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Yoo Y, Kim J, Song IH. Risk prediction criteria for the primary hepatic perivascular epithelioid cell tumour family, including angiomyolipoma: analysis of 132 cases with a literature review. Histopathology 2025; 86:979-992. [PMID: 39731184 DOI: 10.1111/his.15405] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/20/2024] [Revised: 11/22/2024] [Accepted: 12/15/2024] [Indexed: 12/29/2024]
Abstract
AIMS The hepatic perivascular epithelioid cell tumour (PEComa), including angiomyolipoma, exhibits diverse morphology and clinical behaviour; however, its prognostic features remain undefined. This study aimed to investigate its histological features and prognostic factors. METHODS AND RESULTS In total, 132 patients were included. Clinical data and histopathological slides were assessed along with the p53 and Ki-67 immunohistochemistry. Targeted next-generation sequencing was performed in three cases. Based on the histologic subtypes, 7 (10%), 36 (51%), 13 (18%), and 15 (21%) patients were classified as inflammatory angiomyolipoma, conventional angiomyolipoma, epithelioid angiomyolipoma, and PEComa not otherwise specified (NOS), respectively, among 71 patients who underwent surgical resection. We proposed the risk prediction criteria after defining primary tumour size ≥7 cm, infiltrative border, mitotic rate >1/10 mm2, necrosis, vascular invasion, and PEComa NOS as worrisome features, as follows: high-risk: ≥3 worrisome features; intermediate-risk: 1-2 features; low-risk: none of the features. Applying these criteria, 4 (6%), 31 (44%), and 36 (51%) patients were classified into high-, intermediate-, and low-risk groups, respectively. One patient each in the high-risk (25%) and intermediate-risk (3%) groups developed peritoneal metastases and intrahepatic recurrence, respectively, whereas none in the low-risk group showed disease progression. A literature review of clinically malignant hepatic PEComa family tumours was conducted, and upon application of our criteria, 62% (16/26) of the patients were classified as high-risk and 35% (9/26) as intermediate- or high-risk. CONCLUSION Our risk prediction criteria can effectively predict the clinical outcomes in primary hepatic PEComa.
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Affiliation(s)
- Youngeun Yoo
- Department of Pathology, Ewha Womans University College of Medicine, Seoul, Republic of Korea
| | - Jihun Kim
- Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea
| | - In Hye Song
- Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea
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2
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Lay FK, Bernasconi M, Terracciano LM, Procopio F, Palmisano A, Rahal D, Di Tommaso L. Synchronous presentation of hepatic inflammatory pseudotumour and hepatocellular carcinoma within the same nodule: a case report. Virchows Arch 2025:10.1007/s00428-025-04103-8. [PMID: 40249531 DOI: 10.1007/s00428-025-04103-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/07/2025] [Revised: 03/31/2025] [Accepted: 04/04/2025] [Indexed: 04/19/2025]
Abstract
Inflammatory pseudotumours (IPTs) are benign lesions that rarely occur in the liver, usually presenting as mass-forming lesions characterized by bland spindle cells intermingled with a mixture of inflammatory cells, etiologically related to inflammatory response caused by infection and trauma. The combination of hepatic IPTs with primary liver malignancies is anecdotal to our knowledge. This study reports a case of hepatic IPT synchronously arising at the periphery hepatocellular carcinoma (HCC) in a 66-year-old male patient who had 2 liver biopsy procedures before undergoing surgical resection. The occurrence of IPT in this case is perceived as a reactive inflammatory response due to traumatic event (prior biopsy procedures) and fungal infection (Cystobasidium slooffiae) after conducting a thorough evaluation at the histological, immunohistochemical, and molecular levels to rule out the potential differential diagnoses and to identify any infectious agents.
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Affiliation(s)
- Fanny Kamarudy Lay
- Unit of Pathology, IRCCS Humanitas Research Hospital, Rozzano, Via Manzoni 56, 20089, Milan, Italy
| | - Monica Bernasconi
- Unit of Pathology, IRCCS Humanitas Research Hospital, Rozzano, Via Manzoni 56, 20089, Milan, Italy
| | - Luigi Maria Terracciano
- Unit of Pathology, IRCCS Humanitas Research Hospital, Rozzano, Via Manzoni 56, 20089, Milan, Italy
- Department of Biomedical Sciences, Humanitas University, Milan, Italy
| | - Fabio Procopio
- Unit of Liver Surgery, IRCCS Humanitas Research Hospital, Milan, Italy
| | - Angela Palmisano
- Unit of Liver Surgery, IRCCS Humanitas Research Hospital, Milan, Italy
| | - Daoud Rahal
- Unit of Pathology, IRCCS Humanitas Research Hospital, Rozzano, Via Manzoni 56, 20089, Milan, Italy
| | - Luca Di Tommaso
- Unit of Pathology, IRCCS Humanitas Research Hospital, Rozzano, Via Manzoni 56, 20089, Milan, Italy.
- Department of Biomedical Sciences, Humanitas University, Milan, Italy.
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Choi JH, Thung SN. Mesenchymal Tumors of the Liver: An Update Review. Biomedicines 2025; 13:479. [PMID: 40002892 PMCID: PMC11852400 DOI: 10.3390/biomedicines13020479] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/22/2025] [Revised: 02/11/2025] [Accepted: 02/13/2025] [Indexed: 02/27/2025] Open
Abstract
Hepatic mesenchymal tumors (HMTs) are non-epithelial benign and malignant tumors with or without specific mesenchymal cell differentiation. They are relatively uncommon. Except for mesenchymal hamartoma, calcified nested stromal-epithelial tumor, and embryonal sarcoma, most mesenchymal lesions are not specific to the liver. Pathologists face challenges in diagnosing HMTs due to their diverse morphologies and phenotypic variations. Accurate diagnosis is critical for directing appropriate patient care and predicting outcomes. This review focuses on mesenchymal tumors with a relative predilection for the liver, including vascular and non-vascular mesenchymal neoplasms. It provides a thorough and up-to-date overview, concentrating on clinical and pathological features, differential diagnosis, and diagnostic approaches.
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Affiliation(s)
- Joon Hyuk Choi
- Department of Pathology, Yeungnam University College of Medicine, 170 Hyeonchung-ro, Namgu, Daegu 42415, Republic of Korea
| | - Swan N. Thung
- Department of Pathology, Molecular and Cell-Based Medicine, Icahn School of Medicine at Mount Sinai, 1468 Madison Avenue, New York, NY 10029, USA;
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Teng D, Xu Y, Meng Q, Yang Q. Unveiling the mystery of hepatic epithelioid angiomyolipoma: A unique case report with literature review. SAGE Open Med Case Rep 2025; 13:2050313X251314072. [PMID: 39835254 PMCID: PMC11744629 DOI: 10.1177/2050313x251314072] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/08/2024] [Accepted: 01/02/2025] [Indexed: 01/22/2025] Open
Abstract
Hepatic epithelioid angiomyolipoma is a rare mesenchymal liver tumor within the perivascular epithelioid cell tumor family, known for its uncertain malignancy and potential for aggressive behavior. Clinical presentation, laboratory findings, and imaging results lack specificity, making pathological examination critical for definitive diagnosis. This case report presents a 61-year-old male patient with a single tumor in the right liver lobe, measuring approximately 101 mm × 99 mm. Initial imaging misdiagnosed the mass as hepatocellular carcinoma. Following multidisciplinary treatment discussions and subsequent tumor resection, histopathology and immunohistochemistry confirmed hepatic epithelioid angiomyolipoma. Accurate diagnosis of hepatic epithelioid angiomyolipoma is challenging, relying on histopathological examination and immunohistochemistry as diagnostic standards. Continuous monitoring of patients with hepatic epithelioid angiomyolipoma over an extended period is deemed essential. This report highlights the positive impact of multidisciplinary treatment in diagnosing and managing hepatic epithelioid angiomyolipoma.
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Affiliation(s)
- Da Teng
- Department of Hepatobiliary Pancreatic and Splenic Surgery Ward Ⅰ, The Affiliated ChuZhou Hospital of Anhui Medical University (The First People’s Hospital of Chuzhou), ChuZhou, China
| | - Yue Xu
- Department of Ultrasound Medicine, The Affiliated ChuZhou Hospital of Anhui Medical University (The First People’s Hospital of Chuzhou), ChuZhou, China
| | - Qingtao Meng
- Department of Radiology, The Affiliated ChuZhou Hospital of Anhui Medical University (The First People’s Hospital of Chuzhou), ChuZhou, China
| | - Qingsong Yang
- Department of Hepatobiliary Pancreatic and Splenic Surgery Ward Ⅰ, The Affiliated ChuZhou Hospital of Anhui Medical University (The First People’s Hospital of Chuzhou), ChuZhou, China
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López-Terrada D, Stahlschmidt J, Pérez-Atayde AR. "Update on pediatric primary liver tumors". Virchows Arch 2025; 486:23-47. [PMID: 39836187 DOI: 10.1007/s00428-024-03985-4] [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: 08/28/2024] [Revised: 11/14/2024] [Accepted: 11/15/2024] [Indexed: 01/22/2025]
Abstract
Liver masses are common in children, however primary malignant neoplasms are rare, representing only 1% of all pediatric cancers. Hepatocellular neoplasms are the most common primary liver malignancies and hepatoblastoma (HB) is the most frequently diagnosed. The incidence of HB, which is increasing, is approximately of 2 cases per million in the United States, followed by hepatocellular carcinoma (HCC). Pediatric primary liver tumors of mesenchymal origin are less common, except for benign vascular tumors (hemangiomas). Malignant mesenchymal neoplasms represent approximately 10-15% of all, the most common being embryonal sarcoma and malignant rhabdoid tumor. Malignant vascular tumors are rare, but epithelioid hemangioendothelioma (EHE) and angiosarcoma can be seen in children. The development and adoption of consensus diagnostic, therapeutic and risk-stratifying approaches for pediatric patients with malignant liver tumors has been historically challenged by their rarity and by their diverse clinical and histological appearance. On-going collaborative efforts of international consortia including the Children's Oncology Group (COG) in North America, the German Society of Paediatric Oncology and Haematology (GPOH), the Societe Internationale d' Oncologie Pediatrique Liver Tumor Study Group (SIOPEL) in Europe and the Japanese Liver Tumor group (JPLT), have made significant contributions to understanding the clinical and histopathological features, as well as the underlying biology of pediatric liver tumors, in particular HB. A new classification of pediatric liver tumors drafted at the international consensus meeting held in Los Angeles, has been incorporated in the recent WHO classification and is currently used by the PHITT (Paediatric Hepatic Malignancy International Tumour Trial) and other therapeutic protocols. This manuscript provides an overview of salient diagnostic features and updates in classification and molecular characterization for the most common pediatric primary liver neoplasms. It also includes a brief overview of other less common but relevant tumors, which should be considered in the differential diagnosis.
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Affiliation(s)
- Dolores López-Terrada
- Department of Pathology, Texas Children's Hospital, and Baylor College of Medicine, Houston, TX, USA.
| | - Jens Stahlschmidt
- Department of Histopathology and Molecular Pathology, St James's University Hospital, Leeds Teaching Hospitals NHS Trust, Leeds, UK
| | - Antonio R Pérez-Atayde
- Department of Pathology, Boston Children's Hospital and Harvard Medical School, Boston, MA, USA
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Jiang T, Hu Y, Wang J, Li W. Fat-Poor Hepatic Angiomyolipoma in Noncirrhotic Livers: Imaging Features, Pathology, and Differential Diagnosis. J Comput Assist Tomogr 2024; 48:727-733. [PMID: 38595141 DOI: 10.1097/rct.0000000000001605] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 04/11/2024]
Abstract
OBJECTIVE To investigate imaging features of fat-poor hepatic angiomyolipomas in noncirrhotic livers in order to enhance the diagnostic accuracy for this condition. METHODS The clinical and imaging data of 19 patients with fat-poor hepatic angiomyolipoma (fpHAML) was retrospectively analyzed. RESULTS Of the 19 patients without hepatitis, cirrhosis, or sarcoidosis, 16 had no clinical symptoms. There were 20 lesions in 19 patients. Macroscopic fat, calcification, hemorrhage, necrosis, and pseudocapsule were not observed in the 20 lesions. All lesions showed marked enhancement on the arterial phase, and the degree of enhancement was significantly higher than that in the adjacent hepatic parenchyma. In 8 cases, the enhancement of the portal phase was higher than that in the arterial phase. Multiple intratumor vessels were observed in the tumor, and lesions with diameters larger than 3.0 cm were more frequently observed. The degree of enhancement of 18 lesions on portal phase or delayed phase was slightly higher than or equal to that in the surrounding hepatic parenchyma. The lesions were hyperintense on diffusion-weighted imaging and showed homogeneous hypointensity on the hepatobiliary phase. Only 6 cases showed the presence of an early draining vein. CONCLUSIONS These imaging features have some implications for the diagnosis of fpHAML. Therefore, an increased awareness of fpHAML is needed among radiologists.
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Affiliation(s)
- Tianjiao Jiang
- From the Department of Radiology, the Affiliated Hospital of Qingdao University
| | - Yanjiao Hu
- Department of Pathology, the Affiliated Hospital of Qingdao University, Qingdao, China
| | - Jiachen Wang
- From the Department of Radiology, the Affiliated Hospital of Qingdao University
| | - Wei Li
- From the Department of Radiology, the Affiliated Hospital of Qingdao University
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Ji M, Zhang Y, Liu S, Zhang M, Qiao B. Hepatic perivascular epithelioid cell tumor: a retrospective analysis of 36 cases. Front Oncol 2024; 14:1416254. [PMID: 39220650 PMCID: PMC11361986 DOI: 10.3389/fonc.2024.1416254] [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: 04/12/2024] [Accepted: 07/31/2024] [Indexed: 09/04/2024] Open
Abstract
Background and aims Hepatic perivascular epithelioid cell tumor (PEComa) is a rare type of mesenchymal neoplasm and lacks systematic reports. The aim was to analyze the features of hepatic PEComa in order to provide our own experience for diagnosis and management from a single center. Methods We retrospectively analyzed clinical data, imaging findings, pathology, treatments and prognosis of 36 patients with hepatic PEComa in the First Affiliated Hospital of Zhengzhou University from January 2016 to September 2023. Results 29 females and 7 males (median age, 47.8 years) were included in this study. The majority (26/36, 72.2%) of patients were diagnosed incidentally with non-specific symptoms. Abnormal enhancement of enlarged blood vessels (27/36,75%) can be observed on CT/MRI and only 7 patients (19.4%) were correctly diagnosed by imaging examinations. The positive immunohistochemical expressions were HMB-45(35/36,97.2%), Melan-A (34/35,97.1%), SMA (23/26,88.5%) and CD34(86.7%,26/30). Treatments include resection (24/36,67.7%), radiofrequency ablation (6/36,16.7%), transcatheter arterial chemoembolization(1/36,2.7%), conservative clinical follow-up(2/36,5.6%), and sirolimus-chemotherapy (3/36,8.3%). During the follow-up period (range, 2-81 months), except for one patient with a single intrahepatic recurrence and 3 malignant patients died in 6 months, the remaining patients had no signs of recurrence and metastasis. Conclusions Hepatic PEComa has no specific clinical features and mainly depends on clinicopathological characteristics for accurate diagnosis. Resection is the best treatment for benign PEComa, but TACE and radiofrequency ablation can also be considered in case of contraindications for surgery.
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Affiliation(s)
- Min Ji
- Department of Hepatobiliary and Pancreatic Surgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
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Zhang Y, Guo ZX, Liao Y, Yu Y, Guo R, Han X, Lan L, Zhou J. Contrast-enhanced ultrasound features of hepatic angiomyolipoma: comparison with AFP-negative and non-viral hepatocellular carcinoma. Ultrasound Int Open 2024; 10:a23186654. [PMID: 39411752 PMCID: PMC11476071 DOI: 10.1055/a-2318-6654] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/18/2023] [Accepted: 05/01/2024] [Indexed: 10/19/2024] Open
Abstract
Purpose This study aimed to compare contrast-enhanced ultrasound (CEUS) features of hepatic angiomyolipoma (HAML) and challenging cases of HCC, mainly those with no hepatitis infection but also with a low level of AFP (non-viral AFP- HCC). Materials and Methods The study included pathologically confirmed HAMLs and non-viral AFP- HCCs undergoing CEUS from 2012 to 2023. Sonovue (SV) CEUS and Sonazoid (SZ) CEUS characteristics of the two groups were compared. Results The study included 50 HAMLs (24% on SZ-CEUS) and 88 non-viral AFP- HCCs (21.6% on SZ-CEUS). The CEUS characteristics on SZ-CEUS were similar to those on SV-CEUS to a certain extent. HAMLs more frequently displayed no washout and partial washout with partial no washout, so-called PWNW, in the late phase and post-vascular phase, whereas HCCs more commonly exhibited mild washout. In the post-vascular phase, all non-viral AFP- HCCs exhibited washout, thereby facilitating differentiation from no-washoutHAMLs, superior to SV-CEUS, where some non-viral AFP- HCCs still exhibited no washout in late phase that could not be distinguished from HAMLs. It is noteworthy that PWNW was exclusively found in nodules exhibiting hyper- and hypoechoic separation of the nodules, and hyper- and hypoechoic separation of HAMLs in the post-vascular phase on SZ-CEUS demonstrated PWNW more frequently compared to the late phase, which can potentially help distinguish nodules with hyper- and hypoechoic separation as either HAML or non-viral AFP- HCC. Conclusion: This study highlighted the usefulness of SV- and SZ-CEUS for distinguishing HAML and non-viral AFP- HCC and filled in existing gaps regarding the SZ-CEUS features of HAML.
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Affiliation(s)
- Yafang Zhang
- Department of Ultrasound, State Key Laboratory of Oncology in South
China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen
University Cancer Center, Guangzhou, China
| | - Zhi-xing Guo
- Department of Ultrasound, State Key Laboratory of Oncology in South
China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen
University Cancer Center, Guangzhou, China
| | - Ying Liao
- Department of Ultrasound, State Key Laboratory of Oncology in South
China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen
University Cancer Center, Guangzhou, China
| | - Yiwen Yu
- Department of Ultrasound, State Key Laboratory of Oncology in South
China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen
University Cancer Center, Guangzhou, China
| | - Ruohan Guo
- Department of Ultrasound, State Key Laboratory of Oncology in South
China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen
University Cancer Center, Guangzhou, China
| | - Xu Han
- Department of Ultrasound, State Key Laboratory of Oncology in South
China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen
University Cancer Center, Guangzhou, China
| | - Lilong Lan
- Department of Pathology, State Key Laboratory of Oncology in South
China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen
University Cancer Center, Guangzhou, China
| | - Jianhua Zhou
- Department of Ultrasound, State Key Laboratory of Oncology in South
China, Guangdong Provincial Clinical Research Center for Cancer, Sun Yat-sen
University Cancer Center, Guangzhou, China
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Yan S, Lu JJ, Chen L, Cai WH, Wu JZ. Hepatic perivascular epithelioid cell tumors: The importance of preoperative diagnosis. World J Gastroenterol 2024; 30:1926-1933. [PMID: 38659487 PMCID: PMC11036502 DOI: 10.3748/wjg.v30.i13.1926] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/22/2024] [Revised: 03/17/2024] [Accepted: 03/19/2024] [Indexed: 04/03/2024] Open
Abstract
Accurate preoperative diagnosis is highly important for the treatment of perivascular epithelioid cell tumors (PEComas) because PEComas are mainly benign tumors and may not require surgical intervention. By analyzing the causes, properties and clinical manifestations of PEComas, we summarize the challenges and solutions in the diagnosis of PEComas.
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Affiliation(s)
- Shuai Yan
- Department of Medical School, Nantong University, Nantong 226300, Jiangsu Province, China
- Department of Hepatobiliary Surgery, Affiliated Nantong Hospital 3 of Nantong University, Nantong 226006, Jiangsu Province, China
| | - Jia-Jie Lu
- Department of Medical School, Nantong University, Nantong 226300, Jiangsu Province, China
- Department of Hepatobiliary Surgery, Affiliated Nantong Hospital 3 of Nantong University, Nantong 226006, Jiangsu Province, China
| | - Lin Chen
- Nantong Institute of Liver Disease, Affiliated Nantong Hospital 3 of Nantong University, Nantong 226006, Jiangsu Province, China
| | - Wei-Hua Cai
- Department of Medical School, Nantong University, Nantong 226300, Jiangsu Province, China
- Department of Hepatobiliary Surgery, Affiliated Nantong Hospital 3 of Nantong University, Nantong 226006, Jiangsu Province, China
| | - Jin-Zhu Wu
- Department of Medical School, Nantong University, Nantong 226300, Jiangsu Province, China
- Department of Hepatobiliary Surgery, Affiliated Nantong Hospital 3 of Nantong University, Nantong 226006, Jiangsu Province, China
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Khadka L, Luitel P, Paudel S, Neupane N, Awale L, Bhattarai A. Hepatic angiomyolipoma masquerading as abdominal pain: A case report. Int J Surg Case Rep 2024; 117:109516. [PMID: 38479126 PMCID: PMC10950876 DOI: 10.1016/j.ijscr.2024.109516] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/08/2024] [Revised: 03/04/2024] [Accepted: 03/07/2024] [Indexed: 03/24/2024] Open
Abstract
INTRODUCTION AND IMPORTANCE Hepatic angiomyolipoma (HAML) is a rare liver tumor composed of blood vessels, smooth muscle, and fat cells. HAML occurs across a wide age range, with symptoms including abdominal discomfort, bloating, and weight loss. Diagnosis is challenging due to varied imaging appearances, but histopathological examination supplemented by immunohistochemical analysis, particularly using HMB-45, is definitive. CASE PRESENTATION A 33-year-old man presented with a two-year history of right upper quadrant abdominal pain, occasionally relieved with analgesics but worsening over the past month and a half. Examinations revealed a soft, non-distended abdomen with a palpable liver. Laboratory tests, including viral markers and tumor markers were normal. Contrast-enhanced CT revealed a well-defined oval mass in liver segment III with heterogeneous enhancement leading to provisional diagnosis of HAML. The patient underwent a successful en bloc excision with no intraoperative or postoperative complications. CLINICAL DISCUSSION Surgical resection is recommended for symptomatic cases or inconclusive biopsies, with stringent follow-up necessary due to the potential for recurrence and association with other malignancies. CONCLUSION HAML may present with prolonged nonspecific abdominal symptoms. CT imaging aids in diagnosing cases with abundant fatty tissue. En bloc tumor excision proves safe and effective in treating symptomatic presentations.
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Affiliation(s)
- Laxman Khadka
- Department of General Surgery, Maharajgunj Medical Campus, Tribhuvan University Teaching Hospital, Nepal
| | - Prajjwol Luitel
- Maharajgunj Medical Campus, Institute of Medicine, Tribhuvan University, Kathmandu, Nepal.
| | - Sujan Paudel
- Maharajgunj Medical Campus, Institute of Medicine, Tribhuvan University, Kathmandu, Nepal
| | - Nischal Neupane
- Maharajgunj Medical Campus, Institute of Medicine, Tribhuvan University, Kathmandu, Nepal
| | - Laligen Awale
- Department of General Surgery, Maharajgunj Medical Campus, Tribhuvan University Teaching Hospital, Nepal
| | - Abhishek Bhattarai
- Department of General Surgery, Maharajgunj Medical Campus, Tribhuvan University Teaching Hospital, Nepal
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Giannikou K, Klonowska K, Tsuji J, Wu S, Zhu Z, Probst CK, Kao KZ, Wu CL, Rodig S, Marino-Enriquez A, Zen Y, Schaefer IM, Kwiatkowski DJ. TSC2 inactivation, low mutation burden and high macrophage infiltration characterise hepatic angiomyolipomas. Histopathology 2023; 83:569-581. [PMID: 37679051 DOI: 10.1111/his.15005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/20/2023] [Revised: 04/30/2023] [Accepted: 06/26/2023] [Indexed: 09/09/2023]
Abstract
AIMS Although TSC1 or TSC2 inactivating mutations that lead to mTORC1 hyperactivation have been reported in hepatic angiomyolipomas (hAML), the role of other somatic genetic events that may contribute to hAML development is unknown. There are also limited data regarding the tumour microenvironment (TME) of hAML. The aim of the present study was to identify other somatic events in genomic level and changes in TME that contribute to tumorigenesis in hAML. METHODS AND RESULTS In this study, we performed exome sequencing in nine sporadic hAML tumours and deep-coverage targeted sequencing for TSC2 in three additional hAML. Immunohistochemistry and multiplex immunofluorescence were carried out for 15 proteins to characterise the tumour microenvironment and assess immune cell infiltration. Inactivating somatic variants in TSC2 were identified in 10 of 12 (83%) cases, with a median allele frequency of 13.6%. Five to 18 somatic variants (median number: nine, median allele frequency 21%) not in TSC1 or TSC2 were also identified, mostly of uncertain clinical significance. Copy number changes were rare, but detection was impaired by low tumour purity. Immunohistochemistry demonstrated numerous CD68+ macrophages of distinct appearance from Küpffer cells. Multiplex immunofluorescence revealed low numbers of exhausted PD-1+/PD-L1+, FOXP3+ and CD8+ T cells. CONCLUSION hAML tumours have consistent inactivating mutations in TSC2 and have a low somatic mutation rate, similar to other TSC-associated tumours. Careful histological review, standard IHC and multiplex immunofluorescence demonstrated marked infiltration by non-neoplastic inflammatory cells, mostly macrophages.
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Affiliation(s)
- Krinio Giannikou
- Cancer Genetics Laboratory, Division of Pulmonary and Critical Care Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA
- Cancer Genome Program, Broad Institute of MIT and Harvard, Cambridge, MA, USA
- Division of Hematology and Oncology, Cancer and Blood Disease Institute, Children's Hospital Los Angeles, Los Angeles, CA, USA
| | - Katarzyna Klonowska
- Cancer Genetics Laboratory, Division of Pulmonary and Critical Care Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA
- Cancer Genome Program, Broad Institute of MIT and Harvard, Cambridge, MA, USA
| | - Junko Tsuji
- Genomics Program, Broad Institute of MIT and Harvard, Cambridge, MA, USA
| | - Shulin Wu
- Department of Pathology, Massachusetts General Hospital, Boston, MA, USA
| | - Zachary Zhu
- Cancer Genetics Laboratory, Division of Pulmonary and Critical Care Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA
| | - Clemens K Probst
- Cancer Genetics Laboratory, Division of Pulmonary and Critical Care Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA
- Department of Neurological Sciences, Larner College of Medicine, University of Vermont, Burlighton, VT, USA
| | - Katrina Z Kao
- Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA
- Center for Immuno-Oncology, Dana-Farber Cancer Institute, Boston, MA, USA
| | - Chin-Lee Wu
- Department of Pathology, Massachusetts General Hospital, Boston, MA, USA
| | - Scott Rodig
- Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA
| | - Adrian Marino-Enriquez
- Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA
| | - Yoh Zen
- Department of Diagnostic Pathology, Kobe University Hospital, Kobe, Japan
- Institute of Liver Studies, King's College Hospital, London, UK
| | - Inga-Marie Schaefer
- Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA
| | - David J Kwiatkowski
- Cancer Genetics Laboratory, Division of Pulmonary and Critical Care Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA
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Yang X, Wang Q, Zhou X, Zhou H, Jia W, Hu C, Chu J, Kong L. Retrospective analysis of hepatic perivascular epithelioid cell tumour (PEComa) in a single centre for clinical diagnosis and treatment clinical diagnosis and treatment of hepatic PEComa. Medicine (Baltimore) 2022; 101:e29506. [PMID: 35758391 PMCID: PMC9276247 DOI: 10.1097/md.0000000000029506] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/15/2020] [Accepted: 05/06/2022] [Indexed: 12/04/2022] Open
Abstract
Our primary objective was to investigate the clinical features, diagnosis, treatment and prognosis of hepatic perivascular epithelioid cell tumour (PEComa).Thirty-five cases of pathologically proven hepatic PEComa that were treated in the Department of Hepatobiliary Centre of the First Affiliated Hospital of Nanjing Medical University from January 2008 to February 2019 were retrospectively analysed, and the literature was also reviewed.Twenty-nine females and 6 males were included in this study. The mean age of these patients was 48.0 years (range, 21-75 years). Thirteen patients complained of upper abdominal pain or discomfort, while others were accidentally discovered by imaging examination. Hepatic PEComas tended to occur in the right lobe of the liver (20 cases in the right lobe, 13 in the left lobe and 2 in the caudate lobe). Two cases were characterized by multiple tumours, and the remaining cases were single lesions (range, 1.2-12 cm). Only 8 cases were correctly diagnosed by the preoperative imaging examination, and the correct diagnosis rate was only 22.9%. The postoperative immunohistochemistry analysis showed that hepatic PEComas are positive for human melanoma black 45, Melan-A and smooth muscle actin, with the exception of 1 case that was negative for Melan-A. All patients undergoing an operation accepted regular follow-up, and the average time was 66.5 months (range, 3-132 months). Two patients who experienced tumour recurrence and 1 patient who died of cardiovascular disease, but the remaining patients showed no evidence of tumour recurrence or metastasis during the follow-up period.Hepatic PEComas are a rare type of tumours that mainly occur in young and middle-aged women. The lack of clinical manifestations and imaging findings increases the difficulty of determining a preoperative diagnosis, which mainly depends on the results of pathological examinations. Surgery is currently the only effective treatment, and long-term clinical follow-up is necessary due to the aggressive behaviour and relapse of hepatic PEComa in some patients.
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Affiliation(s)
- Xiao Yang
- Department of Hepatobiliary Centre, the First Affiliated Hospital of Nanjing Medical University, Nanjing, China
- Key Laboratory of Liver Transplantation, Chinese Academy of Medical Sciences, NHC Key Laboratory of Living Donor Liver Transplantation (Nanjing Medical University), Nanjing, China
| | - Qingyuan Wang
- Department of Hepatobiliary Centre, the First Affiliated Hospital of Nanjing Medical University, Nanjing, China
- Key Laboratory of Liver Transplantation, Chinese Academy of Medical Sciences, NHC Key Laboratory of Living Donor Liver Transplantation (Nanjing Medical University), Nanjing, China
| | - Xiao Zhou
- Department of Hepatobiliary Centre, the First Affiliated Hospital of Nanjing Medical University, Nanjing, China
- Key Laboratory of Liver Transplantation, Chinese Academy of Medical Sciences, NHC Key Laboratory of Living Donor Liver Transplantation (Nanjing Medical University), Nanjing, China
| | - Haoming Zhou
- Department of Hepatobiliary Centre, the First Affiliated Hospital of Nanjing Medical University, Nanjing, China
- Key Laboratory of Liver Transplantation, Chinese Academy of Medical Sciences, NHC Key Laboratory of Living Donor Liver Transplantation (Nanjing Medical University), Nanjing, China
| | - Wenbo Jia
- Department of Hepatobiliary Centre, the First Affiliated Hospital of Nanjing Medical University, Nanjing, China
- Key Laboratory of Liver Transplantation, Chinese Academy of Medical Sciences, NHC Key Laboratory of Living Donor Liver Transplantation (Nanjing Medical University), Nanjing, China
| | - Chunyang Hu
- Department of Hepatobiliary Centre, the First Affiliated Hospital of Nanjing Medical University, Nanjing, China
- Key Laboratory of Liver Transplantation, Chinese Academy of Medical Sciences, NHC Key Laboratory of Living Donor Liver Transplantation (Nanjing Medical University), Nanjing, China
| | - Jian Chu
- Department of Hepatobiliary Centre, the First Affiliated Hospital of Nanjing Medical University, Nanjing, China
- Key Laboratory of Liver Transplantation, Chinese Academy of Medical Sciences, NHC Key Laboratory of Living Donor Liver Transplantation (Nanjing Medical University), Nanjing, China
| | - Lianbao Kong
- Department of Hepatobiliary Centre, the First Affiliated Hospital of Nanjing Medical University, Nanjing, China
- Key Laboratory of Liver Transplantation, Chinese Academy of Medical Sciences, NHC Key Laboratory of Living Donor Liver Transplantation (Nanjing Medical University), Nanjing, China
- Department of Hepatobiliary Centre, the First Affiliated Hospital of Nanjing Medical University, Nanjing, China
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Li YF, Wang L, Xie YJ. Hepatic perivascular epithelioid cell tumor: A case report. World J Clin Cases 2022; 10:4273-4279. [PMID: 35665123 PMCID: PMC9131209 DOI: 10.12998/wjcc.v10.i13.4273] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/10/2021] [Revised: 12/18/2021] [Accepted: 03/16/2022] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Perivascular epithelioid cell tumor (PEComa) is a mesenchymal tumor with histologic and immunophenotypic characteristics of perivascular epithelioid cells, has a low incidence, and can involve multiple organs. PEComa originating in the liver is extremely rare, with most cases being benign, and only a few cases are malignant. Good outcomes are achieved with radical surgical resection, but there is no effective treatment for some large tumors and specific locations that are contraindicated for surgery.
CASE SUMMARY A 32-year-old woman was admitted to our hospital with a palpable abdominal mass and progressive deterioration since the previous month. An ultrasound-guided percutaneous liver aspiration biopsy was performed. Postoperative pathological immunohistochemical staining was HMB45, Melan-A, and smooth muscle actin positive. Perivascular epithelioid tumor was diagnosed. The tumor was large and could not be completely resected by surgery. Further digital subtraction angiography revealed a rich tumor blood supply, and interventional embolization followed by surgery was recommended. Finally, the patient underwent transarterial embolization (TAE) combined with sorafenib for four cycles. Angiography reexamination indicated no clear vascular staining of the tumor, and the tumor had shrunk. The patient was followed up for a short period of time, achieved a stable condition, and surgery was recommended.
CONCLUSION Adjuvant combination treatment with TAE and sorafenib is safe and feasible as it shrinks the tumor preoperatively and facilitates surgery.
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Affiliation(s)
- Yong-Fang Li
- Department of Hepatology, Lanzhou University The Second Hospital, Lanzhou 730030, Gansu Province, China
| | - Liang Wang
- Department of Hepatology, Lanzhou University The Second Hospital, Lanzhou 730030, Gansu Province, China
| | - Yi-Jing Xie
- Department of Radiology, Lanzhou University The Second Hospital, Lanzhou 730030, Gansu Province, China
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Nguyen CL, Nguyen HH, Luong TH, Nguyen NT, Le VK, Vu TK. Giant hepatic angiomyolipoma presenting with severe anemia: A surgical case report and review of literature. Int J Surg Case Rep 2022; 94:107087. [PMID: 35658280 PMCID: PMC9171536 DOI: 10.1016/j.ijscr.2022.107087] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/17/2022] [Revised: 04/10/2022] [Accepted: 04/12/2022] [Indexed: 02/07/2023] Open
Abstract
Background Angiomyolipoma (AML) is a solid benign neoplasm with mesenchymal features. The clinical signs and symptoms of hepatic angiomyolipoma are nonspecific, and treatment strategy is variable. Presentation A 35-years-old male patient has admitted to the hospital with symptoms of severe anemia. Abdominal multi-slice computed tomography (MSCT) and Gadoxetic acid (GA)-enhanced magnetic resonance imaging (MRI) revealed a heterogeneous 23 × 17 cm-in-sized tumor with heterogeneous enhancement and increased angiogenesis. Percutaneous hepatic biopsy was proceeded and in immunohistochemistry, tumor cells responded positively to the HMB-45, SMA, and Glutamine stains, the CD-34 stain was positive for blood vessels as well as Ki-67 sporadically positive, but the Heppar1, S-100, CK stains reacted negative. The final pathologic result was consistent with the primary hepatic angiomyolipoma. The final surgical management was extended right hepatectomy with ligation of the right hepatic artery (RHA) and vein (RHV), as well as the middle hepatic vein (MHV), one month after portal embolization as well as to enlarge the remnant liver volume. Discussion A hepatic angiomyolipoma (HAML) primary origin is relatively rare, the clinical manifestations are variable and non-specific. Histological examination and immunohistochemistry staining are considered as the gold standard for HAML diagnosis. HAML are commonly expressed benign behaviors, but HAML cases with malignant behaviors were reported in a cumulative incidence. Radical surgery must be still the most effective and major treatment approach. Conclusion The present case being the first case with initial syndrome of severe anemia accounted in the English literature. Giant angiomyolipoma is composed of blood vessels and could lead to extensive internal tumoral hemorrhage. We here present a report of this case with had primary hepatic angiomyolipoma with clinical picture of severe anemia.
A hepatic angiomyolipoma (HAML) is occasionally rare, the clinical manifestations are variable and non-specific. Histological examination and immunohistochemistry staining are considered as the gold standard. Radical surgery is the most effective and major treatment approach for symptomatic or big-sized HAML.
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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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Algashaamy K, Montgomery EA, Garcia-Buitrago M. Liver mesenchymal neoplasms: something old, something new. Pathology 2021; 54:225-235. [PMID: 34965900 DOI: 10.1016/j.pathol.2021.09.022] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/16/2021] [Accepted: 09/12/2021] [Indexed: 10/19/2022]
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Attard A, Piscopo N, Schembri J, Buhagiar T, Cortis K, Ellul P. A Rare Case of PEComa of the Liver. GE-PORTUGUESE JOURNAL OF GASTROENTEROLOGY 2021; 28:217-221. [PMID: 34056048 DOI: 10.1159/000509192] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 02/07/2020] [Accepted: 04/20/2020] [Indexed: 11/19/2022]
Affiliation(s)
- Abigail Attard
- Division of Gastroenterology, Mater Dei Hospital, Triq id-Donaturi tad-Demm, Msida, Malta
| | - Naomi Piscopo
- Division of Gastroenterology, Mater Dei Hospital, Triq id-Donaturi tad-Demm, Msida, Malta
| | - John Schembri
- Division of Gastroenterology, Mater Dei Hospital, Triq id-Donaturi tad-Demm, Msida, Malta
| | - Tiffany Buhagiar
- Histopathology Department, Mater Dei Hospital, Triq id-Donaturi tad-Demm, Msida, Malta
| | - Kelvin Cortis
- Medical Imaging, Hepatobiliary Radiology and Interventional Oncology, Mater Dei Hospital, Triq id-Donaturi tad-Demm, Msida, Malta
| | - Pierre Ellul
- Division of Gastroenterology, Mater Dei Hospital, Triq id-Donaturi tad-Demm, Msida, Malta
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Günster SA, Kim M, Lock JF, Krajinovic K. Hepatic angiomyolipoma: A case report and literature review. Int J Surg Case Rep 2020; 77:345-348. [PMID: 33212308 PMCID: PMC7683232 DOI: 10.1016/j.ijscr.2020.11.045] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/31/2020] [Revised: 11/08/2020] [Accepted: 11/09/2020] [Indexed: 12/16/2022] Open
Abstract
HAML is a rare mesenchymal liver tumour which belongs to the family of perivascular epithelioid cell tumours. HAML is typically composed of blood vessels, smooth muscle, and adipose cells. HAML is characteristically positive for HMB-45. In patients with symptoms, uncertain diagnosis, or tumour growth, surgical resection should be performed. Introduction Hepatic angiomyolipoma (HAML) is a rare mesenchymal liver tumour which belongs to the family of perivascular epithelioid cell tumours (PEComas). It is typically composed of blood vessels, smooth muscle, and adipose cells, and shows strong immunoreactivity for HMB-45. Presentation of the case A 57-year-old woman was referred to our hospital with an unclear liver lesion. A fine needle biopsy revealed a suspicion of hepatic angiomyolipoma with extramedullary haematopoiesis. Preoperative imaging revealed a tumour 17 cm in diameter in the left liver lobe segments II and III. A lobectomy of the left lobe segments II and III was performed. The pathological diagnosis of hepatic angiomyolipoma was obtained. Discussion Variations in the predominance of the tissue components in HAML impedes diagnosis based on imaging alone. The most promising evidence of HAML is the histological identification of lipomatous, myomatous, and angiomatous tissue combined with immunohistochemical positivity for HMB-45. Although the tumour is considered benign, some cases have been described with malignant behaviour. Surgical resection should be considered in case of symptoms, inconclusive biopsy, or growth in follow-up. Other surgical indications may include aggressive patterns such as vascular invasion, p53 immunoreactivity, or rapidly proliferating tumour cells. Conclusion HAML is a rare liver tumour. In patients with symptoms, uncertain diagnosis, or tumour growth, surgical resection should be performed according to oncological criteria.
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Affiliation(s)
- Simone A Günster
- Surgical Department 1, Clinical Center Fürth, Jakob-Henle-Straße 1, D-90766, Fürth, Germany
| | - Mia Kim
- Department of General, Visceral, Transplantation, Vascular and Pediatric Surgery, University Hospital of Würzburg, Würzburg, Germany
| | - Johan F Lock
- Department of General, Visceral, Transplantation, Vascular and Pediatric Surgery, University Hospital of Würzburg, Würzburg, Germany
| | - Katica Krajinovic
- Surgical Department 1, Clinical Center Fürth, Jakob-Henle-Straße 1, D-90766, Fürth, Germany.
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Huang Z, Zhou P, Li S, Li K. Hepatic Angiomyolipoma: Clinical Features and Imaging Findings of Quantitative Contrast-Enhanced Ultrasound Perfusion Analysis and Magnetic Resonance Imaging. JOURNAL OF ULTRASOUND IN MEDICINE : OFFICIAL JOURNAL OF THE AMERICAN INSTITUTE OF ULTRASOUND IN MEDICINE 2020; 39:2111-2122. [PMID: 32383807 DOI: 10.1002/jum.15316] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 12/13/2019] [Revised: 03/31/2020] [Accepted: 04/06/2020] [Indexed: 06/11/2023]
Abstract
OBJECTIVES To investigate whether quantitative contrast-enhanced ultrasound (CEUS) analysis and magnetic resonance imaging (MRI) can differentiate hepatic angiomyolipoma (AML) from hepatocellular carcinoma (HCC) and hepatic epithelial angiomyolipoma (EAML) from nonepithelial AML. METHODS Retrospective analysis of 26 CEUS lesions pathologically confirmed as AML and 60 patients with confirmed HCC. Clinical and ultrasound (US) features of AML versus HCC and EAML versus nonepithelial AML were analyzed. Quantitative parameters, including arrival time, ascending slope, time to peak, peak intensity (PI), fall time (FT), and mean transit time (MTT), were analyzed. Diagnostic performance of MRI and CEUS was compared. RESULTS Angiomyolipoma was significantly different from HCC in US findings of solitary tumor (P < .001), well-defined border (P = .025), and hyperechogenicity (P < .001). Tumor types were significantly different in US findings of centripetal filling pattern (P < .001), less hypoenhancement in the CEUS delayed phase (P = .04), high PI (P = .007) and ascending slope (P = .006), and short FT (P = .001) and MTT (P < .05). The sensitivity and specificity of quantitative parameters were 61.5% to 96.2% and 55.0% to 71.7% (area under the curve, 0.642-0.773). An early draining vein and tumor vessels, intratumor fat, absence of pseudocapsules, mild signal intensity on diffusion-weighted imaging, and hypointensity in the MRI delayed phase were helpful for differentiation of AML from HCC (P < .05). The positive predictive values of CEUS and MRI were 46.3% to 100% and 42.9% to 75%, with no significant difference (P > .05). Epithelial AML was more likely to be hypoechoic with a centripetal filling pattern than nonepithelial AML (P < .05). The MTT and FT of EAML were shorter than those of nonepithelial AML (P < .05). The PI of EAML was significantly stronger than that of nonepithelial AML (P < .01). The sensitivity and specificity of quantitative parameters were 68.7% to 100.0% and 60.0% to 100.0% (area under the curve, 0.825-0.941). Less intratumor fat was helpful for differentiation of EAML from nonepithelial AML (P < .05). The positive predictive values of CEUS and MRI were 77.8% to 100% and 85.7%, with a significant difference (P < .05). CONCLUSIONS Quantitative CEUS findings could be valuable in differentiating EAML from HCC.
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Affiliation(s)
- Zhe Huang
- Department of Medical Ultrasound, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - PingPing Zhou
- Department of Medical Ultrasound, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - ShanShan Li
- Department of Medical Ultrasound, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Kaiyan Li
- Department of Medical Ultrasound, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
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Hepatic angiomyolipoma: an international multicenter analysis on diagnosis, management and outcome. HPB (Oxford) 2020; 22:622-629. [PMID: 31619346 DOI: 10.1016/j.hpb.2019.09.004] [Citation(s) in RCA: 25] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/30/2019] [Revised: 08/15/2019] [Accepted: 09/08/2019] [Indexed: 02/06/2023]
Abstract
BACKGROUND Hepatic angiomyolipoma (HAML) may easily be misdiagnosed as a malignancy. The study aim was to assess diagnostic dilemmas, clinical management and outcome of this rare tumor. METHODS This retrospective international multicenter study included all patients with pathologically proven HAML diagnosed between 1997 and 2017. Data on patient characteristics, diagnostic work-up, management and follow-up were analyzed. RESULTS Thirty-eight patients were included, 32 female. Median age was 56yrs (i.q.r. 43-64) and median HAML-diameter was 57.5 mm (i.q.r. 38.5-95.3). Thirty patients had undergone CT and 27/38 MRI of the liver, diagnostic biopsy was performed in 19/38. Initial diagnosis was incorrect in 15/38 patients, of which 13 were thought to have malignancy. In 84% biopsy resulted in a correct preoperative diagnosis. Twenty-nine patients were managed with surgical resection, 4/38 with surveillance and 3/38 with liver transplantation. Recurrence after resection occurred in two cases. No HAML related deaths or progression to malignancy were documented. CONCLUSION HAML diagnosis proved problematic even in hepatobiliary expertise centers. Biopsy is indicated and may provide valuable additional information when HAML diagnosis is considered on cross-sectional imaging, especially when surgical resection imposes a risk of complications. Conservative management with regular imaging follow-up might be justified when biopsy confirms (classic type) HAML.
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Song Z, Xu F, Dai C. Chills and fever as the first presentation of hepatic perivascular epithelioid cell tumor. Hepatobiliary Surg Nutr 2019; 8:436-438. [PMID: 31489326 DOI: 10.21037/hbsn.2019.07.03] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022]
Affiliation(s)
- Zimin Song
- Department of Hepatobiliary and Splenic Surgery, Shengjing Hospital, China Medical University, Shenyang 110004, China
| | - Feng Xu
- Department of Hepatobiliary and Splenic Surgery, Shengjing Hospital, China Medical University, Shenyang 110004, China
| | - Chaoliu Dai
- Department of Hepatobiliary and Splenic Surgery, Shengjing Hospital, China Medical University, Shenyang 110004, China
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Kong WT, Tang M, Qiu YD, Shi J, Wu M. The differential enhancement pattern of contrast enhanced ultrasound and magnetic resonance imaging characteristics in hepatic angiomyolipoma: 7 case reports. Clin Hemorheol Microcirc 2019; 71:17-26. [PMID: 30010112 DOI: 10.3233/ch-170317] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Affiliation(s)
- Wen-Tao Kong
- Department of Ultrasound, Drumtower Hospital Affiliated to Medicial College of Nanjing University, Nanjing, China
| | - Min Tang
- Department of Radiology, Drumtower Hospital Affiliated to Medicial College of Nanjing University, Nanjing, China
| | - Yu-Dong Qiu
- Department of Hepatobiliary Surgery, Drumtower Hospital Affiliated to Medicial College of Nanjing University, Nanjing, China
| | - Jiong Shi
- Department of Pathology, Drumtower Hospital Affiliated to Medicial College of Nanjing University, Nanjing, China
| | - Min Wu
- Department of Ultrasound, Drumtower Hospital Affiliated to Medicial College of Nanjing University, Nanjing, China
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23
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Garoufalia Z, Machairas N, Kostakis ID, Liakea A, Tsaparas P, Liapis G, Sotiropoulos GC. Malignant potential of epithelioid angiomyolipomas of the liver: A case report and comprehensive review of the literature. Mol Clin Oncol 2018; 9:226-230. [PMID: 30101028 DOI: 10.3892/mco.2018.1659] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/14/2018] [Accepted: 06/06/2018] [Indexed: 12/22/2022] Open
Abstract
Hepatic epithelioid angiomyolipoma (HEAML) is a rare mesenchymal tumor that has been reported to have malignant potential. We herein describe a rare case of atypical HEAML. A 43-year-old Caucasian male patient visited his general practitioner due to a productive cough persisting for >2 months. During evaluation with several imaging tests, a chest computed tomography (CT) incidentally revealed a well-demarcated lesion in the caudate lobe of the liver, sized 7 cm. An abdominal magnetic resonance imaging examination confirmed the findings of the CT. The possibility of hepatocellular carcinoma at that time could not be excluded. Due to inconclusive cross-sectional imaging, the patient underwent left hepatectomy with additional resection of segment I. The patient's postoperative course was uneventful. A diagnosis of 'atypical' HEAML was established in the present case. The majority of HEAMLs are considered to be benign, although there are several reported cases exhibiting malignant behavior, such as tumor growth, presence of atypical cells, recurrence after surgical resection, metastasis and invasive growth into the liver parenchyma and alongside the vessels. From 2000 onwards, 19 cases of malignant hepatic AML have been reported. Malignant transformation is considered to occur mostly in the epithelioid subtype. To that end, when epithelioid or atypical characteristics are identified on preoperative biopsy, resection is indicated due to the high probability of malignancy.
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Affiliation(s)
- Zoe Garoufalia
- Second Department of Propaedeutic Surgery, Laiko General Hospital, 11527 Athens, Greece
| | - Nikolaos Machairas
- Second Department of Propaedeutic Surgery, Laiko General Hospital, 11527 Athens, Greece
| | - Ioannis D Kostakis
- Second Department of Propaedeutic Surgery, Laiko General Hospital, 11527 Athens, Greece
| | - Aliki Liakea
- First Department of Pathology, Medical School, National and Kapodistrian University of Athens, 11527 Athens, Greece
| | - Petros Tsaparas
- Second Department of Propaedeutic Surgery, Laiko General Hospital, 11527 Athens, Greece
| | - George Liapis
- First Department of Pathology, Medical School, National and Kapodistrian University of Athens, 11527 Athens, Greece
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Endo K, Kuroda H, Kakisaka K, Oikawa T, Sawara K, Ishida K, Sugai T, Takikawa Y. Hepatic Angiomyolipoma Staining in the Post-vascular Phase of Contrast-enhanced Ultrasound Due to the Presence of Macrophages. Intern Med 2018; 57:1247-1251. [PMID: 29279500 PMCID: PMC5980804 DOI: 10.2169/internalmedicine.9697-17] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/30/2022] Open
Abstract
A 47-year-old Japanese man was referred to hospital after the detection of a liver tumor. Dynamic computed tomography and gadolinium ethoxybenzyl diethylenetriaminepentaacetic acid (Gd-EOB-DTPA)-enhanced magnetic resonance imaging were consistent with a diagnosis of hepatocellular carcinoma (HCC). No perfusion defect was observed in the post-vascular phase of contrast-enhanced ultrasound (CEUS). Histopathological staining of the tumor cells was positive for antibodies against HMB-45 and cluster of differentiation (CD) 68, confirming the diagnosis of hepatic angiomyolipoma (HAML). These findings indicated the presence of macrophages in HAML. We herein report a case of HAML explain how macrophages that are present within the tumor affect the staining characteristics in the post-vascular phase of CEUS.
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Affiliation(s)
- Kei Endo
- Division of Hepatology, Department of Internal Medicine, Iwate Medical University, Japan
| | - Hidekatsu Kuroda
- Division of Hepatology, Department of Internal Medicine, Iwate Medical University, Japan
| | - Keisuke Kakisaka
- Division of Hepatology, Department of Internal Medicine, Iwate Medical University, Japan
| | - Takayoshi Oikawa
- Division of Hepatology, Department of Internal Medicine, Iwate Medical University, Japan
| | - Kei Sawara
- Division of Hepatology, Department of Internal Medicine, Iwate Medical University, Japan
| | - Kazuyuki Ishida
- Department of Molecular Diagnostic Pathology, Iwate Medical University, Japan
| | - Tamotsu Sugai
- Department of Molecular Diagnostic Pathology, Iwate Medical University, Japan
| | - Yasuhiro Takikawa
- Division of Hepatology, Department of Internal Medicine, Iwate Medical University, Japan
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Yang X, Lei C, Qiu Y, Shen S, Lu C, Yan L, Wang W. Selecting a suitable surgical treatment for hepatic angiomyolipoma: a retrospective analysis of 92 cases. ANZ J Surg 2017; 88:E664-E669. [PMID: 29241297 DOI: 10.1111/ans.14323] [Citation(s) in RCA: 13] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/11/2017] [Revised: 10/26/2017] [Accepted: 10/27/2017] [Indexed: 02/05/2023]
Abstract
BACKGROUND Hepatic angiomyolipoma (HAML) is a rare and difficult-to-diagnose liver tumour. The aim of this study was to summarize experiences in the management of HAML and to recommend a practical treatment strategy. METHODS We retrospectively studied 92 patients who were diagnosed with HAML and analysed the clinical presentation, histopathological features and treatment of the tumours encountered at our institute from May 2009 to June 2016. RESULTS The patients included 67 females and 25 males who underwent at least one radiographic examination. Sixty-eight patients underwent radical hepatectomy, two patients underwent liver biopsy, and 22 patients were treated with radiofrequency ablation after liver biopsy. The tumour cells correspondingly expressed both melanoma cell markers (HMB45, MART-1) and smooth muscle cell markers. Two patients were found to have tumour recurrence (2/92, 2.2%) after radical hepatectomy, and none of the patients died. CONCLUSION Diagnosis of HAML depends on pathological findings. The treatment strategy for HAML should be selected according to the tumour size, liver biopsy, location and clinical symptoms of HAML. Patients should be followed closely after surgery because of the malignant potential of HAML.
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Affiliation(s)
- Xianwei Yang
- Department of Liver Surgery, West China Hospital of Sichuan University, Chengdu, China
| | - Chuanfen Lei
- Department of Pathology, West China Hospital of Sichuan University, Chengdu, China
| | - Yiwen Qiu
- Department of Liver Surgery, West China Hospital of Sichuan University, Chengdu, China
| | - Shu Shen
- Department of Liver Surgery, West China Hospital of Sichuan University, Chengdu, China
| | - Changli Lu
- Department of Pathology, West China Hospital of Sichuan University, Chengdu, China
| | - Lunan Yan
- Department of Liver Surgery, West China Hospital of Sichuan University, Chengdu, China
| | - Wentao Wang
- Department of Liver Surgery, West China Hospital of Sichuan University, Chengdu, China
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Abstract
OBJECTIVE The purpose of this article is to review how fat is detected on imaging and to discuss the differential diagnosis of fat-containing liver lesions. CONCLUSION Fat is a highly useful feature in characterizing liver lesions on imaging. Although a variety of liver lesions can show fat on cross-sectional imaging, the presence of fat usually indicates that the lesion is of hepatocellular origin. Less commonly, nonhepatocellular fatty lesions may be distinguished by ancillary clinical and imaging features.
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Ding H, Wei H, Liu H, Chen Y, Xue X, Weng H. The Histopathological Features and CT/MRI Imaging Performances in Hepatic Angiomyolipoma Patients. Ann Hepatol 2017; 16:759-764. [PMID: 28809731 DOI: 10.5604/01.3001.0010.2769] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
AIM To evaluate the diagnostic value of dynamic contrast-enhanced computed tomography (CT) and magnetic resonance imaging (MRI) in the differential diagnosis of hepatic angiomyolipoma (HAML) and hepatocellular carcinoma (HCC) and to clarify the relationship between histopathological features and CT or MRI imaging performances in HAML. MATERIAL AND METHODS Six HAML and 33 non-cirrhotic HCC patients confirmed by histopathology were retrospectively analyzed. The serum biomarkers, CT and MRI examinations were conventionally performed before the confirmatory histological diagnosis. The clinical data from their medical records was also analyzed. RESULTS Six HAML patients were annotated as two types according to CT and MRI imaging characteristics, including hypovascular type (n = 1) and hypervascular type (n = 5). The imaging performances of the 33 HCC patients were hypervascular type. Moreover, all the 5 hypervascular type HAML patients were misdiagnosed as HCC by CT or MRI. We also found that the hypervascular type of HAML patients contained more vessels and less fatty tissues in histopathology than hypovascular type of HAML patients. However, the clinical features included HCC high risk factors (hepatitis B or C), non-specific symptoms, male and increased serum alpha fetoprotein (AFP) were more common in HCC patients than HAML patients (P < 0.05, respectively). CONCLUSIONS The CT or MRI imaging performances of HAML patients containing more vessels and less fatty tissues in histopathology resemble the imaging performance of HCC patients. These clinical features may be of great help in the differential diagnosis in the current clinical practices.
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Affiliation(s)
- Huiguo Ding
- Capital Medical University, Beijing, China Department of Gastroenterology and Hepatology, Beijing You'an Hospital Center for Collaborative Innovation in Critical Infectious Diseases, Beijing, China
| | - Hongtao Wei
- Capital Medical University, Beijing, China Department of Gastroenterology and Hepatology, Beijing You'an Hospital Center for Collaborative Innovation in Critical Infectious Diseases, Beijing, China
| | - Hui Liu
- Capital Medical University, Beijing, China Department of Pathology, Beijing You'an Hospital
| | - Yuhan Chen
- Capital Medical University, Beijing, China Department of Gastroenterology and Hepatology, Beijing You'an Hospital Center for Collaborative Innovation in Critical Infectious Diseases, Beijing, China
| | - Xiaowei Xue
- Chinese Academy of Medical Sciences, Beijing, China Department of Pathology, Peking Union Medical College Hospital
| | - Honglei Weng
- Heidelberg University, Mannheim, Germany Department of Medicine II, Section Molecular Hepatology, Medical Faculty Mannheim
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Klompenhouwer AJ, Verver D, Janki S, Bramer WM, Doukas M, Dwarkasing RS, de Man RA, IJzermans JNM. Management of hepatic angiomyolipoma: A systematic review. Liver Int 2017; 37:1272-1280. [PMID: 28177188 DOI: 10.1111/liv.13381] [Citation(s) in RCA: 59] [Impact Index Per Article: 7.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/21/2016] [Accepted: 01/30/2017] [Indexed: 12/13/2022]
Abstract
Hepatic Angiomyolipoma (HAML) is a rare mesenchymal liver tumour assumed to be predominantly benign, although incidental cases with malignant behaviour such as invasive growth, recurrence after resection and metastases have been reported. The aim of this systematic review was to assess the biological behaviour, estimate the risk of HAML related mortality and recommend on a justifiable management strategy. We performed a systematic literature search in Embase, Medline, Web-of-Science, Scopus, Pubmed Publisher, Cochrane and Google Scholar. We included all articles published from inception until March 2016 which reported on follow-up of various treatment strategies. We included 18 articles reporting on 292 patients. Male:female ratio was estimated at 1:3 with gender not reported in 31 cases. Of 292 patients 247 were treated with surgery, including one liver transplant, seven with chemotherapy or Sirolimus, three with embolization, and 35 conservatively. Recurrence after resection was described in 6/247 (2.4%) with pathologically proven HAML resulting in metastases and death in 2/247 (mortality rate 0.8%). Progression was described in 6/35 patients treated conservatively (21.4%). Two of 12 patients with malignant behaviour of HAML had an epithelioid-type HAML, of the remaining 10 histological subtype was undefined. With a risk estimate of 0.8% in surgically treated patients HAML related mortality is very low. Biopsy is indicated when imaging is inconclusive. In case of certain HAML diagnosis on imaging conservative management with annual imaging is justified. Resection should be considered in case of symptoms, inconclusive biopsy or growth in follow-up.
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Affiliation(s)
| | - Danielle Verver
- Department of Surgery, Erasmus MC, Rotterdam, The Netherlands
| | - Shiromani Janki
- Department of Surgery, Erasmus MC, Rotterdam, The Netherlands
| | | | - Michail Doukas
- Department of Pathology, Erasmus MC, Rotterdam, The Netherlands
| | | | - Robert A de Man
- Department of Gastroenterology and Hepatology, Erasmus MC, Rotterdam, The Netherlands
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Wang Q, Huang HF, Li ZT, Zhao XR, Duan J, Lin J, Xia ZC, Zeng Z. Diagnosis and treatment of hepatic perivascular epithelioid cell tumor: A case report and literature review. Shijie Huaren Xiaohua Zazhi 2016; 24:4315-4318. [DOI: 10.11569/wcjd.v24.i31.4315] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [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
Liver perivascular epithelioid cell tumor is a rare liver mesenchymal tumor with an insidious onset. This entity is often misdiagnosed due to the lack of typical clinical symptoms and signs and the low diagnosis rate achieved by imaging examinations. Here we report a case of liver perivascular epithelioid cell tumor. We also performed a literature review to summarize and analyze the clinical characteristics and treatment of this disease.
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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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Visualizing Central Vessels of Hepatic Angiomyolipoma Devoid of Fat Using a 2D Multi-Breath-Hold Susceptibility-Weighted Imaging. Case Rep Radiol 2015; 2015:197431. [PMID: 26114004 PMCID: PMC4465709 DOI: 10.1155/2015/197431] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/18/2015] [Accepted: 05/24/2015] [Indexed: 11/18/2022] Open
Abstract
Epithelioid hepatic angiomyolipoma (Epi-HAML) is a rare benign mesenchymal tumor with malignant potential. Most of Epi-HAML contains no or only a minimal amount of adipose tissue and poses a diagnostic challenge. Central vessels are characteristic imaging finding of Epi-HAML, which usually were displayed by dynamic contrast imaging. In this paper, we displayed the central vessels of Epi-HAML invisible on conventional MR images using a new developed abdominal susceptibility-weighted imaging (SWI). To the best of our knowledge, this is the first description for the role of SWI in characterization of Epi-HAML.
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Siegelman ES, Chauhan A. MR characterization of focal liver lesions: pearls and pitfalls. Magn Reson Imaging Clin N Am 2015; 22:295-313. [PMID: 25086931 DOI: 10.1016/j.mric.2014.04.005] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
Magnetic resonance (MR) can characterize specific tissue subtypes, thus facilitating focal liver lesion diagnosis. Focal liver lesions that are isointense to hyperintense to liver on T1-weighted images are usually hepatocellular in origin. Chemical shift imaging can narrow the differential diagnosis by detecting the presence of lipid or iron. T2 and heavily T2-weigthed fast spin echo imaging can differentiate solid from nonsolid focal liver lesions. The authors illustrate these MR imaging pearls and the uncommon exceptions (pitfalls). The authors hope that you will find this less traditional contribution to the Magnetic Resonance Clinics of North America helpful in clinical practice.
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Affiliation(s)
- Evan S Siegelman
- Department of Radiology, Perelman School of Medicine, University of Pennsylvania, 34th and Spruce Streets, 1st Floor Silverstein, Philadelphia, PA 19104-4283, USA.
| | - Anil Chauhan
- Department of Radiology, Perelman School of Medicine, University of Pennsylvania, 34th and Spruce Streets, 1st Floor Silverstein, Philadelphia, PA 19104-4283, USA
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Wang B, Ye Z, Chen Y, Zhao Q, Huang M, Chen F, Li Y, Jiang T. Hepatic angiomyolipomas: ultrasonic characteristics of 25 patients from a single center. ULTRASOUND IN MEDICINE & BIOLOGY 2015; 41:393-400. [PMID: 25542497 DOI: 10.1016/j.ultrasmedbio.2014.09.014] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 04/29/2014] [Revised: 08/29/2014] [Accepted: 09/02/2014] [Indexed: 06/04/2023]
Abstract
Twenty-five pathologically proven hepatic angiomyolipomas (AMLs) were included in the study. Ultrasonic features of hepatic AMLs were reviewed. Three types of echogenicity were observed on ultrasound examination: (i) strong hyper-echogenicity, (ii) moderate hyper-echogenicity and (iii) hypo-echogenicity. Vascular signals within tumors could be detected in 22 (88.00%) tumors as multiple punctiform, filiform or dendriform signals by color Doppler flow imaging. Based on the enhancement patterns in the arterial, portal and late phases, the features of hepatic AMLs on contrast-enhanced ultrasound were divided into four subtypes: (i) "fast in slow out" (68.00%, n = 17); (ii) "fast in same out" (16%, n = 4); (iii) "fast in fast out" (12.00%, n = 3); and (iv) "fast in uneven out" (4.00%, n = 1). Contrast-enhanced ultrasound diagnosed 22 (88.00%) tumors as benign tumors and 13 (52.00%) as hepatic AMLs. Four cases were misdiagnosed as hepatic hemangioma, five cases as focal nodular hyperplasia (total = 36.00%). The rate of correct diagnosis of hepatic AMLs increased significantly from 24.00% for ultrasound alone to 52.00% for contrast-enhanced ultrasound. Therefore, information obtained from ultrasound, color Doppler flow imaging and contrast-enhanced ultrasound should be combined to improve diagnosis.
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Affiliation(s)
- Baohua Wang
- Department of Ultrasound, First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang Province, China
| | - Zhengdu Ye
- Department of Ultrasound, First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang Province, China
| | - Yan Chen
- Department of Ultrasound, First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang Province, China
| | - Qiyu Zhao
- Department of Hepatobiliary Pancreatic Surgery, First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang Province, China
| | - Min Huang
- Department of Ultrasound, First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang Province, China
| | - Fen Chen
- Department of Hepatobiliary Pancreatic Surgery, First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang Province, China
| | - Yanyuan Li
- Department of Pathology, First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang Province, China
| | - Tian'an Jiang
- Department of Ultrasound, First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang Province, China.
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Li YJ, Liu WY, Liu L, Zhou G, Fang MY. Diagnosis and treatment of hepatic angioleiomyolipoma: A case report and literature review. Shijie Huaren Xiaohua Zazhi 2014; 22:2946-2951. [DOI: 10.11569/wcjd.v22.i20.2946] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [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 investigate the clinical features, imaging manifestations, pathological characteristics, differential diagnosis, treatment and prognosis of hepatic angiomyolipoma (HAML).
METHODS: A case of HAML diagnosed at our hospital is reported. A review of the literature was performed to retrospectively analyze 540 cases reported in China between 1987 and 2014.
RESULTS: HAML was more frequently found in females (2.7:1). Most of the patients (66.7%) presented no significant symptoms, and tumor marks were negative in all the patients. CT performed better for preoperative diagnosis than MRI (CT vs MRI: χ2 = 11.321, P = 0.001) and ultrasound. Immunohistochemical analyses revealed that the positive expression rates of human melanoma black-45 antigen (HMB-45), smooth muscle actin (SMA), Vimentin, CD34 and CD117 in HAML were 97.8% (357/365), 93.7% (207/221), 62.5% (30/48), 40.5% (51/126) and 39.0% (48/123), respectively. The expression of HMB-45 was considered to have great significance in confirming the diagnosis. Of all the patients, 521 (96.3%) underwent surgical intervention, and recurrence or metastasis was found in only 3 patients during follow-up.
CONCLUSION: HAML lacks specific symptoms or signs, has diverse imaging manifestations, and can be easily misdiagnosed. Pathological and immunohistochemical analyses are essential for diagnosis, and surgical treatment has a good prognosis.
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Zhu Z, Yang L, Zhao XM, Luo DQ, Zhang HT, Zhou CW. Myomatous hepatic angiomyolipoma: imaging findings in 14 cases with radiological-pathological correlation and review of the literature. Br J Radiol 2014; 87:20130712. [PMID: 24670055 DOI: 10.1259/bjr.20130712] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/15/2022] Open
Abstract
OBJECTIVE To display and analyse the imaging features of myomatous hepatic angiomyolipomas (MHAMLs). METHODS The imaging features (CT = 9; MRI = 10; ultrasound = 10; contrast-enhanced ultrasound = 4) of 14 patients with pathologically proven MHAMLs were reviewed retrospectively. RESULTS MHAMLs were surgically resected in the 14 patients (10 females and 4 males; age, 27-64 years; mean, 45 years), all of whom had negative hepatitis markers and were positive for the immunohistochemical stain homatropine methylbromide-45. The tumours were solitary and well defined, and ranged in size from 1.9 to 9.1 cm (mean, 5.7 cm). On dynamic contrast-enhanced CT, MRI and ultrasound scans, all tumours showed fast strong enhancement in the arterial phase and moderate washout in the portal venous and delayed phases, and the greater portions of the tumours were slightly lower than the surrounding hepatic parenchyma. In some cases, a small area of prolonged or increasing enhancement in the tumour was recognized in the delayed phase. Early draining vessels to the portal vein or hepatic vein could be seen in some cases. However, no capsular signs could be confidently identified in the delayed phase. Haemorrhagic cavities were recognized in two cases, and nodular low-intensity areas in the tumours on T2 weighted imaging that showed slow and faint enhancement on dynamic scans were seen in two cases. However, no necrosis was identified. CONCLUSION Dynamic enhanced imaging studies revealed some specific features of MHAMLs that distinguish them from other hypervascular hepatic tumours, especially when combined with clinical features. Familiarity with imaging and clinical features of MHAMLs could avoid unnecessary surgical resection of these generally benign tumours. ADVANCES IN KNOWLEDGE This article systematically describes the imaging features of MHAMLs.
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Affiliation(s)
- Z Zhu
- Department of Diagnostic Radiology, Cancer Institute and Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing, China
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