza, Suite 1621, Los Angeles, CA 90095-7532, USA
| | - Maitraya Patel
- University of California, Los Angeles, David Geffen School of Medicine at UCLA, Department of Radiological Sciences, 757 Westwood Plaza, Suite 1621, Los Angeles, CA 90095-7532, USA
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Chen Q, Wang P, Zhang X, Zhang J. Rare perivascular epithelial cell tumor of the colon 18F-FDG PET/CT imaging: A case report. Medicine (Baltimore) 2023; 102:e33802. [PMID: 37335716 PMCID: PMC10194671 DOI: 10.1097/md.0000000000033802] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/23/2023] [Accepted: 04/27/2023] [Indexed: 06/21/2023] Open
Abstract
RATIONALE Neoplasms with perivascular epithelioid cell differentiation (PEComas) are mesenchymal tumors that rarely occur in the colon. Here, we report the occurrence of a malignant PEcoma in the colon using 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT). PATIENT CONCERNS A 55-year-old woman was admitted to the hospital with abdominal pain for 10 days and a self-induced abdominal mass for 3 days. 18F-FDG PET/CT imaging showed a large hypermetabolic nodule and mass in the right mid-upper abdomen with heterogeneous density and a further increase in metabolism on the delayed scan. DIAGNOSES PEComa of the colon. INTERVENTIONS Tumor resection was performed. OUTCOMES The patient is well after 2 months of treatment, pending further follow-up. LESSONS Malignant perivascular epithelioid cell tumors originating in the colon are extremely rare, and our report suggests that PEComa should be considered as a differential diagnosis for 18F-FDG gastrointestinal malignancies. Additionally, 18F-FDG PET/CT may play a key role in the staging and extent of lesions in intestinal malignancies.
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Affiliation(s)
- Qianqian Chen
- Guangzhou University of Chinese Medicine, Guangzhou, China
- Department of Nuclear Medicine, General Hospital of Southern Theater Command, Guangzhou, China
| | - Peiqi Wang
- Department of Nuclear Medicine, General Hospital of Southern Theater Command, Guangzhou, China
| | - Xiao Zhang
- Department of Nuclear Medicine, General Hospital of Southern Theater Command, Guangzhou, China
| | - Jinhe Zhang
- Department of Nuclear Medicine, General Hospital of Southern Theater Command, Guangzhou, China
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Gulmez AO, Aydin S, Kantarci M. A complementary comment on primary hepatic angiosarcoma: A case report. World J Clin Cases 2023; 11:1814-1822. [PMID: 36969989 PMCID: PMC10037275 DOI: 10.12998/wjcc.v11.i8.1814] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/17/2022] [Revised: 12/01/2022] [Accepted: 01/20/2023] [Indexed: 03/07/2023] Open
Abstract
BACKGROUND This article examines primary hepatic angiosarcoma (PHA) and fat-poor angiomyolipoma (AML), two uncommon vascular cancers. Clinical decisions in these situations are frequently aided by pathology reports and imaging techniques. Uncommon malignant tumors of the vascular endothelium include PHA. Another diagnosis that should not be overlooked when employing contrast-enhanced MR and contrast-enhanced computed tomography (CT) imaging techniques is fat-poor AML, one of the uncommon vascular tumors of the liver. In both conditions, biopsy is the primary means of diagnosis.
CASE SUMMARY In our article, besides the diagnosis of PHA, fat-poor AML, one of the other rare vascular tumors of the liver, is mentioned. In the case, a 50-year-old female patient with VHL Syndrome was admitted to our hospital with nonspecific lesions such as right upper quadrant pain, weight loss, and nausea. Abdominal ultrasonography (US) revealed a hypoechoic heterogeneous lesion with occasional faint contours. In computed tomography, it was observed as a hyperdense nodular lesion in segment 4. Magnetic resonance imaging (MRI) revealed that the lesion did not contain fat. In connection with the known history of VHL Syndrome, we first evaluated the possibility of AML. Thereupon, a histopathological sample was taken and the diagnosis was made as fat-poor AML with 5% fat content.
CONCLUSION In conclusion, PHA in our case report and fat-poor AML in our clinic are two uncommon liver vascular malignancies with comparable incidences. Important imaging techniques like contrast-enhanced US (CEUS), CECT, and CEMRI give us substantial advantages in both cases. However, a biopsy is used to provide the final diagnosis.
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Affiliation(s)
- Ali Osman Gulmez
- Department of Radiology, Erzincan University, Erzincan 24100, Turkey
| | - Sonay Aydin
- Department of Radiology, Erzincan University, Erzincan 24100, Turkey
| | - Mecit Kantarci
- Department of Radiology, Erzincan University, Erzincan 24100, Turkey
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Junhao L, Hongxia Z, Jiajun G, Ahmad I, Shanshan G, Jianke L, Lingli C, Yuan J, Mengsu Z, Mingliang W. Hepatic epithelioid angiomyolipoma: magnetic resonance imaging characteristics. Abdom Radiol (NY) 2023; 48:913-924. [PMID: 36732407 DOI: 10.1007/s00261-023-03818-z] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/08/2022] [Revised: 01/13/2023] [Accepted: 01/16/2023] [Indexed: 02/04/2023]
Abstract
PURPOSE The aim of the study was to analyze MR imaging features of hepatic epithelioid angiomyolipoma (HEAML). METHODS This study included 113 patients with 122 pathologically confirmed HEAML who underwent enhanced MRI scanning before partial hepatectomy. MR images were retrospectively reviewed and correlated with pathological findings. RESULTS The mean age of 113 patients was 48.12 ± 11.77 years old, and the male to female ratio was 1:3.35 with 87 females (76.99%). 107 (94.69%) patients presented as single tumor, and 96 patients (84.96%) were asymptomatic. 122 HEAML lesions were diagnosed pathologically in 113 patients, with the average of 4.47 ± 3.26 cm. 109 lesions (89.34%) and 108 lesions (88.52%) showed regular and well-defined boundary. On T1WI, 121 lesions (99.18%) mainly presented hypointensity. On T2WI, 118 lesions (96.72%) and 109 lesions (89.34%) mainly showed hyperintensity and heterogeneous signals. Most of the lesions (97.46%) showed hyperintensity on DWI. 118 lesions (96.72%) manifested as severe hyperenhancement and 106 lesions (86.89%) showed heterogeneity during the arterial phase. As for the lesion enhancement pattern, 73 lesions (59.84%) presented as persistent enhancement, 37 lesions (30.33%) as wash out, 8 lesions (6.56%) as degressive enhancement, and 4 lesions (3.28%) as poor blood supply. Additionally, 96 lesions (78.69%) with intra-tumor vessels and 85 lesions (69.67%) with outer rim were confirmed during the delayed phase. CONCLUSION At enhanced MRI, hypointensity on T1WI, hyperintensity and heterogeneous signals on T2WI, hyperintensity on DWI, little or no fat component, heterogenous hyperenhancement, persistent enhancement, intra-tumor vessels, and outer rim would be helpful to diagnose HEAML.
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Affiliation(s)
- Li Junhao
- Department of Radiology, Shanghai Geriatric Medical Center, Zhongshan Hospital, Fudan University, Shanghai, China.,Department of Interventional Radiology, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Zhang Hongxia
- Department of Radiology, Taizhou Clinical Medical School of Nanjing Medical University (Taizhou People's Hospital), Nanjing, Jiangsu Province, China
| | - Guo Jiajun
- Department of Radiology, Shanghai Geriatric Medical Center, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Ikram Ahmad
- Department of Plastic and Reconstructive Surgery, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Gao Shanshan
- Department of Radiology, Shanghai Geriatric Medical Center, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Li Jianke
- Department of Interventional Radiology, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Chen Lingli
- Department of Pathology, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Ji Yuan
- Department of Pathology, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Zeng Mengsu
- Department of Radiology, Shanghai Geriatric Medical Center, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Wang Mingliang
- Department of Radiology, Shanghai Geriatric Medical Center, Zhongshan Hospital, Fudan University, Shanghai, China.
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Zhu J, Wang G, Sun G, Xie B, Xiao W, Li Y. Primary hepatic epithelioid angiomyolipoma: a small case series. ANZ J Surg 2022; 92:1803-1808. [PMID: 35578781 DOI: 10.1111/ans.17777] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/28/2022] [Revised: 04/07/2022] [Accepted: 05/03/2022] [Indexed: 11/26/2022]
Abstract
BACKGROUND Hepatic epithelioid angiomyolipomas (HEAMLs) are rare and usually arise in case reports, and thus, we aimed to study the clinicopathological features, treatment and prognosis of these lesions. METHODS We identified patients from institutional database with HEAMLs and retrospectively collected clinical data. RESULTS Of 12 patients, 10 were females, and the median age was 49 years. A liver mass was found on chance upon routine health screening in nine patients. Imaging features included clear border (9/12), internal heterogeneity (8/12), arterial enhancement (12/12), fat (5/12), intra-tumour vessel (3/12), draining hepatic vein (2/12) and pseudocapsule (1/12). The preoperative diagnoses contained HEAML (n = 5), hepatocellular carcinoma (n = 6), and hepatic cystadenocarcinoma (n = 1). All cases had received surgery and obtained a negative margin. All specimens showed positive findings for HMB-45 and Melan A. No tumour recurrence or mortality was described with a mean follow-up time of 23.5 months. CONCLUSIONS HEAML is a low potential malignancy tumour that frequently appears in middle-aged females. It has nonspecific symptoms and may present special imaging features including intra-tumour vessel, early draining hepatic vein and lack of a pseudo capsule. The lesion is confirmed by pathological and immunohistochemical findings. Surgery and subsequent long-term follow-up are the most appropriate management approaches.
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Affiliation(s)
- Jisheng Zhu
- Department of General Surgery, The First Affiliated Hospital of Nanchang University, Nanchang, China
| | - Guiyan Wang
- Department of Gastroenterology, The First Affiliated Hospital of Nanchang University, Nanchang, China
| | - Gen Sun
- Department of General Surgery, The First Affiliated Hospital of Nanchang University, Nanchang, China
| | - Bin Xie
- Department of General Surgery, The First Affiliated Hospital of Nanchang University, Nanchang, China
| | - Weidong Xiao
- Department of General Surgery, The First Affiliated Hospital of Nanchang University, Nanchang, China
| | - Yong Li
- Department of General Surgery, The First Affiliated Hospital of Nanchang University, Nanchang, China
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Jia J, Luo J, Pan CG, Ge G, Feng M, Zou B, Liu L, Zheng S, Yu J. Single-center Experience in the Diagnosis and Treatment of Hepatic Perivascular Epithelioid Cell Neoplasm. J Clin Transl Hepatol 2022; 10:72-79. [PMID: 35233375 PMCID: PMC8845148 DOI: 10.14218/jcth.2020.00170] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/17/2020] [Revised: 03/04/2021] [Accepted: 05/20/2021] [Indexed: 12/04/2022] Open
Abstract
BACKGROUND AND AIMS Perivascular epithelioid cell neoplasms (PEComas) are a rare type of mesenchymal neoplasm and their preoperative diagnosis is challenging. In this study, we summarized the experience from a single medical center to study the examinations, clinical presentations, and pathological and histological characteristics of PEComas in the liver in order to optimize overall understanding of the diagnosis and treatment of these neoplasms. METHODS We conducted a retrospective analysis to investigate the clinical and pathological characteristics as well as imaging presentations of 75 patients diagnosed with hepatic PEComa in The First Affiliated Hospital of Zhejiang University between April 2010 and April 2020. RESULTS Among the 75 patients, 52 were women, and the median age was 48 years. Most patients had no specific symptoms, and two were admitted to the hospital for a second time owing to relapse. All patients underwent surgical resection. Histologically, 38 patients had classical angiomyolipoma (AML) and 37 had epithelioid AML. The PEComas were accompanied by positive immunohistochemical expression of HMB45, Melan-A, and smooth muscle actin. Follow-up data were obtained from 47 of the total 75 patients, through October 2020. Two patients had metastasis after surgery. CONCLUSIONS AML is the most common type of hepatic PEComa. There are no specific symptoms of hepatic PEComa, and serological examinations and imaging modalities for accurate preoperative diagnosis are lacking. Epithelioid AML should be considered a tumor of uncertain malignant potential; however, the prognosis of PEComa after resection is promising.
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Affiliation(s)
- Junjun Jia
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China
| | - Jia Luo
- Key Laboratory of Organ Transplantation, Research Center for Diagnosis and Treatment of Hepatobiliary Diseases, Hangzhou, Zhejiang, China
| | - Cheng-Gen Pan
- Key Laboratory of Organ Transplantation, Research Center for Diagnosis and Treatment of Hepatobiliary Diseases, Hangzhou, Zhejiang, China
| | - Guomei Ge
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China
| | - Meibao Feng
- Department of Pathology, The First Affiliated Hospital, College of Medicine, Hangzhou, Zhejiang, China
| | - Bei Zou
- Key Laboratory of Organ Transplantation, Research Center for Diagnosis and Treatment of Hepatobiliary Diseases, Hangzhou, Zhejiang, China
| | - Li Liu
- Department of Library, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China
| | - Shusen Zheng
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China
- Correspondence to: Shusen Zheng and Jun Yu, Department of Hepatobiliary and Pancreatic Surgery, First Affiliated Hospital of Zhejiang University School of Medicine, 79 Qingchun Road, Hangzhou, Zhejiang 310003, China. ORCID: https://orcid.org/0000-0003-1459-8261. Tel: +86-18767197319 (SZ), +86-571-87236575 (JY), E-mail: (SZ), (JY)
| | - Jun Yu
- Division of Hepatobiliary and Pancreatic Surgery, Department of Surgery, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, Zhejiang, China
- Correspondence to: Shusen Zheng and Jun Yu, Department of Hepatobiliary and Pancreatic Surgery, First Affiliated Hospital of Zhejiang University School of Medicine, 79 Qingchun Road, Hangzhou, Zhejiang 310003, China. ORCID: https://orcid.org/0000-0003-1459-8261. Tel: +86-18767197319 (SZ), +86-571-87236575 (JY), E-mail: (SZ), (JY)
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Correlation of in-vivo imaging with histopathology: A review. Eur J Radiol 2021; 144:109964. [PMID: 34619617 DOI: 10.1016/j.ejrad.2021.109964] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/28/2021] [Revised: 08/26/2021] [Accepted: 09/17/2021] [Indexed: 11/21/2022]
Abstract
Despite tremendous advancements in in vivo imaging modalities, there remains substantial uncertainty with respect to tumor delineation on in these images. Histopathology remains the gold standard for determining the extent of malignancy, with in vivo imaging to histopathologic correlation enabling spatial comparisons. In this review, the steps necessary for successful imaging to histopathologic correlation are described, including in vivo imaging, resection, fixation, specimen sectioning (sectioning technique, securing technique, orientation matching, slice matching), microtome sectioning and staining, correlation (including image registration) and performance evaluation. The techniques used for each of these steps are also discussed. Hundreds of publications from the past 20 years were surveyed, and 62 selected for detailed analysis. For these 62 publications, each stage of the correlative pathology process (and the sub-steps of specimen sectioning) are listed. A statistical analysis was conducted based on 19 studies that reported target registration error as their performance metric. While some methods promise greater accuracy, they may be expensive. Due to the complexity of the processes involved, correlative pathology studies generally include a small number of subjects, which hinders advanced developments in this field.
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Zhang S, Yang PP, Huang YC, Chen HC, Chen DL, Yan WT, Yang NN, Li Y, Li N, Feng ZZ. Hepatic perivascular epithelioid cell tumor: Clinicopathological analysis of 26 cases with emphasis on disease management and prognosis. World J Gastroenterol 2021; 27:5967-5977. [PMID: 34629813 PMCID: PMC8475011 DOI: 10.3748/wjg.v27.i35.5967] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/29/2021] [Revised: 06/11/2021] [Accepted: 08/13/2021] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Perivascular epithelioid cell tumor (PEComa) is an uncommon tumor of mesenchymal origin. Cases of PEComa in the liver are extremely rare.
AIM To analyze the clinicopathological features and treatment of hepatic PEComa and to evaluate the prognosis after different treatments.
METHODS Clinical and pathological data of 26 patients with hepatic PEComa were collected. All cases were analyzed by immunohistochemistry and clinical follow-up.
RESULTS This study included 17 females and 9 males, with a median age of 50 years. Lesions were located in the left hepatic lobe in 13 cases, in the right lobe in 11, and in the caudate lobe in 2. The median tumor diameter was 6.5 cm. Light microscopy revealed that the tumor cells were mainly composed of epithelioid cells. The cytoplasm contained heterogeneous eosinophilic granules. There were thick-walled blood vessels, around which tumor cells were radially arranged. Immunohistochemical analysis of pigment-derived and myogenic markers in PEComas revealed that 25 cases were HMB45 (+), 23 were Melan-A (+), and 22 SMA (+). TFE3 and Desmin were negative in all cases. All the fluorescence in situ hybridization samples were negative for TFE3 gene break-apart probe. Tumor tissues were collected by extended hepatic lobe resection or simple hepatic tumor resection as the main treatments. Median follow-up was 62.5 mo. None of the patients had metastasis or recurrence, and there were no deaths due to the disease.
CONCLUSION Hepatic PEComa highly expresses melanin and smooth muscle markers, and generally exhibits an inert biological behavior. The prognosis after extended hepatic lobe resection and simple hepatic tumor resection is semblable.
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Affiliation(s)
- Shan Zhang
- Department of Pathology, The Second People’s Hospital of Hefei, Hefei 230011, Anhui Province, China
| | - Pan-Pan Yang
- Department of Pathology, The Second Affiliated Hospital of Anhui Medical University, Hefei 230601, Anhui Province, China
| | - Yu-Chen Huang
- Department of Pathology, The First Affiliated Hospital of Bengbu Medical College, Bengbu 233000, Anhui Province, China
| | - Hong-Chun Chen
- Department of Pathology, The First Affiliated Hospital of Bengbu Medical College, Bengbu 233000, Anhui Province, China
| | - De-Li Chen
- Department of Gastrointestinal Surgery, The First Affiliated Hospital of Bengbu Medical College, Bengbu 233000, Anhui Province, China
| | - Wen-Tian Yan
- Department of Pathology, The First Affiliated Hospital of Bengbu Medical College, Bengbu 233000, Anhui Province, China
| | - Ning-Ning Yang
- Department of Pathology, The First Affiliated Hospital of Bengbu Medical College, Bengbu 233000, Anhui Province, China
| | - Yuan Li
- Department of Pathology, The First Affiliated Hospital of University of Science and Technology of China, Anhui Provincial Hospital, Hefei 230001, Anhui Province, China
| | - Nan Li
- Department of Pathology, The First Affiliated Hospital of Bengbu Medical College, Bengbu 233000, Anhui Province, China
| | - Zhen-Zhong Feng
- Department of Pathology, The Second Affiliated Hospital of Anhui Medical University, Hefei 230601, Anhui Province, China
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Steinborn JK, Rawitzer J, Gallinat A, Evliya C, Paul A, Schmid KW, Reis H. [Angiomyolipoma of the liver-a case report and review of the literature]. DER PATHOLOGE 2020; 41:643-648. [PMID: 32955648 DOI: 10.1007/s00292-020-00834-4] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 06/11/2023]
Abstract
We report the case of a 70-year-old female patient with an unclear liver mass after clinical and radiological evaluation. In histopathological evaluation, we diagnosed a hepatic angiomyolipoma, which is a rare entity and belongs to the perivascular epithelioid group of tumours (PEComa). In this report, we illustrate the typical histomorphology, immunohistochemical profile and molecular changes based on the case presentation and discuss and review the current literature on hepatic angiomyolipomas. In addition, typical differential diagnostic problems are highlighted.
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Affiliation(s)
- Julia Kristina Steinborn
- Institut für Pathologie, Westdeutsches Tumorzentrum, Universitätsklinikum Essen, Universität Duisburg-Essen, Hufelandstr. 55, 45147, Essen, Deutschland
| | - Josefine Rawitzer
- Institut für Pathologie, Westdeutsches Tumorzentrum, Universitätsklinikum Essen, Universität Duisburg-Essen, Hufelandstr. 55, 45147, Essen, Deutschland
| | - Anja Gallinat
- Klinik für Allgemeinchirurgie, Viszeral- und Transplantationschirurgie, Universitätsmedizin Essen, Universität Duisburg-Essen, Essen, Deutschland
| | - Cahit Evliya
- Radprax, Medizinische Versorgungszentren, Arnsberg, Deutschland
| | - Andreas Paul
- Klinik für Allgemeinchirurgie, Viszeral- und Transplantationschirurgie, Universitätsmedizin Essen, Universität Duisburg-Essen, Essen, Deutschland
| | - Kurt Werner Schmid
- Institut für Pathologie, Westdeutsches Tumorzentrum, Universitätsklinikum Essen, Universität Duisburg-Essen, Hufelandstr. 55, 45147, Essen, Deutschland
| | - Henning Reis
- Institut für Pathologie, Westdeutsches Tumorzentrum, Universitätsklinikum Essen, Universität Duisburg-Essen, Hufelandstr. 55, 45147, Essen, Deutschland.
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Liu W, Wang J, Huang Q, Lu Q, Liang W. Comparison of MRI Features of Epithelioid Hepatic Angiomyolipoma and Hepatocellular Carcinoma: Imaging Data From Two Centers. Front Oncol 2018; 8:600. [PMID: 30619742 PMCID: PMC6299843 DOI: 10.3389/fonc.2018.00600] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/29/2018] [Accepted: 11/26/2018] [Indexed: 12/13/2022] Open
Abstract
Introduction: Epithelioid hepatic angiomyolipoma (Epi-HAML) can easily be misdiagnosed as a malignant tumor such as hepatocellular carcinoma (HCC) because of the low-fat content on imaging. We analyzed and compared the magnetic resonance imaging (MRI) features of Epi-HAML and HCC, which would aid in disease diagnosis. Methods: We included 30 pathologically confirmed patients with Epi-HAML and 80 with HCC, who underwent both MRI unenhanced scan and three-phase contrast-enhanced MRI scan. The clinical and MRI features of the two groups were summarized and analyzed. Results: Epi-HAML showed significant differences compared to HCC group in terms of clinical features such as sex preference, age, concomitant diseases (hepatitis B and cirrhosis), and elevated plasma alpha-fetoprotein (AFP) (P < 0.001). In addition, there were statistically significant differences between both tumor types with regard to conventional MRI findings such as a solitary tumor (100 vs. 83.8%, P = 0.018), well-defined (93.3 vs. 71.3%, P = 0.027), mild hyperintensity (40.0 vs. 3.7%, P < 0.001) on DWI with high b-value, fat within the tumor (43.3 vs. 8.8%, P < 0.001), and rare necrosis (3.3 vs. 26.3%, P = 0.016). Besides, Epi-HAML displayed significant differences compared to HCC in terms of contrast-enhanced MRI characteristics such as draining hepatic vein (30.0 vs. 3.8%, P < 0.001), portal vein tumor thrombus (0 vs. 13.8%, P = 0.033), hypointensity at delayed phase (70.0 vs. 95%, P = 0.001), intra-tumor vessel at delayed phase (36.7 vs. 10.0%, P = 0.003), pseudocapsule (20.0 vs. 78.8%, P < 0.001), and prolonged enhancement (56.7 vs. 1.2%, P < 0.001). Conclusion: Epi-HAML frequently occurs in middle-aged women and usually lacks characteristic clinical symptoms. Typically, Epi-HAML presents as an isolated and well-defined tumor with rich vasculature. Specific MRI features such as intra-tumor fat, intra-tumor vessel, draining hepatic vein, prolonged enhancement, and lack of capsule may contribute to a more confident diagnosis of Epi-HAML.
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Affiliation(s)
- Weihai Liu
- Department of Radiology, The People's Hospital of Beilun District, Ningbo, China
| | - Jiawei Wang
- Department of Radiology, The Second Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Qiang Huang
- Department of Radiology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
| | - Qinyan Lu
- Department of Radiology, Hangzhou Aeromedicine Evaluation and Training Center of the PLA Air Force, Hangzhou, China
| | - Wenjie Liang
- Department of Radiology, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou, China
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O'Malley ME, Chawla TP, Lavelle LP, Cleary S, Fischer S. Primary perivascular epithelioid cell tumors of the liver: CT/MRI findings and clinical outcomes. Abdom Radiol (NY) 2017; 42:1705-1712. [PMID: 28246920 DOI: 10.1007/s00261-017-1074-y] [Citation(s) in RCA: 25] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/29/2022]
Abstract
OBJECTIVES The purpose of our study was to describe the CT and MRI features of primary PEComas of the liver and to document the associated clinical outcomes. METHODS Retrospective study included 20 patients with primary hepatic perivascular epithelioid cell tumors (PEComa) with pathology and clinical outcomes for correlation. RESULTS Study group included 20 patients: 16 women, 4 men; mean age 53 (range 35-77) years. Initial pathology diagnoses were classic angiomyolipoma (AML) (n = 11), epithelioid AML (n = 7), and PEComa not otherwise specified (n = 2). Mean tumor size was 5.1 (range 1.3-15.0) cm. CT/MRI features included well-defined margins 20/20 (100%), arterial enhancement 18/19 (95%), subcapsular location 17/20 (85%), heterogeneous 16/20 (80%), dysmorphic vessels 14/20 (70%), fat 13/20 (65%), hemorrhage 4/20 (20%), cystic components 4/20 (20%), and calcification 1/20 (5%). At the time of discovery, 18 patients were asymptomatic and their tumors were incidentally detected on imaging, and 2 patients were symptomatic. Ultimately, 18 tumors were benign and 2 developed metastases. CONCLUSIONS On CT/MRI, most primary hepatic PEComas were well-defined, arterial enhancing, subcapsular, heterogeneous masses that often had dysmorphic vessels and contained fat. Most tumors were benign but complications included local symptoms, bleeding, and malignant change.
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Affiliation(s)
- Martin E O'Malley
- Joint Department of Medical Imaging, Princess Margaret Hospital, University of Toronto, 3-961, 610 University Avenue, Toronto, ON, M5G 2M9, Canada. martin.o'
| | - Tanya P Chawla
- Joint Department of Medical Imaging, Mount Sinai Hospital, University of Toronto, 600 University Avenue, Room 567, Toronto, ON, M5G 1X5, Canada
| | - Lisa P Lavelle
- Joint Department of Medical Imaging, Toronto General Hospital, University of Toronto, NCSB 1C572, 585 University Avenue, Toronto, ON, M5G 2N2, Canada
- Department of Radiology, NYU Langone Medical Center, 660 First Avenue, 3rd Floor, New York, NY, 10016, USA
| | - Sean Cleary
- Division of Hepatobiliary-Pancreatic Surgery, Toronto General Hospital, University Health Network, 585 University Avenue, Toronto, ON, M5G 2N2, Canada
| | - Sandra Fischer
- Laboratory Medicine Program, Toronto General Hospital, University Health Network, University of Toronto, 200 Elizabeth Street, Toronto, ON, M5G 2C4, Canada
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Yoo-Bee H, Ri SY, Jun KK, Jiyoung K. Computerized Tomography and Magnetic Resonance Imaging Findings in Malignant Perivascular Epithelioid Cell Tumors of the Ovaries with Pulmonary Metastasis. IRANIAN JOURNAL OF RADIOLOGY 2016; 13:e34712. [PMID: 27895874 PMCID: PMC5117114 DOI: 10.5812/iranjradiol.34712] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 11/16/2015] [Revised: 02/06/2016] [Accepted: 05/02/2016] [Indexed: 12/16/2022]
Abstract
Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor composed of immunohistochemically and histologically distinctive perivascular epithelioid cells. Here, we report on the computed tomography (CT) and magnetic resonance imaging (MRI) findings of ovarian PEComa with pulmonary metastasis. The tumor was visible as a multilocular hemorrhagic mass that encased the ovarian vessels. These findings were different to those of other common ovarian tumors.
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Affiliation(s)
- Han Yoo-Bee
- Department of Radiology, Incheon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Incheon, Republic of Korea
| | - Shin Yu Ri
- Department of Radiology, Incheon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Incheon, Republic of Korea
- Corresponding author: Shin Yu Ri, Department of Radiology, Incheon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Incheon, Republic of Korea. Tel: +82-322805233, Fax: +82-322805230, E-mail:
| | - Kim Ki Jun
- Department of Radiology, Incheon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Incheon, Republic of Korea
| | - Kim Jiyoung
- Department of Hospital Pathology, Incheon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Incheon, Republic of Korea
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Chen Z, Han S, Wu J, Xiong M, Huang Y, Chen J, Yuan Y, Peng J, Song W. A systematic review: perivascular epithelioid cell tumor of gastrointestinal tract. Medicine (Baltimore) 2016; 95:e3890. [PMID: 27428182 PMCID: PMC4956776 DOI: 10.1097/md.0000000000003890] [Citation(s) in RCA: 26] [Impact Index Per Article: 2.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/14/2022] Open
Abstract
Perivascular epithelioid cell tumor (PEComa) is a rare entity with distinctive morphology and of expressing myomelanocytic markers. Gastrointestinal tract (GI) is one of the most common anatomic sites of origin and counts for 20% to 25% of all reported cases of perivascular epithelioid cell tumors not otherwise specified (PEComas-NOS). However, the biologic behavior of perivascular epithelioid cell tumors of gastrointestinal tract (GI PEComas-NOS) is still unclear. The aim of conducting this systematic review is to sum up what is known so far of the epidemiology, natural history, management and prognosis of GI PEComas-NOS.A systematic research was performed on PubMed and EMBASE using the following terms: ("perivascular epithelioid cell tumor" or "PEComa") and ("gastrointestinal tract" or "GI" or "oral " or "mouth" or "esophagus" or "gullet" or "gastric" or "stomach" or "duodenum" or "jejunum" or "ileum" or "cecum" or "colon" or "colorectal" or "sigmoid" or "rectum" or "anus" or "mesentery") up to December 1, 2015. Retrieved GI PEComas-NOS publications, which included these terms, contains case reports, case series to case characteristic researches.A total of 168 articles were reviewed, 41 GI PEComa-NOS English studies among which were retrieved for analysis. We reviewed epidemiology, natural history, management and prognosis of GI PEComa-NOS. Generally GI PEComa-NOS is believed to have women predomination. The most frequently involved location is colon with non-specific clinical signs. Pathologically, GI PEComas-NOS shows epithelioid predominance (70%), meanwhile coexpresses melanocytic and muscle markers characteristically, while immunohistochemistry is a useful tool for identify, which indicates that HMB-45 is regarded as the most sensitive reagent. Complete resection served as mainstay of treatment, while chemotherapy should be unanimously considered to apply in malignant cases. Eventually, it is necessary for closed and long-term follow-up with endoscope and imaging for ruling out local recurrence or distant metastasis of this tumor.GI PEComas-NOS lives with unclear behavior. There are still many unverified clinicopathological issues of GI PEComas-NOS that needs to be clarified. Further studies and analyses concerning this rare entity should be brought out. Thus, the randomized clinical researches (RCTs) are required to be conducted.
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Affiliation(s)
- Zehong Chen
- Department of Gastrointestinal Surgery, The First Affiliated Hospital,Sun Yat-Sen University
| | - Siqi Han
- Faculty of Medical Statistics and Epidemiology, School of Public Health,Sun Yat-Sen University
| | - Jialin Wu
- Department of Gastrointestinal Surgery, The First Affiliated Hospital,Sun Yat-Sen University
| | - Minmin Xiong
- Center for Stem Cell Biology and Tissue Engineering, Key Laboratory for Stem Cells and Tissue Engineering, Ministry of Education, Sun Yat-Sen University
| | - Yanqiao Huang
- Zhongshan Ophthalmic Center, Sun Yat-Sen University, Guangzhou, Guangdong, China
| | - Jianhui Chen
- Department of Gastrointestinal Surgery, The First Affiliated Hospital,Sun Yat-Sen University
| | - Yujie Yuan
- Department of Gastrointestinal Surgery, The First Affiliated Hospital,Sun Yat-Sen University
| | - Jianjun Peng
- Department of Gastrointestinal Surgery, The First Affiliated Hospital,Sun Yat-Sen University
| | - Wu Song
- Department of Gastrointestinal Surgery, The First Affiliated Hospital,Sun Yat-Sen University
- Correspondence: Wu Song, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, China (e-mail: )
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Comparison of CT and contrast-enhanced ultrasound findings in hepatic angiomyolipoma with pathological correlations. Abdom Radiol (NY) 2016; 41:248-56. [PMID: 26867906 DOI: 10.1007/s00261-015-0571-0] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
OBJECTIVE To compare imaging findings of CT and contrast-enhanced US (CEUS) in hepatic angiomyolipoma (HAML) and investigate their pathological correlations. METHODS Imaging findings and preoperative diagnosis of CT and CEUS were retrospectively compared head to head in 46 patients with 54 histologically proven HAMLs. Correlations between imaging features and preoperative diagnosis with pathological types of HAMLs were analyzed. RESULTS Fat was detected in 100% of lipomatous type, 84.6% of mixed type, and 7.1% of myomatous type (p = 0.000) of HAML at unenhanced CT. Well-defined hyper-echogenicity was displayed in 100% of lipomatous type, 88.5% of mixed type, 50% of myomatous type, and 66.7% of angiomatous type of HAMLs at unenhanced US. More arterial hyper-enhancement was noted on CEUS (100%) than on CT (73.1%) in mixed type (p = 0.015) and in lipomatous type (90.9% vs. 9.1%, p = 0.000) of HAMLs. Washout was present in more HAMLs on CT than on CEUS (42.6% vs. 18.5%, p = 0.007). Correct preoperative diagnosis was suggested in more HAMLs of myomatous type on CEUS than on CT (42.9% vs. 0%, p = 0.016) but showed no difference in other types of HAMLs. CONCLUSION There are considerable discrepancies between CT and CEUS findings of HAMLs, and the imaging appearance and preoperative diagnosis of HAMLs on CT and CEUS are significantly affected by pathological types of HAMLs.
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Wang ZS, Xu L, Ma L, Song MQ, Wu LQ, Zhou X. Hepatic falciform ligament clear cell myomelanocytic tumor: A case report and a comprehensive review of the literature on perivascular epithelioid cell tumors. BMC Cancer 2015; 15:1004. [PMID: 26698563 PMCID: PMC4690247 DOI: 10.1186/s12885-015-1992-4] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/02/2015] [Accepted: 12/11/2015] [Indexed: 12/11/2022] Open
Abstract
Background The objective of the study was to explore the clinical expression, radiological and pathological features, differential diagnosis, and biological behavior of a clear cell myomelanocytic tumor. In a case involving a clear cell myomelanocytic tumor located in the hepatic falciform ligament, we evaluated clinical expression, radiological characteristics, histopathology, immunohistochemistry, and biological behavior; we also reviewed the relevant literature. Case presentation Clear cell myomelanocytic tumor is a benign soft-tissue neoplasm that often occurs in women, and is expressed as a painless mass. The falciform ligament is its most frequent site of occurrence. The imaging characteristics of this lesion were uneven enhancement in the arterial phase, continuing to strengthen in the venous phase, and equal density in the balance phase. Histological and immunohistochemical analysis revealed the main transparent epithelioid cells and smooth muscle spindle cells to be HMB-45(+), smooth muscle actin(+), and melan-A (+). Conclusion Hepatic vascular epithelioid cell tumors are very rare mesenchymal neoplasms. Few studies have investigated this tumor in the hepatic falciform ligament; consequently, its diagnosis and the selection of an appropriate treatment and follow-up protocol are challenging. Treatment outcome remains unpredictable. Therefore, clear cell myomelanocytic tumor should be viewed as a tumor with uncertain malignant potential requiring long-term follow-up.
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Affiliation(s)
- Zu-Sen Wang
- Department of Hepatobiliary Surgery, Affiliated Hospital of Qingdao University, Qingdao, Shandong, 266003, China.
| | - Lin Xu
- Department of Hepatobiliary Surgery, Affiliated Hospital of Qingdao University, Qingdao, Shandong, 266003, China.
| | - Lin Ma
- Department of General Surgery, Qingdao Eighth People's Hospital, Qingdao, Shandong, 266003, China.
| | - Meng-Qi Song
- Department of Hepatobiliary Surgery, Affiliated Hospital of Qingdao University, Qingdao, Shandong, 266003, China.
| | - Li-Qun Wu
- Department of Hepatobiliary Surgery, Affiliated Hospital of Qingdao University, Qingdao, Shandong, 266003, China.
| | - Xuan Zhou
- Department of Pathology, Affiliated Hospital of Qingdao University, Qingdao, Shandong, 266003, China.
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17
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Kim R, Lee JM, Joo I, Lee DH, Woo S, Han JK, Choi BI. Differentiation of lipid poor angiomyolipoma from hepatocellular carcinoma on gadoxetic acid-enhanced liver MR imaging. ACTA ACUST UNITED AC 2015; 40:531-41. [PMID: 25231411 DOI: 10.1007/s00261-014-0244-4] [Citation(s) in RCA: 27] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/28/2022]
Abstract
PURPOSE To investigate magnetic resonance (MR) findings of angiomyolipoma (AML) on gadoxetic acid-enhanced MR imaging, and to identify features that differentiate AML from hepatocellular carcinoma (HCC) in patients with a low risk of HCC development. METHODS This retrospective study was institutional review board approved, and the requirement for informed consent was waived. Twelve patients with hepatic AML who underwent gadoxetic acid-enhanced MRI with no risk factors for HCC development were recruited. Twenty-seven patients with HCC under the same inclusion criteria were recruited as control. Two radiologists analyzed the images in consensus for morphologic features, enhancement patterns, and hepatobiliary phase (HBP) findings. All results were analyzed using the Mann-Whitney test, two-tailed Fisher exact test, and chi-square test. RESULTS Patients with AML were younger than those with HCC (48.8 ± 15 years for AML vs. 62.7 ± 14.2 years for HCC, p = 0.008) with female predominance, while most HCC patients were male (75% (9/12) vs. 15% (4/27), p < 0.001). The most prevalent enhancement pattern was arterial enhancement followed by hypointensity at portal or transitional phases for both AMLs (58% (7/12)) and HCCs (74% (20/27)) (p = 0.455). However, during the HBP, AMLs frequently showed more homogeneous hypointensity than HCCs (83% (10/12) vs. 41% (11/27), p = 0.018). When compared with the signal intensity of the spleen, the mean relative signal intensity of the AML was 91.2 ± 15.4%, while in HCCs, it was 128.7 ± 40% (p < 0.001). CONCLUSIONS Although AMLs showed similar enhancement patterns to HCCs during the dynamic phases of gadoxetic acid-enhanced MRI, using characteristic MR features of AML during the HBP and demographic differences, one can better differentiate AML from HCC.
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Affiliation(s)
- Rihyeon Kim
- Department of Radiology, Seoul National University Hospital, 101 Daehak-ro, Jongno-Gu, Seoul, 110-744, Korea
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Li R, Tang CL, Zhang Y, Ma KS, Zhang XH, Guo DY, Yan XC. Diagnosis of Hepatic Angiomyolipoma by Combination of Baseline and Contrast-Enhanced Ultrasound--A Prospective Study in Non-Cirrhotic Patients. PLoS One 2015; 10:e0132290. [PMID: 26147859 PMCID: PMC4492985 DOI: 10.1371/journal.pone.0132290] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/03/2015] [Accepted: 06/11/2015] [Indexed: 12/14/2022] Open
Abstract
Objectives Hepatic angiomyolipoma (HAML) is a rare, benign mesenchymal tumor of the liver and its diagnosis has been considered challenging. The aim of this study was to investigate prospectively the diagnostic efficacy of the incorporation of both baseline ultrasound (US) and contrast-enhanced ultrasound (CEUS) features of HAML in patients without cirrhosis. Materials and Methods Consecutive 1748 non-cirrhotic patients with focal liver lesions (FLLs) were prospectively enrolled. Baseline US and CEUS were performed before resection or biopsy. Ultrasound imaging diagnosis of FLLs was compared with the pathological results. Results Final diagnoses were established in 41 patients with HAML (2.3%) with normal alpha fetal protein (AFP) level and in 1707 patients with FLL other than HAML. Diagnostic criteria for HAML was based on the combination of baseline US and CEUS appearance of the nodule: (1) Well-defined, marked hyper-echoic nodule without surrounding hypo-echoic halo on baseline US; (2) hyper-enhancement in the arterial phase (exclude initial peripheral nodular enhancement and spoke-wheel arteries) and remains hyper-enhancement or iso-enhancement in the late phase. The diagnostic criteria were fulfilled in 31 HAMLs, 1 hepatocellular adenoma and 1 hemangioma. Ten HAMLs were misdiagnosed as other liver tumors because they did not meet the diagnostic criteria mentioned above and consequently yielded a sensitivity, specificity, positive predictive values, negative predictive values and Youden index of 75.61%, 99.88%, 93.94%, 99.42%, and 0.75 respectively. Conclusion The combination of baseline US and CEUS may lead to the correct diagnosis noninvasively in the majority of HAMLs in non-cirrhotic patients with normal AFP level.
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Affiliation(s)
- Rui Li
- Dept. Hepato-biliary-Pancreatic Surgery, Southwest Hospital Affiliated to Third Military Medical University, Chongqing, China
- * E-mail:
| | - Chun-Lin Tang
- Dept. Ultrasound, Southwest Hospital Affiliated to Third Military Medical University, Chongqing, China
| | - Yi Zhang
- Dept. Ultrasound, Southwest Hospital Affiliated to Third Military Medical University, Chongqing, China
| | - Kuan-sheng Ma
- Dept. Hepato-biliary-Pancreatic Surgery, Southwest Hospital Affiliated to Third Military Medical University, Chongqing, China
| | - Xiao-Hang Zhang
- Dept. Ultrasound, Southwest Hospital Affiliated to Third Military Medical University, Chongqing, China
| | - De-Yu Guo
- Dept. Pathology, Southwest Hospital Affiliated to Third Military Medical University, Chongqing, China
| | - Xiao-Chu Yan
- Dept. Pathology, Southwest Hospital Affiliated to Third Military Medical University, Chongqing, China
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Abstract
Background Benign liver tumors are common. They do not spread to other areas of the body, and they usually do not pose a serious health risk. In fact, in most cases, benign liver tumors are not diagnosed because patients are asymptomatic. When they are detected, it’s usually because the person has had medical imaging tests, such as an ultrasound (US), computed tomography (CT) scan, or magnetic resonance imaging (MRI), for another condition. Materials and methods A search of the literature was made using cancer literature and the PubMed, Scopus, and Web of Science (WOS) database for the following keywords: “hepatic benign tumors”, “hepatic cystic tumors”, “polycystic liver disease”, “liver macroregenerative nodules”, “hepatic mesenchymal hamartoma”, “hepatic angiomyolipoma”, “biliary cystadenoma”, and “nodular regenerative hyperplasia”. Discussion and conclusion Hepatocellular carcinoma (HCC) is one of the most common malignant tumors in some areas of the world; there is an increasing incidence worldwide. Approximately 750,000 new cases are reported per year. More than 75 % of cases occur in the Asia-Pacific region, largely in association with chronic hepatitis B virus (HBV) infection. The incidence of HCC is increasing in the USA and Europe because of the increased incidence of hepatitis C virus (HCV) infection. Unlike the liver HCC, benign tumors are less frequent. However, they represent a chapter always more interesting of liver disease. In fact, a careful differential diagnosis with the forms of malignant tumor is often required in such a way so as to direct the patient to the correct therapy. In conclusion, many of these tumors present with typical features in various imaging studies. On occasions, biopsies are required, and/or surgical removal is needed. In the majority of cases of benign hepatic tumors, no treatment is indicated. The main indication for treatment is the presence of significant clinical symptoms or suspicion of malignancy or fear of malignant transformation.
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Liu D, Shi D, Xu Y, Cao L. Management of perivascular epithelioid cell tumor of the liver: A case report and review of the literature. Oncol Lett 2013; 7:148-152. [PMID: 24348838 PMCID: PMC3861588 DOI: 10.3892/ol.2013.1689] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/04/2013] [Accepted: 10/28/2013] [Indexed: 01/28/2023] Open
Abstract
Perivascular epithelioid cell tumor (PEComa) is a rare tumor that originates from mesenchymal tissues. Cases of PEComa in the liver are extremely rare. The present study aimed to analyze the clinical features of PEComa in the liver and discuss its management. Here we report a 25-year-old female with multiple lesions of low density with poorly defined borders in the liver, as shown by a computed tomography (CT) scan. A partial hepatectomy was proceeded and PEComa was diagnosed by immunohistochemistry. No evidence of recurrence was observed during the one year follow-up. A total of 20 patients with hepatic PEComa, including one case from the present study and 19 cases that were reported in literature between June 2001 and December 2012, were reviewed and analyzed. The mean patient age was 43.4 years (range, 25-67 years) and the cases consisted of 18 female and two male patients. The tumor size ranged between 2.0×1.6 and 15.0×12.0 cm. Of the 20 patients, nine were asymptomatic and 11 had mild to significant complaints. Immunohistochemistry plays a key role in the diagnosis of PEComa. All the cases in this study were strongly positive for human melanoma black-45. A surgical resection is the gold standard for curative intent. All the patients underwent a surgical resection and none were administered perioperative chemotherapy or radiotherapy. In total, 13 of the 14 patients with follow-up information survived during the 8-36-month follow-up period and one patient succumbed due to recurrence two years after the surgery.
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Affiliation(s)
- Daren Liu
- Department of Surgery, Second Affiliated Hospital, Zhejiang University School of Medicine, Zhejiang, Hangzhou 310009, P.R. China
| | - Dike Shi
- Department of Surgery, Second Affiliated Hospital, Zhejiang University School of Medicine, Zhejiang, Hangzhou 310009, P.R. China
| | - Yuanliang Xu
- Department of Surgery, Second Affiliated Hospital, Zhejiang University School of Medicine, Zhejiang, Hangzhou 310009, P.R. China
| | - Liping Cao
- Department of Surgery, Second Affiliated Hospital, Zhejiang University School of Medicine, Zhejiang, Hangzhou 310009, P.R. China
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Ji JS, Lu CY, Wang ZF, Xu M, Song JJ. Epithelioid angiomyolipoma of the liver: CT and MRI features. ACTA ACUST UNITED AC 2013; 38:309-14. [PMID: 22610058 PMCID: PMC3594823 DOI: 10.1007/s00261-012-9911-5] [Citation(s) in RCA: 45] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
The purpose of our study was to retrospectively evaluate the computed tomography (CT)/magnetic resonance imaging features of epithelioid angiomyolipoma of the liver (Epi-HAML), with pathology as a reference. We reviewed the CT/MRI findings of six lesions of Epi-HAML and found absence of adipose tissue in the lesions. In addition, recognizing the imaging features of no capsule, and hypervasularity with central punctiform or filiform vessels as a characteristic enhancement may distinguish Epi-HAML from other hepatic tumors.
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Affiliation(s)
- Jian-song Ji
- Department of Radiology, Lishui Central Hospital, Southeast University, 289 Kuocang Road, Lishui, 323000, Zhejiang, China.
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Tan Y, Zhang H, Xiao EH. Perivascular epithelioid cell tumour: dynamic CT, MRI and clinicopathological characteristics--analysis of 32 cases and review of the literature. Clin Radiol 2012; 68:555-61. [PMID: 23245276 DOI: 10.1016/j.crad.2012.10.021] [Citation(s) in RCA: 46] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/02/2012] [Revised: 10/26/2012] [Accepted: 10/31/2012] [Indexed: 12/14/2022]
Abstract
AIM To evaluate the dynamic computed tomography (CT), magnetic resonance imaging (MRI), and clinicopathological characteristics of perivascular epithelioid cell tumours (PEComas), thus improving the diagnosis of the tumour. MATERIALS AND METHODS A retrospective analysis was undertaken of the dynamic CT, MRI, and clinicopathological characteristics of 32 PEComas diagnosed at histopathology during the period 1 January 2005 to 1 March 2012 at two hospitals. RESULTS The age of the patients ranged from 14-80 years (mean 43.3 years). There were more women in this group (19/32). Solitary tumours were identified in kidney (n = 16), liver (n = 7), gynaecological organs (n = 2), retroperitoneal soft tissue (n = 2), lung (n = 2), palate (n = 1), left groin (n = 1). One patient had multiple tumours in the liver, kidney, and retroperitoneal soft tissue. Dynamic CT (32 cases) and MRI (15 cases) demonstrated tumours that were of low density or hypointense on T1-weighted imaging (WI) and hyperintense on T2WI; some were isodense with fat (CT: 10/32; MRI: 6/15). The tumours usually had well-defined borders and were of a regular shape (CT: 26/32; MRI: 12/15). Tumour diameters ranged from 1.5-18 cm (mean 5.1 cm). Most tumours (CT: 21/32, MRI: 10/15) enhanced heterogeneously and significantly on arterial and venous phases. Tumours appeared slightly hypodense on delayed CT imaging, although some (6/32) had delayed enhancement. The expression rate of HMB-45 (human melanoma black monoclonal antibody) was 100% (32/32). Histological classification in 22 cases (22/32) was epithelioid angiomyolipoma (AML), three (3/32) were clear cell "sugar" tumours (CCSTs), two (2/32) were lymphangioleiomyomatosis (LAM), and two (2/32) were clear cell myomelanocytic tumours of the falciform ligament/ligamentum teres (CCMMT). Three tumours did not have a specific classification. CONCLUSION Knowledge of dynamic CT, MRI, and clinicopathological characteristics could help improve the diagnosis of PEComa.
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Affiliation(s)
- Y Tan
- Department of MRI, First Clinical Medical College, Shanxi Medical University, Taiyuan, Shanxi Province, China
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Ronot M, Paradis V, Duran R, Kerbaol A, Vullierme MP, Belghiti J, Valla DC, Vilgrain V. MR findings of steatotic focal nodular hyperplasia and comparison with other fatty tumours. Eur Radiol 2012; 23:914-23. [PMID: 23064717 DOI: 10.1007/s00330-012-2676-y] [Citation(s) in RCA: 39] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/25/2012] [Revised: 09/06/2012] [Accepted: 09/22/2012] [Indexed: 12/13/2022]
Abstract
OBJECTIVES To correlate MR findings with pathology in steatotic FNHs and to compare the MR findings with those of other fatty tumours developed on noncirrhotic liver in a consecutive series of resected lesions. METHODS Our population included resected FNH with intralesional steatosis (n = 25) and other resected fatty tumours selected as controls (hepatocellular adenomas and angiomyolipomas, n = 34). Lesions were classified into three groups: those with typical FNH without (group 1) or with (group 2) fat on MR and those with atypical lesions (group 3). In group 3, diagnostic criteria for other fatty tumours were applied. RESULTS There were 9 lesions in group 1 (15.3 %), 4 in group 2 (16.8 %) and 46 in group 3 (77.9 %). Group 3 contained 12 FNHs (26 %) and all the other fatty tumours. In group 3, the association of lesion homogeneity, signal intensity similar to or slightly different from adjacent liver on in-phase T1- and T2-weighted sequences, and strong arterial enhancement was observed in 7/12 (58 %) of steatotic FNHs and 3/34 (9 %) of other tumours. CONCLUSION On MR, fat within a typical FNH should not reduce the diagnostic confidence. We recommend further investigations including liver biopsy if necessary when fatty tumours exhibit atypical MR findings.
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Affiliation(s)
- Maxime Ronot
- Department of Radiology, University Paris Diderot, Sorbonne Paris Cité, University Hospitals Paris Nord Val de Seine, Beaujon, 100 bd General Leclerc, 92118 Clichy, Hauts-de-Seine, France.
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Tan Y, Xiao EH. Hepatic perivascular epithelioid cell tumor (PEComa): dynamic CT, MRI, ultrasonography, and pathologic features--analysis of 7 cases and review of the literature. Abdom Radiol (NY) 2012; 37:781-7. [PMID: 22278345 DOI: 10.1007/s00261-012-9850-1] [Citation(s) in RCA: 33] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023]
Abstract
OBJECTIVE To evaluate the dynamic CT, MRI, ultrasonography, and pathologic features of hepatic perivascular epithelioid cell tumor (PEComa), improving the understanding and diagnosis of the tumor. METHODS A retrospective analysis of CT, MRI, ultrasonography, and pathologic features of 7 hepatic PEComas diagnosed by pathology during 1st January 2005 to 1st September 2011 in our hospital. RESULTS The performance of dynamic CT, MRI, and ultrasonography revealed that lesions were regular masses with well-defined borders, the maximum diameters were 2.5-8.5 cm (mean = 4 cm), density was homogeneous, contrast-enhanced CT and MRI showed the lesions were significantly and heterogeneously enhanced on arterial phase, less enhanced on portal venous phase, and slightly hypodense on delayed phase. One patient had multiple hepatic lesions and had delayed enhancement. There were no backgrounds of hepatitis and cirrhosis, enlarged lymph nodes, or distant metastases. Pathology showed the gross appearance of the tumor was smooth. Tumor cells were round or polygonal, with clear boundaries and clear membranes, and had abundant translucent cytoplasm. Nuclei were round, with medium size. Tumor cells were epithelial-like cells and arranged in dense sheets. Immunohistochemistry showed that most of them were positive in HMB45 and MelanA, S-100, SMA, while negative in CgA, Syn, CK, CD117, CD10, and CD34. CONCLUSIONS Dynamic CT, MRI, ultrasonography, and pathology of PEComa had some characteristics of benign tumor's performance. Enhanced scan showed PEComa quickly enhanced on arterial phase and enhanced less on portal venous phase. Knowing these characteristics could help to improve the understanding and diagnosis of hepatic PEComa.
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Affiliation(s)
- Yan Tan
- Department of Radiology, Second Xiangya Hospital, Central South University, Changsha, 410011, Hunan Province, China
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Lee SJ, Kim SY, Kim KW, Shin YM, Kim HJ, Lee JS, Kim SA. Hepatic angiomyolipoma with minimal fat, mimicking hepatocellular carcinoma. Clin Mol Hepatol 2012; 18:330-5. [PMID: 23091816 PMCID: PMC3467439 DOI: 10.3350/cmh.2012.18.3.330] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/03/2023] Open
Affiliation(s)
- So Jung Lee
- Department of Radiology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
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Cai PQ, Wu YP, Xie CM, Zhang WD, Han R, Wu PH. Hepatic angiomyolipoma: CT and MR imaging findings with clinical–pathologic comparison. ACTA ACUST UNITED AC 2012; 38:482-9. [DOI: 10.1007/s00261-012-9932-0] [Citation(s) in RCA: 36] [Impact Index Per Article: 2.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/26/2023]
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Anysz-Grodzicka A, Pacho R, Grodzicki M, Koperski L, Górnicka B, Cieszanowski A, Zieniewicz K, Krawczyk M. Angiomyolipoma of the liver: analysis of typical features and pitfalls based on own experience and literature. Clin Imaging 2012; 37:320-6. [PMID: 23465986 DOI: 10.1016/j.clinimag.2012.05.010] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/02/2012] [Accepted: 05/14/2012] [Indexed: 11/19/2022]
Abstract
We present imaging findings (ultrasound, computed tomography, and magnetic resonance imaging) of eight patients with hepatic angiomyolipoma (HAML). The lesions were solitary in seven patients, and one patient had multiple tumors (n=11). Angiomyolipoma, even though a rare liver tumor, should be included in the differential diagnosis in cases of highly vascularized lesion containing a significant amount of fat. Suggestion of the diagnosis of HAML might be helpful for the pathologist in the selection of the typical histochemical staining of the tumor, allowing accurate diagnosis, which, in turn, determines the implementation of appropriate therapeutic intervention.
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Lewis RB, Lattin GE, Makhlouf HR, Levy AD. Tumors of the liver and intrahepatic bile ducts: radiologic-pathologic correlation. Magn Reson Imaging Clin N Am 2011; 18:587-609, xii. [PMID: 21094457 DOI: 10.1016/j.mric.2010.08.010] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Primary tumors of the liver can be classified pathologically based on their cell of origin into epithelial tumors, arising from hepatocytes or biliary epithelium, and nonepithelial tumors, including mesenchymal tumors and lymphoma. Characteristic findings on MR imaging can be seen in many cases. This article reviews the MR imaging appearance of these tumors with pathologic correlation.
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Affiliation(s)
- Rachel B Lewis
- Department of Radiologic Pathology, Armed Forces Institute of Pathology, 6825 16th Street NW, Washington, DC 20306-6000, USA.
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Real-time contrast-enhanced ultrasonography of resected and immunohistochemically proven hepatic angiomyolipomas. ACTA ACUST UNITED AC 2011; 35:676-82. [PMID: 20020286 DOI: 10.1007/s00261-009-9592-x] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
PURPOSE The purpose of this study was to assess real-time contrast-enhanced ultrasound in patients with hepatic angiomyolipomas with respect to contrast-enhancing kinetics and enhancement patterns. MATERIALS AND METHODS Nonlinear, low mechanical index (mechanical index less than 0.2), real-time contrast-enhanced ultrasonography was performed in 18 patients with 19 tumors after 2.4 mL bolus injection of contrast agent SonoVue. All the 19 tumors were surgically resected and immunohistochemically proven sporadic hepatic angiomyolipomas. RESULTS Inhomogeneous hyperenhancing pattern was detected 16 tumors (84.2%) and homogeneous hyperenhancing pattern in 1 tumor (0.5%) in arterial phase and portal phase, 16 lesions remained hyperenhancement but one lesion became isoenhancement in late phase. Two lesions (10.5%) were detected inhomogeneously hypoenhancement compared to liver parenchyma during arterial, portal, and late phases, with only punctiform internal enhancement. The margin of all the lesions were smooth and well defined on contrast-enhanced ultrasonography, whereas no peripheral nodular arterial enhancement, centripetal filling, or spoke-wheel like enhancement pattern were depicted. Early strong enhancement in arterial phase, rapid washout of contrast agent, and appear hypoechoic to surrounding liver tissue during portal or late phase was not at all observed. CONCLUSION Real-time contrast-enhanced ultrasonography can demonstrate typical imaging characteristics of hepatic angiomyolipomas in most cases, that is, inhomogeneous hyperenhancing pattern in arterial phase, prolonged hyperenhancement during portal and late phase with smooth and well-defined margin. This real-time dynamic imaging technique may therefore improve noninvasive characterization and differentiation of this rare, benign, hepatic mesenchymal neoplasm.
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Hepatic angiomyolipoma: a clinical experience in diagnosis and treatment. Dig Dis Sci 2010; 55:3235-40. [PMID: 20165978 DOI: 10.1007/s10620-010-1144-2] [Citation(s) in RCA: 30] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/30/2009] [Accepted: 01/26/2010] [Indexed: 12/15/2022]
Abstract
BACKGROUND Hepatic angiomyolipoma is a rare mesenchyme-derived neoplasm often misdiagnosed as hepatocellular carcinoma, and the treatment for it remains controversial. AIMS To develop the optimal preoperative diagnoses means and treatment modalities of hepatic angiomyolipoma. METHODS Retrospective analysis of the clinical features, treatment, and prognostic data of 17 hepatic AML patients admitted to Chinese People's Liberation Army Generation Hospital between 1996 and 2006. RESULTS Most hepatic angiomyolipoma were solitary. The overall preoperative diagnostic rate was 18% and the most common misdiagnosis was hepatocellular carcinoma (10/17, 59%). In three patients, observation was performed for 2-3 years before being admitted. The tumors increased 1-9 cm in size in all patients. All of the 17 patients finally received various liver resection procedures, and postoperative mortality and morbidity was 0 and 12% (2/17). After a median follow-up period of 73 months, the tumor recurred only in one patient 9 years post-operatively. CONCLUSIONS Hepatic angiomyolipoma should be suspected in liver tumor patients with normal α-fetoprotein levels and no concomitant hepatitis. Preoperative MRI combined with percutaneous fine-needle biopsy should be the diagnostic methods of choice. Small hepatic AML proved through pathologic examination (<5 cm) may be managed by observation with close follow-up, but surgery is indicated in patients suffering from large tumors or significantly larger tumors during follow-up.
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Banshodani M, Ishiyama K, Amano H, Tashiro H, Arihiro K, Itamoto T, Ohdan H. Hepatic Angiomyolipoma with Minimal Intratumoral Fat Content. Case Rep Gastroenterol 2009; 3:324-331. [PMID: 21103249 PMCID: PMC2988925 DOI: 10.1159/000255355] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
Abstract
We report a rare case of hepatic angiomyolipoma with minimal fat content. The low fat content led to an incorrect preoperative diagnosis. A 38-year-old man who was a carrier of hepatitis B virus infection incidentally presented with a hepatic tumor. His serum alpha-fetoprotein level was normal. Ultrasonography revealed a well-circumscribed, heterogeneous hypoechoic nonencapsulated liver tumor measuring 34 × 24 mm. Precontrast computed tomography (CT) did not reveal fatty attenuation in the lesion. Contrast-enhanced CT revealed a hypervascular nonencapsulated tumor in the arterial phase and moderate washing out of the contrast medium in the portal phase. A hypervascular tumor was observed on CT hepatic arteriography, and complete washing out of the contrast medium on CT during arterial portography. These findings are compatible with hepatocellular carcinoma. The tumor exhibited low signal intensity on T1-weighted images and high signal intensity on T2-weighted images; no hypointensity was observed on fat suppression images. The patient underwent left hemihepatectomy because of a preoperative diagnosis of hepatocellular carcinoma. The histopathological diagnosis was a hepatic angiomyolipoma with 5% fat content. Low fat content makes the diagnosis of this condition difficult. The absence of serum tumor markers and the presence of a nonencapsulated hypervascular tumor may facilitate the accurate preoperative diagnosis of hepatic angiomyolipomas that have a low fat content and mimic hepatocellular carcinoma.
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Affiliation(s)
- Masataka Banshodani
- Department of Surgery, Division of Frontier Medical Science, Programs for Biomedical Research, Graduate School of Biomedical Sciences, Hiroshima University, Hiroshima, Japan
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Xu PJ, Shan Y, Yan FH, Ji Y, Ding Y, Zhou ML. Epithelioid angiomyolipoma of the liver: cross-sectional imaging findings of 10 immunohistochemically-verified cases. World J Gastroenterol 2009. [PMID: 19777618 DOI: 10.3748/wjg.15.4576.pmid:] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
AIM To retrospectively evaluate the computed tomography (CT)/magnetic resonance imaging (MRI) imaging features of epithelioid angiomyolipoma of the liver (Epi-HAML), with pathology as a reference. METHODS The CT/MRI findings (number, diameter, lobar location, and appearance of lesions) in a series of 10 patients with 12 pathologically proven epithelioid angiomyolipomas of the liver were retrospectively analyzed. The imaging features, including attenuation/signal intensity characteristics, presence of fat, hypervascular, outer rim, and vessels within lesion, were evaluated and compared with that of non-Epi-HAML in 11 patients (13 lesions). The Fisher exact test was used to compare difference in probability of imaging features between the two types. RESULTS For 21 patients, CT images of 15 patients and MR images of six patients were available. No patient underwent two examinations. For the 15 patients with a CT scan, all HAML lesions in the two groups (10 Epi-HAML and seven non-Epi-HAML) manifested as hypoattenuation. For the six patients with MRI, all lesions (two Epi-HAML and six non-Epi-HAML) were hypointense on T1WI (fat suppression) and hyperintense on T2WI. There were 10 non-Epi-HAML, but only two Epi-HAML lesions showed the presence of fat, which significantly different between the two types (P = 0.005). On the dynamic contrast enhancement (DCE) imaging, eight Epi-HAML, and 13 non-Epi lesions manifested as hypervascular. Punctate or curved vessels were displayed in 10 Epi-HAML as well as in nine non-Epi lesions and outer rim enhancement could be found with eight Epi-HAML as well as six non-Epi lesions. CONCLUSION Little or no presence of adipose tissue was found to be an imaging feature of Epi-HAML, compared with the non-Epi type. In addition, hypervascularity with opacification of central punctiform or filiform vessels on DCE would be a characteristic enhancement pattern for Epi-HAML.
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Affiliation(s)
- Peng-Ju Xu
- Department of Radiology, Zhongshan Hospital Fudan University, Shanghai 200032, China
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Xu PJ, Shan Y, Yan FH, Ji Y, Ding Y, Zhou ML. Epithelioid angiomyolipoma of the liver: Cross-sectional imaging findings of 10 immunohistochemically-verified cases. World J Gastroenterol 2009; 15:4576-81. [PMID: 19777618 PMCID: PMC2752004 DOI: 10.3748/wjg.15.4576] [Citation(s) in RCA: 35] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
AIM: To retrospectively evaluate the computed tomography (CT)/magnetic resonance imaging (MRI) imaging features of epithelioid angiomyolipoma of the liver (Epi-HAML), with pathology as a reference.
METHODS: The CT/MRI findings (number, diameter, lobar location, and appearance of lesions) in a series of 10 patients with 12 pathologically proven epithelioid angiomyolipomas of the liver were retrospectively analyzed. The imaging features, including attenuation/signal intensity characteristics, presence of fat, hypervascular, outer rim, and vessels within lesion, were evaluated and compared with that of non-Epi-HAML in 11 patients (13 lesions). The Fisher exact test was used to compare difference in probability of imaging features between the two types.
RESULTS: For 21 patients, CT images of 15 patients and MR images of six patients were available. No patient underwent two examinations. For the 15 patients with a CT scan, all HAML lesions in the two groups (10 Epi-HAML and seven non-Epi-HAML) manifested as hypoattenuation. For the six patients with MRI, all lesions (two Epi-HAML and six non-Epi-HAML) were hypointense on T1WI (fat suppression) and hyperintense on T2WI. There were 10 non-Epi-HAML, but only two Epi-HAML lesions showed the presence of fat, which significantly different between the two types (P = 0.005). On the dynamic contrast enhancement (DCE) imaging, eight Epi-HAML, and 13 non-Epi lesions manifested as hypervascular. Punctate or curved vessels were displayed in 10 Epi-HAML as well as in nine non-Epi lesions and outer rim enhancement could be found with eight Epi-HAML as well as six non-Epi lesions.
CONCLUSION: Little or no presence of adipose tissue was found to be an imaging feature of Epi-HAML, compared with the non-Epi type. In addition, hypervascularity with opacification of central punctiform or filiform vessels on DCE would be a characteristic enhancement pattern for Epi-HAML.
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Wang Z, Xu HX, Xie XY, Xie XH, Kuang M, Xu ZF, Liu GJ, Chen LD, Lin MX, Lu MD. Imaging features of hepatic angiomyolipomas on real-time contrast-enhanced ultrasound. Br J Radiol 2009; 83:411-8. [PMID: 19723766 DOI: 10.1259/bjr/81174247] [Citation(s) in RCA: 28] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/12/2022] Open
Abstract
The aim of this study was to evaluate the imaging features of hepatic angiomyolipoma (AML) on contrast-enhanced ultrasound (CEUS). The imaging features of 12 pathologically proven hepatic AML lesions in 10 patients who had undergone baseline ultrasound (BUS) and CEUS examinations were evaluated retrospectively. The enhancement extent, pattern and dynamic change, along with the enhancement process, on CEUS were analysed. The diagnostic results of BUS and CEUS before pathological examination were also recorded. The results showed that 75% (9/12) of the AML lesions exhibited mixed echogenicity on BUS and most showed remarkable hyperechogenicity in combination with a hypoechoic or anechoic portion. Arterial flow signals were detected in 75% (9/12) of the lesions on colour Doppler imaging. On CEUS, 66.7% (n = 8) of the 12 lesions exhibited hyperenhancement in the arterial phase, slight hyperenhancement (n = 2) or isoenhancement (n = 6) in the portal phase, and slight hyperenhancement (n = 1) or isoenhancement (n = 7) in the late phase. Three (25%) lesions exhibited hyperenhancement in the arterial phase and hypoenhancement in both portal and late phases. One (8.3%) lesion exhibited hypoenhancement throughout the CEUS process. Before pathological examination with BUS, only 3 (25%) lesions were correctly diagnosed as hepatic AML. Conversely, on CEUS, correct diagnoses were made for 66.8% (8/12) of hepatic AMLs. Therefore, arterial hyperenhancement and subsequent sustained enhancement on CEUS were found in the majority of hepatic AMLs. The combination of BUS and CEUS leads to the correct diagnosis in the majority of hepatic AMLs, and is higher than the success rate achieved by BUS alone.
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Affiliation(s)
- Z Wang
- Department of Medical Ultrasonics, The First Affiliated Hospital, Institute of Diagnostic and Interventional Ultrasound, Sun Yat-Sen University, Guangzhou, China
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Xiao W, Zhou M, Lou H, Wang Z, Zhang M. Hemodynamic characterization of hepatic angiomyolipoma with least amount of fat evaluated by contrast-enhanced magnetic resonance angiography. ACTA ACUST UNITED AC 2009; 35:203-7. [DOI: 10.1007/s00261-009-9508-9] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/16/2009] [Accepted: 02/19/2009] [Indexed: 12/20/2022]
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Akitake R, Kimura H, Sekoguchi S, Nakamura H, Seno H, Chiba T, Fujimoto S. Perivascular epithelioid cell tumor (PEComa) of the liver diagnosed by contrast-enhanced ultrasonography. Intern Med 2009; 48:2083-6. [PMID: 20009396 DOI: 10.2169/internalmedicine.48.2133] [Citation(s) in RCA: 25] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/04/2023] Open
Abstract
Perivascular epithelioid cell (PEC) is a unique cell which expresses both myogenic and melanocytic markers, and forms PEComa. A 36-year-old woman presented with a 35 mm-diameter liver tumor. MRI showed poor fat component in the tumor. Contrast-enhanced ultrasonography using the newly developed enhancing reagent, Sonazoid, clearly demonstrated early-phase enhancement of the tumor and rapid drainage of the reagent to veins, suggesting a PEComa. Lateral segmentectomy of the liver was performed. Histologically, epithelioid tumor cells around the vessels were immunostained with both HMB-45 and alpha-smooth muscle actin, confirming the diagnosis of PEComa. No recurrence has been found for 18 months following the operation.
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Affiliation(s)
- Reiko Akitake
- Division of Gastroenterology, Kyoto First Red Cross Hospital, Kyoto
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Abstract
Benign liver tumors and tumor-like lesions cover a broad spectrum of differential diagnoses, varying from dysontogenetic cysts to pseudolesions. Focal nodular hyperplasia, adenoma and regenerating nodules play a major role in clinical practice. Based on imaging findings, these lesions can be classified as cystic or solid, solitary or multiple, and hyper- or hypovascular. Haemangioma, FNH, steroid-associated adenoma, cystadenoma and echinococcus cysts can be reliably diagnosed using imaging techniques. The same is true for pseudolesions, which are found in particular on dynamic computed tomography. Other entities such as adenoma in cirrhotic livers, angiomyolipoma, regenerating nodules or Echinococcus alveolaris cannot be safely diagnosed by imaging and require biopsy. Morphological and functional characteristics of the most common lesions in the different imaging modalities will be discussed. Where possible, a diagnostic strategy will be presented.
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Li T, Wang L, Yu HH, Sun HC, Qin LX, Ye QH, Fan J, Tang ZY. Hepatic angiomyolipoma: a retrospective study of 25 cases. Surg Today 2008; 38:529-35. [PMID: 18516533 DOI: 10.1007/s00595-007-3669-3] [Citation(s) in RCA: 24] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/18/2007] [Accepted: 07/23/2007] [Indexed: 01/22/2023]
Abstract
PURPOSE We report our experience of diagnosing and treating hepatic angiomyolipoma (HAML), a rare benign mesenchymal tumor. METHODS We analyzed retrospectively the clinicopathologic, radiological, and operative data of 25 patients who underwent surgery for HAML at our institute between November 2001 and May 2006. RESULTS Most patients (20/25) were asymptomatic and had normal liver function. Ultrasonography (US) showed a heterogeneous hyperechoic mass in 13 of 23 patients, precontrast computed tomography (CT) showed that all of 12 lesions scanned were hypodense, and magnetic resonance imaging (MRI) showed hypointensity on T1-weighted images and hyperintensity on T2-weighted images in most (5/6) lesions. Marked enhancement in the arterial phase was seen in 10 of 12 lesions on CT scans and in 6 of 6 lesions on MRI scans. All tumors were composed of varying proportions of smooth muscle, adipose tissue, and blood vessels, and showed positive immunohistochemical staining for HMB-45. All patients underwent partial hepatectomy and there was no evidence of recurrence after a median follow-up of 43 months. CONCLUSION The radiological features of HAML vary according to its histological components. The definitive diagnosis of HAML is challenging and depends on the presence of HMB-45-positive myoid cells. Hepatic angiomyolipoma is treated effectively with surgery and the prognosis is good.
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Affiliation(s)
- Tao Li
- Department of General Surgery, Qilu Hospital, Shandong University, Jinan, P.R. China
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Low SCS, Peh WCG, Muttarak M, Cheung HS, Ng IOL. Imaging features of hepatic angiomyolipomas. J Med Imaging Radiat Oncol 2008; 52:118-23. [PMID: 18373801 DOI: 10.1111/j.1440-1673.2008.01927.x] [Citation(s) in RCA: 12] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/16/2022]
Abstract
We review the imaging appearances of hepatic angiomyolipomas in patients with and without tuberous sclerosis. Sporadic hepatic angiomyolipomas have a varied appearance because of the inconstant proportion of fat, making confident imaging diagnosis difficult and necessitating biopsy in many cases. In patients with tuberous sclerosis, hepatic angiomyolipomas have a more consistent imaging appearance and, together with other features of the syndrome, can be more easily diagnosed. Preoperative diagnosis helps obviate unnecessary surgery.
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Affiliation(s)
- S C S Low
- Department of Diagnostic Radiology, KK Women's and Children's Hospital, Singapore
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40
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Xu HX, Xie XY, Lu MD, Liu GJ, Xu ZF, Liang JY, Chen LD. Unusual benign focal liver lesions: findings on real-time contrast-enhanced sonography. JOURNAL OF ULTRASOUND IN MEDICINE : OFFICIAL JOURNAL OF THE AMERICAN INSTITUTE OF ULTRASOUND IN MEDICINE 2008; 27:243-254. [PMID: 18204015 DOI: 10.7863/jum.2008.27.2.243] [Citation(s) in RCA: 13] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/25/2023]
Abstract
OBJECTIVE This presentation aims to provide an overview of the manifestations of some unusual benign focal liver lesions (FLLs) on low-acoustic power contrast-enhanced sonography (CES) with a sulfur hexafluoride contrast agent. METHODS The patients were selected retrospectively from 2209 cases with FLLs who had undergone CES examinations for characterization during the past 3 years. The pathologic examinations proved that they were intrahepatic biliary cystadenoma (n = 1), angiomyolipoma (AML; n = 4), lipoma (n = 1), biliary epithelial dysplasia (n = 1), a fungal inflammatory mass (n = 1), tuberculoma (n = 2), an inflammatory pseudotumor (n = 7), sarcoidosis (n = 1), solitary necrotic nodules (n = 2), peliosis hepatis (n = 2), and focal fibrosis after surgery (n = 4). RESULTS Contrast-enhanced sonography was beneficial in leading to a diagnosis of benignity for some lesions showing hyperenhancement during the arterial phase and sustained enhancement during the portal or late phase, such as liver AML and lipoma. The benign nature of other lesions showing no enhancement during all phases, such as solitary necrotic nodules and focal fibrosis, was also suggestible. On the other hand, for those lesions showing hyperenhancement, isoenhancement, or hypoenhancement during the arterial phase and hypoenhancement during the late phase, including intrahepatic biliary cystadenoma, biliary epithelial dysplasia, infected liver diseases, the inflammatory pseudotumor, sarcoidosis, and peliosis hepatis, the differential diagnosis between benignity and malignancy was difficult, and pathologic tests were mandatory. CONCLUSIONS The CES features of unusual benign FLLs may enrich knowledge when performing CES examinations for characterization and may provide clues for a specific diagnosis of an individual lesion such as liver AML.
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Affiliation(s)
- Hui-Xiong Xu
- Department of Medical Ultrasonics, First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, China
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Affiliation(s)
- Won Jae Lee
- Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
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Li YY, Duan YY, Yan GZ, Lv FQ, Cao W, Cao TS, Yuan LJ. Application of ultrasonography in the diagnosis and treatment tracing of hepatocellular carcinoma-associated arteriovenous fistulas. Liver Int 2007; 27:869-75. [PMID: 17617131 DOI: 10.1111/j.1478-3231.2007.01520.x] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/13/2023]
Abstract
OBJECTIVE Hepatic arteriovenous fistula (HAVF) can be caused by trauma, hepatic biopsy, bile duct radiology, etc. Small intrahepatic HAVF can be found in lesions of carcinoma and hepatocirrhosis. Accurate detection of HAVF was magnitude in the process to take appropriate treatment in clinic. The aim of this article is to evaluate the imaging diagnostic value on HAVF and to study the imaging character of HAVF in patients with hepatocellular carcinoma (HCC), and to evaluate the role of ultrasonographic and radiological techniques in the diagnosis and management of developmental intrahepatic shunts so as to assess the imaging diagnostic evaluation in a follow-up study. METHODS Seventy-eight patients diagnosed with HCC were enrolled in this study, and retrospective analysis of ultrasonographic and radiological data was carried out on all 78 patients, and 25 patients suspected of having HAVF were selected. The results from ultrasonography were compared with that from digital subtraction angiography (DSA) as a gold standard. The portal and hepatic veins, hepatic arteries and vessels around and inside the tumour patients were detected and the haemodynamic indices were recorded with ultrasonography. Ten patients with HAVF were followed up after the therapy of arterial embolization and the reversal effect of the therapy was observed. Associations of HAVF with clinical and ultrasonographic features were evaluated by stepwise logistic regression analysis. RESULTS Twenty-five of 78 HAVF patients were detected by ultrasonography and other imaging methods. Ultrasonographic parameters made excellent predictions for the patients with HAVF; sensitivity (SE) 83.3%, specificity (SP) 90.7%, positive predictive value (pPV, 80.0%), negative predictive value (nPV) 92.5% and accuracy 88.5%. Among the 25 HAVF patients, 16 were central hepatic artery-portal vein fistulae, seven were peri-hepatic artery-portal vein fistulae and two were hepatic artery-vein shunts. Characteristic ultrasonographic methods of hepatic artery-portal vein fistulae were as follows: 10 patients with hepatic artery-portal vein fistula were followed up after embolization. Compared with that of preembolization, seven cases returned to normal and in three patients abnormalities were still detected. CONCLUSION Ultrasonographic evaluation of HAVF is easy to perform, reproducible and, when present, gives a high degree of certainty for the diagnosis of HAVF. Ultrasonography is a valuable method for the diagnoses HAVF; it can offer imaging evidence after the treatment of hepatic cell cancer. HAVF in patients with HCC can be detected by ultrasonographic methods, which are characterized by changes of hepatic arteries and veins involved in fistulas. These can be used for diagnosing HAVF and evaluating its arterial embolization effect in patients with HCC.
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Affiliation(s)
- Ying Ying Li
- Department of ultrasonography diagnostics, TangDu Hospital, Fourth Military Medical University, Xi'an, China
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Bilbao I, Castro E, Dopazo C, Castro S, Allende E, Genesca J, Tallada N, Quiroga S, Margarit C. Angiomiolipoma hepático en dos pacientes con infección por el virus de la hepatitis C. GASTROENTEROLOGIA Y HEPATOLOGIA 2007; 30:222-8. [PMID: 17408551 DOI: 10.1157/13100591] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/21/2022]
Abstract
AIM The aim of this study is to present our experience with two cases of hepatic angiomyolioma in hepatitis C virus (HCV) positive patients, and to up-date the clinical manage, diagnostic and treatment of this entity. CLINICAL OBSERVATIONS Both cases were presented in women in their 4-5th decade of life. Clinical presentation was with symptoms in one but incidental in the other. Both were HCV positive. Values of alpha-fetoprotein were normal. Radiological imaging was not diagnostic. Histopathological examination and immunohistochemical findings gave the diagnosis of angiomyolipoma. At time of diagnosis the size of tumours was 4.8 and 8 cm of diameter. Both cases were treated with surgery in order to definetly rule out malignancy. After 6 and 3 years of follow-up, there is no evidence of recurrence. DISCUSSION AND CONCLUSION The hepatic angiomyolipoma is a rare benign tumour, mimicking other liver tumours. Although no patognomonic features, there are some radiological findings that point out to the diagnosis of angiomy olipoma. Nevertheless, definitive diagnosis is done by his tological and immunohistochemical findings (HMB-45). The hepatic angiomyolipoma consists of varing proportion of three elements, mature fat cells, smooth muscle cells and blood vessels. Although it is a benign tumour, the difficulty in ruling out malignancy, prompted surgical management. It is not described its relation with HCV virus, thus we consider our cases as an coincident finding.
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Affiliation(s)
- Itxarone Bilbao
- Unidad de Cirugía Hepatobiliopancreática, Servicio de Cirugía General, Unidad de Cirugía Hepatobiliopancreática y Trasplante Hepático, Hospital Vall d'Hebron, Barcelona, España.
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Wang SN, Tsai KB, Lee KT. Hepatic angiomyolipoma with trace amounts of fat: a case report and literature review. J Clin Pathol 2006; 59:1196-9. [PMID: 17071805 PMCID: PMC1860516 DOI: 10.1136/jcp.2005.027227] [Citation(s) in RCA: 15] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/03/2022]
Abstract
Hepatic angiomyolipoma (AML), a rare benign mesenchymal tumour, is characterised by the presence of mature adipose tissue, smooth-muscle cells and thick-walled blood vessels. Increasing attention to hepatic AMLs has led to the discovery that sufficient proportions of fat often allow for definite diagnoses preoperatively. However, the proportion of fatty tissue in these tumours is highly variable. One case of hepatic AML is reported, where the amount of fat was <1%. In this case, the viral hepatitis markers, including hepatitis B antigen and anti-hepatitis C virus antibody, were negative. The serum alpha-fetoprotein level was 3.4 ng/ml and in the normal range. Abdominal ultrasonography showed a hypoechoic mass measuring 5 cm in diameter and without an obvious capsule in the left lobe of the liver. A dynamic computed tomography scan showed a well-defined and slightly enhanced mass in the medial segment of the left lobe of the liver. Angiography showed that the mass was hypervascular in character. As hepatocellular carcinoma was highly suspected from these preoperative image studies, a left lobectomy was carried out. Microscopically, the amount of fat was too low to establish a diagnosis of hepatic AML. However, positive homatropine methylbromide 45 immunoreactivity of the smooth-muscle cells seemed to assist in arriving at the diagnosis.
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Affiliation(s)
- S-N Wang
- Division of Hepatobiliary Surgery, Department of Surgery, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan, USA
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Valls C, Iannacconne R, Alba E, Murakami T, Hori M, Passariello R, Vilgrain V. Fat in the liver: diagnosis and characterization. Eur Radiol 2006; 16:2292-308. [PMID: 16477402 DOI: 10.1007/s00330-006-0146-0] [Citation(s) in RCA: 97] [Impact Index Per Article: 5.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/19/2005] [Revised: 12/23/2005] [Accepted: 12/23/2005] [Indexed: 12/13/2022]
Abstract
The purpose of this article is to provide an update on imaging techniques useful for detection and characterization of fat in the liver. Imaging findings of liver steatosis, both diffuse steatosis and focal fatty change, as well as focal fatty sparing, are presented. In addition, we will review computed tomography (CT) and magnetic resonance (MR) findings of focal liver lesions with fatty metamorphosis, including hepatocellular carcinoma, hepatocellular adenoma, focal nodular hyperplasia, angiomyolipoma, lipoma, and metastases.
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Affiliation(s)
- Carlos Valls
- Department of Radiology, Hospital Universitari de Bellvitge, Barcelona, Spain.
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Abstract
Benign hepatic tumors include a broad spectrum of regenerative and true neoplastic processes. Because of advances in imaging studies such as computed tomography (CT) and magnetic resonance imaging (MRI) as well as progress in immunohistochemistry, accurate diagnosis can now be made in a large percentage of patients without surgical laparotomy or resection. This article will focus on the pathogenesis, diagnosis, and management of focal benign lesions of the liver. Many of these tumors present with typical features in various imaging studies. On occasions, biopsies are required and/or surgical removal is needed. The most common benign hepatic tumors include cavernous hemangioma, focal nodular hyperplasia, hepatic adenoma, and nodular regenerative hyperplasia. In the majority of cases of benign hepatic tumors, patients are asymptomatic, and no treatment is indicated. The main indication for treatment is the presence of significant clinical symptoms or suspicion of malignancy or fear of malignant transformation.
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Affiliation(s)
- Bo Yoon Choi
- Division of Gastroenterology and Hepatology, Department of Medicine, Stanford University Medical Center, Stanford, CA, USA
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Abstract
Angiomyolipoma is a tumor of the kidney and, more rarely, the liver, which histologically consists of smooth muscle cells, adipose cells, and abnormal blood vessels in varying proportions. This tumor is generally benign and resection is curative, but here the authors present the case of a 14-year-old girl with an unusual primary hepatic angiomyolipoma that recurred following resection and behaved aggressively. Despite stabilization with a number of novel therapies, the child ultimately died of progressive disease.
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Affiliation(s)
- Craig A McKinney
- Department of Pediatric Surgery, CS Mott Children's Hospital, University of Michigan Health System, Ann Arbor, Michigan, USA
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Current awareness. NMR IN BIOMEDICINE 2002; 15:75-86. [PMID: 11840556 DOI: 10.1002/nbm.746] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 05/23/2023]
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Yan F, Zeng M, Zhou K, Shi W, Zheng W, Da R, Fan J, Ji Y. Hepatic angiomyolipoma: various appearances on two-phase contrast scanning of spiral CT. Eur J Radiol 2002; 41:12-8. [PMID: 11750147 DOI: 10.1016/s0720-048x(01)00392-8] [Citation(s) in RCA: 42] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/19/2022]
Abstract
The purpose of this study was to describe and analyze the various appearances of hepatic angiomyolipoma (AML) on the two-phase contrast scans of spiral CT and find out the valuable signs suggesting the diagnosis. Spiral CT scanning of pre- and post-contrast arterial phase, portal venous phase were performed in 12 cases. We found that 11 of 12 lesions appeared as hypodense, the other one appeared as slightly hyperdense on pre-contrast CT scans. On the arterial phase, 11 lesions were markedly enhanced, with the central vasculature opacification in eight lesions; the only other lesion showed no marked enhancement, but with massive vessels seen within the lesion. On the portal venous phase, eight lesions remained in enhancement with the central vessels also seen in six lesions. Our results revealed that two-phase contrast scanning could demonstrate some characteristic features of AML, such as fat components within the lesions, hypervascular tumors, the absence of capsule. Especially the central vessels within the lesions suggested strongly the diagnosis of AML. As for atypical cases, the diagnosis remained difficult and should be differentiated from other hypervascular lesions by means of other imaging modalities or needle puncture biopsy.
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Affiliation(s)
- Fuhua Yan
- Department of Radiology, Zhongshan Hospital, Fudan University (former Zhongshan Hospital, Shanghai Medical University), No.180 Fenglin Road, Shanghai 200032, People's Republic of China.
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