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Möller K, Görg C, Krix M, Jenssen C, Dong Y, Cui XW, Dietrich CF. Washout on Contrast-Enhanced Ultrasound of Benign Focal Liver Lesions-A Review on Its Frequency and Possible Causes. Diagnostics (Basel) 2025; 15:998. [PMID: 40310346 PMCID: PMC12025567 DOI: 10.3390/diagnostics15080998] [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: 03/03/2025] [Revised: 04/07/2025] [Accepted: 04/10/2025] [Indexed: 05/02/2025] Open
Abstract
In all imaging methods, including contrast-enhanced ultrasound (CEUS), enhancement in the late phase (LP) is an important criterion for differentiating between benign and malignant focal liver lesions (FLLs). In general, malignant liver lesions are characterized by hypoenhancement and washout in the LP. A lesion with LP hyperenhancement or isoenhancement in the non-cirrhotic liver is usually benign. However, LP hypoenhancement in benign lesions is not so rare, and is even normal and the standard for some lesions, and there are exceptions for each tumor entity that can represent a diagnostic challenge. Knowing these contrast patterns and exceptions is key for correct diagnosis and patient management. The following narrative review describes the contrast behaviors and the frequency of washout and LP hypoenhancement for common as well as rare benign liver lesions and analyzes its causes.
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Affiliation(s)
- Kathleen Möller
- Medical Department I/Gastroenterology, SANA Hospital Lichtenberg, 10365 Berlin, Germany;
| | - Christian Görg
- Interdisciplinary Center of Ultrasound Diagnostics, Gastroenterology, Endocrinology, Metabolism and Clinical Infectiology, University Hospital Giessen and Marburg, Philipp University of Marburg, Baldingerstraße, 35037 Marburg, Germany;
| | - Martin Krix
- Global Medical & Regulatory Affairs, Bracco Imaging, 78467 Konstanz, Germany;
| | - Christian Jenssen
- Department of Internal Medicine, Krankenhaus Märkisch Oderland, 15344 Strausberg, Germany;
- Brandenburg Institute for Clinical Ultrasound (BICUS) at Brandenburg Medical University, 16816 Neuruppin, Germany
| | - Yi Dong
- Department of Ultrasound, Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai 200082, China;
| | - Xin-Wu Cui
- Medical Ultrasound, Tongji Hospital of Tongji Medical College of Huazhong University of Science and Technology, Wuhan 430030, China;
| | - Christoph F. Dietrich
- Department General Internal Medicine (DAIM), Hospitals Hirslanden Bern Beau Site, Salem and Permanence, 3013 Bern, Switzerland
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Amante MF. Hepatic perivascular epithelioid cell tumors: Benign, malignant, and uncertain malignant potential. World J Gastroenterol 2024; 30:2374-2378. [PMID: 38764772 PMCID: PMC11099397 DOI: 10.3748/wjg.v30.i18.2374] [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: 12/24/2023] [Revised: 02/20/2024] [Accepted: 04/25/2024] [Indexed: 05/11/2024] Open
Abstract
In 2013, the World Health Organization defined perivascular epithelioid cell tumor (PEComa) as "a mesenchymal tumor which shows a local association with vessel walls and usually expresses melanocyte and smooth muscle markers." This generic definition seems to better fit the PEComa family, which includes angiomyolipoma, clear cell sugar tumor of the lung, lymphangioleiomyomatosis, and a group of histologically and immunophenotypically similar tumors that include primary extrapulmonary sugar tumor and clear cell myomelanocytic tumor. Clear cell tumors with this immunophenotypic pattern have also had their malignant variants described. When localizing to the liver, preoperative radiological diagnosis has proven to be very difficult, and most patients have been diagnosed with hepatocellular carcinoma, focal nodular hyperplasia, hemangioma, or hepatic adenoma based on imaging findings. Examples of a malignant variant of the liver have been described. Finally, reports of malignant variants of these lesions have increased in recent years. Therefore, we support the use of the Folpe criteria, which in 2005 established the criteria for categorizing a PEComa as benign, malignant, or of uncertain malignant potential. Although they are not considered ideal, they currently seem to be the best approach and could be used for the categorization of liver tumors.
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Affiliation(s)
- Marcelo Fabián Amante
- División Patología, Hospital General de Agudos Cosme Argerich, Buenos Aires C1155AHA, Argentina
- División Patología, Hospital Alemán de Buenos Aires, Buenos Aires C1155AHA, Argentina
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Kvietkauskas M, Samuolyte A, Rackauskas R, Luksaite-Lukste R, Karaliute G, Maskoliunaite V, Valkiuniene RB, Sokolovas V, Strupas K. Primary Liver Perivascular Epithelioid Cell Tumor (PEComa): Case Report and Literature Review. MEDICINA (KAUNAS, LITHUANIA) 2024; 60:409. [PMID: 38541135 PMCID: PMC10972467 DOI: 10.3390/medicina60030409] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Figures] [Subscribe] [Scholar Register] [Received: 01/27/2024] [Revised: 02/15/2024] [Accepted: 02/17/2024] [Indexed: 07/23/2024]
Abstract
A primary liver perivascular epithelioid cell tumor (PEComa) is an extremely rare entity. In this article, we present a case report with a review of the literature on the patients diagnosed with primary liver PEComa and an elaboration of diagnostic and treatment modalities. A systematic literature search was conducted using the terms "perivascular epithelioid cell tumor", "PEComa", "liver", and "hepatic". All articles describing patients diagnosed with primary liver PEComa were included. We identified a total of 224 patients of primary liver PEComa from 75 articles and a case from the present study with a significant preponderance of females (ratio 4:1) and with a mean age of 45.3 ± 12.1 years. Most of the patients (114 out of 224, 50.9%) were asymptomatic. A total of 183 (81.3%) patients underwent surgical hepatic resection at the time of diagnosis, while 19 (8.4%) underwent surveillance. Recurrence and metastases were detected in seven (3.1%) and six (2.7%) patients, respectively. In conclusion, surgical resection remains the cornerstone of therapy; however, the presence of nonspecific imaging features makes it difficult to reach a definite diagnosis preoperatively. Therefore, a multidisciplinary approach should be the gold standard in selecting the treatment modality.
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Affiliation(s)
- Mindaugas Kvietkauskas
- Clinic of Gastroenterology, Nephrourology, and Surgery, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, M. K. Ciurlionio 21, 03101 Vilnius, Lithuania
| | - Austeja Samuolyte
- Clinic of Gastroenterology, Nephrourology, and Surgery, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, M. K. Ciurlionio 21, 03101 Vilnius, Lithuania
| | - Rokas Rackauskas
- Clinic of Gastroenterology, Nephrourology, and Surgery, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, M. K. Ciurlionio 21, 03101 Vilnius, Lithuania
| | - Raminta Luksaite-Lukste
- Department of Radiology, Nuclear Medicine and Medical Physics, Institute of Biomedical Sciences, Faculty of Medicine, Vilnius University, M. K. Ciurlionio 21, 03101 Vilnius, Lithuania
| | - Gintare Karaliute
- Clinic of Gastroenterology, Nephrourology, and Surgery, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, M. K. Ciurlionio 21, 03101 Vilnius, Lithuania
| | - Vygante Maskoliunaite
- Department of Pathology, Forensic Medicine and Pharmacology, Institute of Biomedical Sciences, Faculty of Medicine, Vilnius University, M. K. Ciurlionio 21, 03101 Vilnius, Lithuania
| | - Ruta Barbora Valkiuniene
- Department of Pathology, Forensic Medicine and Pharmacology, Institute of Biomedical Sciences, Faculty of Medicine, Vilnius University, M. K. Ciurlionio 21, 03101 Vilnius, Lithuania
| | - Vitalijus Sokolovas
- Clinic of Gastroenterology, Nephrourology, and Surgery, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, M. K. Ciurlionio 21, 03101 Vilnius, Lithuania
| | - Kestutis Strupas
- Clinic of Gastroenterology, Nephrourology, and Surgery, Institute of Clinical Medicine, Faculty of Medicine, Vilnius University, M. K. Ciurlionio 21, 03101 Vilnius, Lithuania
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Zhu D, Song S, Wang D, Kuang D, Cheng S, Zhou J, Zou S. Hepatic perivascular epithelioid cell tumor resembling hepatic adenoma and hepatocellular carcinoma on preoperative imaging: a case report. Front Oncol 2024; 14:1292313. [PMID: 38361782 PMCID: PMC10867255 DOI: 10.3389/fonc.2024.1292313] [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: 09/26/2023] [Accepted: 01/10/2024] [Indexed: 02/17/2024] Open
Abstract
Perivascular epithelioid cell tumor (PEComa), an uncommon mesenchymal neoplasm, arises from specialized perivascular epithelioid cells exhibiting distinct features of smooth muscle and melanocytic differentiation with unpredictable behavior. PEComa tends to occur more commonly in the uterus and kidneys; its occurrence in the liver is exceedingly rare. We presented a case of a 29-year-old woman with hepatic PEComa and evaluated the tumor with MRI, integrated 18F-fluorodeoxyglucose (FDG), and 68Ga-fibroblast activation protein inhibitor (FAPI) PET/CT scans at presentation. The patient had a history of intermittent utilization of oral contraceptive drugs for several years. An abdominal ultrasound in a physical examination from an outside institution revealed a mass in the liver. A contrast-enhanced abdominal MRI revealed restricted diffusion on diffusion-weighted imaging (DWI) and rapid contrast enhancement and washout patterns in the hepatic lesion, suggesting hepatic adenoma (HA) or hepatocellular carcinoma (HCC). Further assessment was carried out using 18F-FDG and 68Ga-FAPI PET/CT scans. The hepatic lesion was non-FDG avid, whereas increased tracer uptake was observed on the 68Ga-FAPI PET/CT. Subsequently, laparoscopic partial resection of liver segment V was performed. Immunohistochemical analyses demonstrated positive staining for HMB45, Melan-A, and SMA while showing negative results for AFP, glypican-3, hepatocyte, and arginase-1. The results were indicative of a hepatic PEComa diagnosis based on these findings. We also review the current literature on the clinical characteristics, pathological features, and challenges in the diagnosis of hepatic PEComa.
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Affiliation(s)
- Dongling Zhu
- Department of Nuclear Medicine and PET, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Shuang Song
- Department of Nuclear Medicine and PET, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Dongdong Wang
- Department of Nuclear Medicine and PET, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Dong Kuang
- Department of Pathology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Siyuan Cheng
- Department of Nuclear Medicine and PET, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Jianyuan Zhou
- Department of Nuclear Medicine and PET, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
| | - Sijuan Zou
- Department of Nuclear Medicine and PET, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
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Matrood S, Görg C, Safai Zadeh E, Alhyari A. Hepatic perivascular epithelioid cell tumor (PEComa): contrast-enhanced ultrasound (CEUS) characteristics-a case report and literature review. Clin J Gastroenterol 2023; 16:444-449. [PMID: 36964879 DOI: 10.1007/s12328-023-01779-w] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/26/2022] [Accepted: 02/23/2023] [Indexed: 03/26/2023]
Abstract
Perivascular epithelioid cell tumors (PEComa) are rare mesenchymal neoplasms that arise from soft tissue of various organs such as the stomach, intestines, and lungs. We report a rare case of a primary PEComa of the liver and its characteristics on contrast-enhanced ultrasound (CEUS) in a 51-year-old female patient with an incidental finding of a hypoechoic liver lesion with peripheral hypervascularization on Doppler ultrasound. CEUS showed homogenous hypervascularity in the arterial phase that was consistent in the portal phase. In the late phase, a central washout phenomenon was evident. Histopathologic findings on sonographic biopsy of the lesion revealed a mesenchymal tumor with positivity for melanocytic markers Human Melanin Black-45 (HMB45) and Melan-A consistent with a PEComa. Despite the absence of high-risk features for malignancy, surgical resection was recommended due to the uncertain malignant potential of PEComas. The patient refused the operation and preferred sonographic follow-up; the lesion was stable over a period of 2 years. CEUS can provide valuable information regarding PEComa. After histological confirmation, the choice between resection and a watchful waiting must be made on individual basis.
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Affiliation(s)
- Sami Matrood
- Department of Gastroenterology, Endocrinology, Metabolism and Clinical Infectiology, University Hospital Marburg, Philipps-University Marburg, Marburg, Germany
| | - Christian Görg
- Department of Gastroenterology, Endocrinology, Metabolism and Clinical Infectiology, University Hospital Marburg, Philipps-University Marburg, Marburg, Germany
- Interdisciplinary Centre of Ultrasound Diagnostics, University Hospital Marburg, Philipps-University Marburg, University Hospital Giessen and Marburg, Baldingerstraße, 35042, Marburg, Germany
| | - Ehsan Safai Zadeh
- Department of Gastroenterology, Endocrinology, Metabolism and Clinical Infectiology, University Hospital Marburg, Philipps-University Marburg, Marburg, Germany
- Interdisciplinary Centre of Ultrasound Diagnostics, University Hospital Marburg, Philipps-University Marburg, University Hospital Giessen and Marburg, Baldingerstraße, 35042, Marburg, Germany
| | - Amjad Alhyari
- Department of Gastroenterology, Endocrinology, Metabolism and Clinical Infectiology, University Hospital Marburg, Philipps-University Marburg, Marburg, Germany.
- Interdisciplinary Centre of Ultrasound Diagnostics, University Hospital Marburg, Philipps-University Marburg, University Hospital Giessen and Marburg, Baldingerstraße, 35042, Marburg, Germany.
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Li Z, Zhou Y, Wang C, Yu H, Yang G, Ma Y. Insights into clinical diagnosis and treatment of malignant hepatic perivascular epithelioid cell tumor. Intractable Rare Dis Res 2022; 11:202-205. [PMID: 36457582 PMCID: PMC9709624 DOI: 10.5582/irdr.2022.01111] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/20/2022] [Revised: 11/18/2022] [Accepted: 11/21/2022] [Indexed: 11/25/2022] Open
Abstract
Perivascular epithelioid cell tumors (PEComas) are infrequent mesenchymal tumors. They are usually benign, and only a few are malignant. These tumors are more commonly found in middle-aged women. PEComas are mainly composed of differentiated perivascular epithelioid cells arranged radially around the vascular cavity, and they are usually positive for melanocyte markers and smooth muscle cell differentiation markers. Among the PEComas, hepatic PEComas generally have no obvious symptoms and no typical imaging manifestations. Malignant hepatic PEComas are even rarer. So, we explained our insights into clinical diagnosis and treatment of malignant hepatic PEComas, in order to help clinicians and pathologists to further understand PEComas.
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Affiliation(s)
- Zhongyu Li
- Key Laboratory of Hepatosplenic Surgery, Ministry of Education, Harbin, Heilongjiang, China
- Department of Minimal Invasive Hepatic Surgery, The First Affiliated Hospital of Harbin Medical University, Harbin, Heilongjiang, China
| | - Yongzhi Zhou
- Key Laboratory of Hepatosplenic Surgery, Ministry of Education, Harbin, Heilongjiang, China
- Department of Minimal Invasive Hepatic Surgery, The First Affiliated Hospital of Harbin Medical University, Harbin, Heilongjiang, China
| | - Chaoqun Wang
- Key Laboratory of Hepatosplenic Surgery, Ministry of Education, Harbin, Heilongjiang, China
- Department of Minimal Invasive Hepatic Surgery, The First Affiliated Hospital of Harbin Medical University, Harbin, Heilongjiang, China
| | - Hongjun Yu
- Key Laboratory of Hepatosplenic Surgery, Ministry of Education, Harbin, Heilongjiang, China
- Department of Minimal Invasive Hepatic Surgery, The First Affiliated Hospital of Harbin Medical University, Harbin, Heilongjiang, China
| | - Guangchao Yang
- Key Laboratory of Hepatosplenic Surgery, Ministry of Education, Harbin, Heilongjiang, China
- Department of Minimal Invasive Hepatic Surgery, The First Affiliated Hospital of Harbin Medical University, Harbin, Heilongjiang, China
| | - Yong Ma
- Key Laboratory of Hepatosplenic Surgery, Ministry of Education, Harbin, Heilongjiang, China
- Department of Minimal Invasive Hepatic Surgery, The First Affiliated Hospital of Harbin Medical University, Harbin, Heilongjiang, China
- Address correspondence to:Yong Ma, Department of Minimal Invasive Hepatic Surgery, The First Affiliated Hospital of Harbin Medical University, No. 23 Post Street, Nangang District, Harbin 150001, Heilongjiang, China. E-mail:
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Gao X, Tang H, Wang J, Yao Q, Wang H, Wang Y, Ma M, Yang W, Yan K, Wu W. Specific imaging features indicate the clinical features of patients with hepatic perivascular epithelioid cell tumor by comparative analysis of CT and ultrasound imaging. Front Oncol 2022; 12:908189. [PMID: 36324566 PMCID: PMC9618795 DOI: 10.3389/fonc.2022.908189] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/31/2022] [Accepted: 09/29/2022] [Indexed: 11/24/2022] Open
Abstract
Objective The objective of the study was to explore the CT and ultrasound features and clinical significance of perivascular epithelioid cell tumor (PEComa) of the liver. Methods Eleven hepatic PEComa patients treated in our hospital were retrospectively analyzed based on the characteristics of the imaging results of the patients, including conventional ultrasound, CDFI, contrast-enhanced ultrasound (CEUS), and contrast-enhanced CT (CECT). Results CT scans showed that all lesions were hypodense. Ultrasonography showed that lesions were either hyperechoic (4/11, 36.36%), hypoechoic (4/11, 36.36%), isoechoic (1/11, 9.09%), or heterogeneously echoic (2/11, 18.18%). CDFI showed that most of the lesions had an abundant blood supply (9/11, 81.82%). Whether on CT scan or ultrasonography, the margins of the lesions were dominated by clear margins. Ultrasonography revealed more features: hyperechoic patterns around lesions (3/11, 27.27%) and lateral shadow (5/11, 45.45%). The CDFI showed that large blood vessels were observed around the lesions (9/11, 81.82%). CECT shows two enhancement patterns: “fast in and fast out (FIFO)” (8/11, 72.72%) and “fast in and slow out (FISO)” (3/11, 27.27%). CEUS shows that all lesions had the enhancement pattern of “FISO,” which was different from CECT. All lesions displayed rapid enhancement during HAP in CEUS during 7–20 s. Four patients (36.36%) washed out at 60–180 s, another four (36.36%) washed out at 180–300 s, and the remaining three patients (27.27%) showed no signs of washout even at 360 s. Conclusion Some imaging features, such as clear margins, peripheral hyperechoic around the lesion, lateral shadow, the large blood vessels around lesions, and the “FISO” enhancement pattern, may indicate expansive growth of the tumor and be helpful in the diagnosis of PEComa. Ultrasound images may provide more details for clinical reference.
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Affiliation(s)
- Xudong Gao
- Department of Ultrasound, Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education), Peking University Cancer Hospital and Institute, Beijing, China
- Hepatology Department and Emergency Department, The 5th Medical Center of PLA General Hospital, Beijing, China
| | - Hewen Tang
- Department of Ultrasound, Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education), Peking University Cancer Hospital and Institute, Beijing, China
| | - Jianying Wang
- Department of Information, Medical Supplies Center of PLA General Hospital, Beijing, China
| | - Qian Yao
- 4Department of Pathology, Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education), Peking University Cancer Hospital and Institute, Beijing, China
| | - Hong Wang
- Department of Ultrasound, Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education), Peking University Cancer Hospital and Institute, Beijing, China
| | - Yan Wang
- Hepatology Department and Emergency Department, The 5th Medical Center of PLA General Hospital, Beijing, China
| | - Mingming Ma
- Hepatology Department and Emergency Department, The 5th Medical Center of PLA General Hospital, Beijing, China
| | - Wei Yang
- Department of Ultrasound, Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education), Peking University Cancer Hospital and Institute, Beijing, China
| | - Kun Yan
- Department of Ultrasound, Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education), Peking University Cancer Hospital and Institute, Beijing, China
- *Correspondence: Wei Wu, ; Kun Yan,
| | - Wei Wu
- Department of Ultrasound, Key Laboratory of Carcinogenesis and Translational Research (Ministry of Education), Peking University Cancer Hospital and Institute, Beijing, China
- *Correspondence: Wei Wu, ; Kun Yan,
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Zhang X, Zhong X, Lin X, Li X, Tian H, Chang B, Wang Y, Tong J, Wang N, Li D, Jin X, Huang D, Wang Y, Cui H, Guan L, Li Y. Tuberous Sclerosis Complex With Multiple Organ Tumors: Case Report and Literature Review. Front Oncol 2022; 12:916016. [PMID: 35928867 PMCID: PMC9343591 DOI: 10.3389/fonc.2022.916016] [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: 04/08/2022] [Accepted: 06/21/2022] [Indexed: 11/13/2022] Open
Abstract
Pancreatic neuroendocrine neoplasms (PNEN) are tumors that originate from neuroendocrine cells. Only about 1% patients are related to mutation of tuberous sclerosis complex gene. Here, we reported a rare case with involvement of multiple organs and space-occupying lesions. Initially, the patient was thought to have metastasis of a pancreatic tumor. However, the patient was diagnosed as pancreatic neuroendocrine tumors, liver perivascular epithelioid tumors, splenic hamartoma, and renal angiomyolipoma by pathological examination after surgery. We performed genetic mutation detection to identify that tuberous sclerosis complex 2 gene presented with a heterozygous variant. Tuberous sclerosis often presents with widespread tumors, but it is less common to present with pancreatic neuroendocrine tumors and liver perivascular tumors as highlighted in the case. So we analyzed the relationship between TSC gene mutations and related tumors. And we also reviewed the current molecular mechanisms and treatments for tuberous sclerosis complex.
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Affiliation(s)
- Xinhe Zhang
- Gastroenterology Department, The First Affiliated Hospital of China Medical University, Shenyang, China
| | - Xinping Zhong
- Department of Hepatobiliary Surgery, The First Affiliated Hospital of China Medical University, Shenyang, China
| | - Xuyong Lin
- Department of Pathology, The First Affiliated Hospital of China Medical University, Shenyang, China
| | - Xuedan Li
- Radiology Department, The First Affiliated Hospital of China Medical University, Shenyang, China
| | - Haoyu Tian
- The 3rd Clinical Department, China Medical University, Shenyang, China
| | - Bing Chang
- Gastroenterology Department, The First Affiliated Hospital of China Medical University, Shenyang, China
| | - Ying Wang
- Gastroenterology Department, The First Affiliated Hospital of China Medical University, Shenyang, China
| | - Jing Tong
- Gastroenterology Department, The First Affiliated Hospital of China Medical University, Shenyang, China
| | - Ningning Wang
- Gastroenterology Department, The First Affiliated Hospital of China Medical University, Shenyang, China
| | - Dan Li
- Gastroenterology Department, The First Affiliated Hospital of China Medical University, Shenyang, China
| | - Xiuli Jin
- Gastroenterology Department, The First Affiliated Hospital of China Medical University, Shenyang, China
| | - Die Huang
- Gastroenterology Department, The First Affiliated Hospital of China Medical University, Shenyang, China
| | - Yanmeng Wang
- Gastroenterology Department, The First Affiliated Hospital of China Medical University, Shenyang, China
| | - Huipeng Cui
- Gastroenterology Department, The First Affiliated Hospital of China Medical University, Shenyang, China
| | - Lin Guan
- Gastroenterology Department, The First Affiliated Hospital of China Medical University, Shenyang, China
- *Correspondence: Yiling Li, ; Lin Guan,
| | - Yiling Li
- Gastroenterology Department, The First Affiliated Hospital of China Medical University, Shenyang, China
- *Correspondence: Yiling Li, ; Lin Guan,
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Luo L, Wang T, Cheng M, Ge X, Song S, Zhu G, Xiao Y, Deng W, Xie J, Shan R. Rare benign liver tumors that require differentiation from hepatocellular carcinoma: focus on diagnosis and treatment. J Cancer Res Clin Oncol 2022:10.1007/s00432-022-04169-w. [PMID: 35789428 DOI: 10.1007/s00432-022-04169-w] [Citation(s) in RCA: 3] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/14/2022] [Accepted: 06/21/2022] [Indexed: 11/24/2022]
Abstract
BACKGROUND/AIM Recently, an increase in the number of asymptomatic rare benign liver tumors (BLTs) has been reported during health check-ups. It is difficult to determine the nature of partial rare BLTs and not easy to distinguish from malignant liver tumors. This study aimed to analysis clinical features, diagnosis and treatment of rare BLTs to reduce misdiagnosis and provide reference for clinical practice. METHODS From January 2012 to January 2021, we treated 112 rare BLTs by hepatectomy, including 54 focal nodular hyperplasias, 14 hepatocellular adenomas, 28 hepatic angiomyolipomas, 3 hepatic granulomas, 2 inflammatory pseudotumors of the liver, 2 nodular regenerative hyperplasia, 2 hepatic lipomas, 1 solitary fibrous tumor of the liver, 1 hepatic schwannoma and 1 hepatic myelolipoma. RESULTS The majority of patients were middle-aged female and asymptomatic. Single tumors were dominant. The diagnostic accuracies of computed tomography (CT) and magnetic resonance imaging (MRI) were 32.5% and 44.2%, respectively. The majority of tumors were likely to be misdiagnosed as hepatocellular carcinoma (HCC) or difficult to distinguish from HCC. All patients underwent surgical treatment. Postoperative pathological and immunohistochemical examination can confirm the diagnosis. No patients without tumor recurrence or metastasis during follow-up period. CONCLUSION Altogether, the clinical symptoms of rare BLTs lack specificity, and their preoperative diagnosis largely depends on imaging examination, with a low diagnostic accuracy rate and high chances of misdiagnosis as HCC. Diagnosis is confirmed by pathological and immunohistochemical examination. Surgical resection for rare BLT is safe and effective, regular postoperative follow-up is necessary.
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Affiliation(s)
- Laihui Luo
- Department of General Surgery, First Affiliated Hospital of Nanchang University, 17 Yong Wai Zheng Street, Nanchang, 330006, Jiangxi, China
| | - Tao Wang
- Department of Day Surgery Ward, First Affiliated Hospital of Nanchang University, 17 Yong Wai Zheng Street, Nanchang, 330006, Jiangxi, China
| | - Mengting Cheng
- Department of General Surgery, First Affiliated Hospital of Nanchang University, 17 Yong Wai Zheng Street, Nanchang, 330006, Jiangxi, China
| | - Xian Ge
- Department of Pathology, First Affiliated Hospital of Nanchang University, 17 Yong Wai Zheng Street, Nanchang, 330006, Jiangxi, China
| | - Shengjiang Song
- Department of General Surgery, First Affiliated Hospital of Nanchang University, 17 Yong Wai Zheng Street, Nanchang, 330006, Jiangxi, China
| | - Guoqing Zhu
- Department of General Surgery, First Affiliated Hospital of Nanchang University, 17 Yong Wai Zheng Street, Nanchang, 330006, Jiangxi, China
| | - Yongqiang Xiao
- Department of General Surgery, First Affiliated Hospital of Nanchang University, 17 Yong Wai Zheng Street, Nanchang, 330006, Jiangxi, China
| | - Wei Deng
- Department of General Surgery, First Affiliated Hospital of Nanchang University, 17 Yong Wai Zheng Street, Nanchang, 330006, Jiangxi, China
| | - Jin Xie
- Department of General Surgery, First Affiliated Hospital of Nanchang University, 17 Yong Wai Zheng Street, Nanchang, 330006, Jiangxi, China
| | - Renfeng Shan
- Department of General Surgery, First Affiliated Hospital of Nanchang University, 17 Yong Wai Zheng Street, Nanchang, 330006, Jiangxi, 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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11
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Malignant perivascular epithelioid cell neoplasm in the liver: report of a pediatric case. Surg Case Rep 2021; 7:212. [PMID: 34542724 PMCID: PMC8452810 DOI: 10.1186/s40792-021-01300-w] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/31/2021] [Accepted: 09/13/2021] [Indexed: 11/29/2022] Open
Abstract
Background Perivascular epithelioid cell neoplasm (PEComa) in a child is very rare. We herein report the first malignant case of PEComa developing in the liver of a pediatric patient.
Case presentation A 10-year-old boy visited a private clinic with prolonged fever of unknown etiology. Abdominal ultrasonography was performed to evaluate the fever’s origin, revealing a large tumor in the liver. He was thus referred to a nearby hospital to investigate the tumor further. Enhanced computed tomography (CT) showed a 6.8 × 5.9 × 10.5-cm solid lesion on S4 and S5. On magnetic resonance imaging (MRI), the tumor had a low signal intensity on T1 imaging and high signal intensity on T2 imaging, with partial diffusion restriction. 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET) showed a marked uptake in the mass lesion with no evidence of metastasis. The patient was negative for all tumor markers, including AFP, CEA and PIVKA-II. The results of a needle biopsy suggested hepatocellular carcinoma. The tumor’s rapid growth suggested malignancy. Hepatic segmentectomy (S4 + S5 + S8) was performed. The tumor was resected en bloc with a margin. Microscopically, the tumor showed atypical spindle, polygonal or oval-shaped cells with a high nuclear grade, and vascular invasion. Immunohistochemistry was positive for alpha-smooth muscle antigen (α-SMA), human melanin black-45 (HMB-45) and melan A. The pathological diagnosis was malignant PEComa. In the 6 months after surgery, the patient complained of shoulder pain. MRI showed a dumbbell-shaped tumor at the 2nd thoracic vertebrae, which was confirmed to be bone metastasis of PEComa. After chemotherapy, including ifosfamide and doxorubicin, vertebrectomy was performed. Two years later, thoracoabdominal CT showed a 10-cm solid mass occupying the pelvis and a 15-mm nodule in the middle lobe of the right lung. Under a diagnosis of peritoneal and lung metastases, they were surgically removed and metastasis of PEComa was pathologically confirmed. Four months after the 2nd relapse, pelvic metastasis appeared again and mTOR (mammalian target of rapamycin) inhibitor was initiated. To our knowledge, this is the first report of malignant hepatic PEComa in a pediatric patient. Conclusion Although extremely rare, malignant hepatic PEComa can develop in a child.
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Krawczyk M, Ziarkiewicz-Wróblewska B, Wróblewski T, Podgórska J, Grzybowski J, Gierej B, Krawczyk P, Nyckowski P, Kornasiewicz O, Patkowski W, Remiszewski P, Zając K, Grąt M. PEComa-A Rare Liver Tumor. J Clin Med 2021; 10:1756. [PMID: 33919494 PMCID: PMC8072725 DOI: 10.3390/jcm10081756] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/16/2021] [Revised: 04/09/2021] [Accepted: 04/14/2021] [Indexed: 12/16/2022] Open
Abstract
PEComa (perivascular epithelioid cell tumor) is a rare liver tumor. Decisions regarding patient management are currently based on a few small case series. The aim of this study was to report the clinicopathological features of PEComa in order to provide guidance for management, complemented by our own experience. This retrospective observational study included all patients with PEComa who underwent surgical treatment in two departments between 2002 and 2020. A total of 20 patients were diagnosed with PEComa following histopathological examination. The age of the patients ranged from 21 to 73 years. The majority of patients were women (85%). In most patients, the tumors were incidental. In diagnostic studies, PEComas with high arterial vascularization have been described. Liver resection was the treatment of choice. There was only one postoperative complication. During histopathological evaluation, tumors were composed mostly of epithelioid cells, rarely with spindle cell components, thick-walled vessels, and adipocytes in different proportions. Melanocytic markers (HMB45, MelanA) and at least one smooth muscle marker were expressed in all tumors. Features suggestive of malignancy were found in three cases. In conclusion, PEComa is a rare liver tumor that is usually diagnosed incidentally. In radiological studies, tumors with high arterial vascularization are observed. Liver resection is the treatment of choice.
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Affiliation(s)
- Marek Krawczyk
- Department of General, Transplant and Liver Surgery, Medical University Warsaw, 02-097 Warsaw, Poland; (M.K.); (T.W.); (P.K.); (O.K.); (W.P.); (P.R.); (K.Z.)
| | | | - Tadeusz Wróblewski
- Department of General, Transplant and Liver Surgery, Medical University Warsaw, 02-097 Warsaw, Poland; (M.K.); (T.W.); (P.K.); (O.K.); (W.P.); (P.R.); (K.Z.)
| | - Joanna Podgórska
- 2nd Department of Clinical Radiology, Medical University of Warsaw, 02-097 Warsaw, Poland;
| | - Jakub Grzybowski
- Department of Pathology, Medical University of Warsaw, 02-097 Warsaw, Poland; (B.Z.-W.); (J.G.); (B.G.)
| | - Beata Gierej
- Department of Pathology, Medical University of Warsaw, 02-097 Warsaw, Poland; (B.Z.-W.); (J.G.); (B.G.)
| | - Piotr Krawczyk
- Department of General, Transplant and Liver Surgery, Medical University Warsaw, 02-097 Warsaw, Poland; (M.K.); (T.W.); (P.K.); (O.K.); (W.P.); (P.R.); (K.Z.)
| | - Paweł Nyckowski
- Department of General, Gastroenterological and Oncological Surgery, Medical University Warsaw, 02-097 Warsaw, Poland;
| | - Oskar Kornasiewicz
- Department of General, Transplant and Liver Surgery, Medical University Warsaw, 02-097 Warsaw, Poland; (M.K.); (T.W.); (P.K.); (O.K.); (W.P.); (P.R.); (K.Z.)
| | - Waldemar Patkowski
- Department of General, Transplant and Liver Surgery, Medical University Warsaw, 02-097 Warsaw, Poland; (M.K.); (T.W.); (P.K.); (O.K.); (W.P.); (P.R.); (K.Z.)
| | - Piotr Remiszewski
- Department of General, Transplant and Liver Surgery, Medical University Warsaw, 02-097 Warsaw, Poland; (M.K.); (T.W.); (P.K.); (O.K.); (W.P.); (P.R.); (K.Z.)
| | - Krzysztof Zając
- Department of General, Transplant and Liver Surgery, Medical University Warsaw, 02-097 Warsaw, Poland; (M.K.); (T.W.); (P.K.); (O.K.); (W.P.); (P.R.); (K.Z.)
| | - Michał Grąt
- Department of General, Transplant and Liver Surgery, Medical University Warsaw, 02-097 Warsaw, Poland; (M.K.); (T.W.); (P.K.); (O.K.); (W.P.); (P.R.); (K.Z.)
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Janks M, Heaford A, Deheragoda M, Hadzic ND. Hepatic perivascular epithelioid cell tumours in children with tuberous sclerosis. BMJ Case Rep 2020; 13:13/12/e236288. [PMID: 33298476 DOI: 10.1136/bcr-2020-236288] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/03/2022] Open
Abstract
Perivascular epithelioid cell tumours are a novel histological description of mesenchymal tumours consisting of perivascular and epithelioid cells. Angiomyolipomas are one of the commoner types of this tumour group. They are typically associated with the inherited condition tuberous sclerosis (TS). In TS they are often seen arising in the kidneys and brain, although much more rarely can be seen in the liver. While usually thought of as benign tumours there is little evidence to predict whether they will progress to malignancy. Currently, there is no recommended best management strategy between resection and surveillance. We report two patients with TS seen in our centre with these described PEComa liver lesions histologically, however only one required a resection. On review of the literature, features such as increased size, rapid growth and vascular invasion would be concerning for possible malignant potential and therefore merit resection, as well as significant symptoms.
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Affiliation(s)
- Michaela Janks
- Paediatric Liver Medicine Department, King's College Hospital, London, UK
| | - Amelia Heaford
- Liver Histopathology Laboratory, Institute of Liver Studies, King's College Hospital, London, UK
| | - Maesha Deheragoda
- Liver Histopathology Laboratory, Institute of Liver Studies, King's College Hospital, London, UK
| | - Nedim Dino Hadzic
- Paediatric Liver Medicine Department, King's College Hospital, London, UK
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14
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Galera López MDM, Márquez Rodas I, Agra Pujol C, García Pérez Á, Velasco Sánchez E, Álvarez Álvarez R. Simultaneous diagnosis of liver PEComa in a family with known Li-Fraumeni syndrome: a case report. Clin Sarcoma Res 2020; 10:24. [PMID: 33292569 PMCID: PMC7684891 DOI: 10.1186/s13569-020-00143-7] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/12/2020] [Accepted: 11/03/2020] [Indexed: 11/23/2022] Open
Abstract
Background Li–Fraumeni syndrome (LFS) is an autosomal dominant hereditary disease. It is associated with the loss of function of the p53 protein and an increased risk of malignant tumor development at early age. The most frequently detected tumors include breast cancer, sarcomas, leukemia, brain tumors, and adrenocortical carcinomas. While sarcomas account for only 1% of solid tumors, they are more frequently detected in these families. Case presentation We report a simultaneous diagnosis of hepatic perivascular epithelioid cell tumor (PEComa), a very rare subtype of sarcoma, in two siblings with a LFS. Conclusions The simultaneous diagnosis of PEComa in two siblings presented in this case allowed us to review the frequency of PEComa in this genetic syndrome previously reported, which was very little. Despite its rarity, PEComa must be considered in the differential diagnosis of new-onset liver lesions in patients who were previously diagnosed with LFS.
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Affiliation(s)
- María Del Mar Galera López
- Department of Medical Oncology, Hospital General Universitario Gregorio Marañón, Calle Dr. Esquerdo, 46, 28007, Madrid, Spain.
| | - Iván Márquez Rodas
- Department of Medical Oncology, Hospital General Universitario Gregorio Marañón, Calle Dr. Esquerdo, 46, 28007, Madrid, Spain.,CIBERONC, Madrid, Spain
| | - Carolina Agra Pujol
- Department of Pathology, Hospital General Universitario Gregorio Marañón, Madrid, Spain
| | - Ángela García Pérez
- Department of Radiology, Hospital General Universitario Gregorio Marañón, Madrid, Spain
| | - Enrique Velasco Sánchez
- Department of General Surgery, Hospital General Universitario Gregorio Marañón, Madrid, Spain
| | - Rosa Álvarez Álvarez
- Department of Medical Oncology, Hospital General Universitario Gregorio Marañón, Calle Dr. Esquerdo, 46, 28007, Madrid, Spain
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15
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Razik A, Malla S, Goyal A, Gamanagatti S, Kandasamy D, Das CJ, Sharma R, Gupta AK. Unusual Primary Neoplasms of the Adult Liver: Review of Imaging Appearances and Differential Diagnosis. Curr Probl Diagn Radiol 2020; 51:73-85. [PMID: 33199074 DOI: 10.1067/j.cpradiol.2020.10.001] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/16/2020] [Accepted: 10/16/2020] [Indexed: 12/24/2022]
Abstract
The radiological appearance of common primary hepatic tumors such as hepatocellular carcinoma (HCC) and cholangiocarcinoma (CCA) is widely recognized. Hepatic masses with unusual histology are occasionally encountered, but seldom suspected on imaging. However, many possess characteristic imaging findings, which when assessed along with the clinical and demographic background and serum tumor markers, may enable a prospective diagnosis. This review attempts to familiarize the reader with the clinicopathological characteristics, imaging manifestations, and differential diagnosis of these unusual liver tumors in adults. Biphenotypic primary liver carcinoma is suspected in masses showing distinct areas of HCC and CCA-type enhancement pattern in cirrhotic livers. Fibrolamellar carcinoma occurs in young individuals without underlying chronic liver disease and shows a characteristic T2-hypointense scar frequently showing calcification. Perivascular epithelioid cell tumors are differentials for any arterial hyperenhancing mass in the noncirrhotic liver, particularly in patients with tuberous sclerosis. Multifocal subcapsular tumors showing target-like morphology, capsular retraction and "lollipop" sign are suspicious for epithelioid hemangioendothelioma. On the other hand, multiple hemorrhagic lesions showing patchy areas of bizarre-shaped arterial phase hyperenhancement are suspicious for angiosarcoma. Primary hepatic lymphoma (PHL) is suspected when patients with immunosuppression present with solitary or multifocal masses that insinuate around vessels and bile ducts without causing luminal narrowing. Intense diffusion restriction and low-level homogeneous or target-like enhancement are also ancillary features of PHL. Primary hepatic neuroendocrine tumor shows uptake on Ga-68 DOTANOC PET/CT. Although a straightforward diagnosis may be difficult in these cases, awareness of the characteristic imaging appearances is helpful in suspecting the diagnosis.
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Affiliation(s)
- Abdul Razik
- Department of Radiodiagnosis, All India Institute of Medical Sciences (A.I.I.M.S), New Delhi, India
| | - Sundeep Malla
- Department of Radiodiagnosis, All India Institute of Medical Sciences (A.I.I.M.S), New Delhi, India
| | - Ankur Goyal
- Department of Radiodiagnosis, All India Institute of Medical Sciences (A.I.I.M.S), New Delhi, India
| | - Shivanand Gamanagatti
- Department of Radiodiagnosis, All India Institute of Medical Sciences (A.I.I.M.S), New Delhi, India
| | | | - Chandan Jyoti Das
- Department of Radiodiagnosis, All India Institute of Medical Sciences (A.I.I.M.S), New Delhi, India
| | - Raju Sharma
- Department of Radiodiagnosis, All India Institute of Medical Sciences (A.I.I.M.S), New Delhi, India.
| | - Arun Kumar Gupta
- Department of Radiodiagnosis, All India Institute of Medical Sciences (A.I.I.M.S), New Delhi, India
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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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Huang Z, Wu X, Li S, Li K. Contrast-Enhanced Ultrasound Findings and Differential Diagnosis of Hepatic Epithelioid Angiomyolipoma Compared with Hepatocellular Carcinoma. ULTRASOUND IN MEDICINE & BIOLOGY 2020; 46:1403-1411. [PMID: 32165015 DOI: 10.1016/j.ultrasmedbio.2020.02.001] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 05/02/2019] [Revised: 01/12/2020] [Accepted: 02/03/2020] [Indexed: 06/10/2023]
Abstract
Hepatic epithelioid angiomyolipoma (EAML) has malignant potential that can easily be misdiagnosed as hepatocellular carcinoma (HCC), but the treatment options of these conditions are different. This retrospective study investigated whether contrast-enhanced ultrasound (CEUS) can differentiate EAML from HCC. We included 24 patients with pathologically confirmed EAML and 36 patients with HCC who underwent pre-operative conventional ultrasound and CEUS. The conventional ultrasound and CEUS features of the two groups were analyzed. Time intensity curves (TICs) were analyzed for all lesions using quantitative perfusion analysis software. This study found that significant differences existed between the EAML and HCC groups in terms of clinical features such as sex, age, symptoms, alpha-fetoprotein and history of hepatitis B virus infection (p < 0.05). Tumor size, number, border and echogenicity on conventional ultrasound were significantly different between EAML and HCC (p < 0.05). Regarding CEUS, EAML was more likely to have a centripetal enhancement pattern than HCC (p < 0.05). The tumor enhancement degree in the delayed phase in CEUS was significantly different between EAML and HCC (p < 0.05). Quantitative analysis of CEUS parameters revealed that the wash-in area under the curve (AUC) was larger in EAML than in HCC (p < 0.05). Time to peak was significantly shorter in EAML than in HCC (p < 0.05). The peak intensity was significantly stronger in EAML than in HCC (p < 0.05). Regarding the diagnostic performance of CEUS parameters that showed statistical significance on univariate analysis, the sensitivity and specificity for distinguishing EAML from HCC was 66.7%-100.0% and 55.6%-91.7%, respectively. Overall, CEUS could be valuable in differentiating EAML from HCC. Specific features such as the centripetal filling and prolonged enhancement patterns on CEUS, higher peak intensity, bigger wash-in AUC and shorter time to peak on TICs may contribute to a more confirmative differential diagnosis of EAML.
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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
| | - XiaoBei Wu
- 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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18
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Machado I, Marhuenda A, Trallero M, Caballero M, Santos J, Cruz J, Estevan R. [Hepatic epithelioid angiomyolipoma/PEComa and focal nodular hyperplasia in a patient with a previous history of cutaneous melanoma]. REVISTA ESPANOLA DE PATOLOGIA : PUBLICACION OFICIAL DE LA SOCIEDAD ESPANOLA DE ANATOMIA PATOLOGICA Y DE LA SOCIEDAD ESPANOLA DE CITOLOGIA 2019; 52:250-255. [PMID: 31530409 DOI: 10.1016/j.patol.2018.05.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 03/26/2018] [Revised: 05/06/2018] [Accepted: 05/11/2018] [Indexed: 02/07/2023]
Abstract
Hepatic perivascular epithelioid cell tumors (PEComas) are uncommon mesenchymal neoplasms. PEComas concurrent with other hepatic lesions is a very rare occurrence, with only two previously reported cases. We report a primary hepatic PEComa associated with focal nodular hyperplasia in a patient with a previous history of cutaneous melanoma. Diagnostic imaging studies suggested a hepatic adenoma and the patient underwent a segmentectomy. The tumor was mainly composed of epithelioid cells, adipose tissue and smooth muscle fibers intermixed with blood vessels. The neoplastic cells were diffusely immunoreactive for HMB-45, Melan-A and smooth muscle actin, but not for Hepatocyte, S100, MITF or BRAF. Molecular studies were negative for BRAFV600 mutation. The final diagnosis was hepatic epithelioid angiomyolipoma/PEComa. The differential diagnosis of hepatic PEComa is discussed.
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Affiliation(s)
- Isidro Machado
- Departamento de Patología, Instituto Valenciano de Oncología, Valencia, España.
| | - Ana Marhuenda
- Departamento de Radiología, Instituto Valenciano de Oncología, Valencia, España
| | - Marta Trallero
- Servicio de Cirugía, Instituto Valenciano de Oncología, Valencia, España
| | - María Caballero
- Servicio de Cirugía, Instituto Valenciano de Oncología, Valencia, España
| | - Jesús Santos
- Departamento de Radiología, Instituto Valenciano de Oncología, Valencia, España
| | - Julia Cruz
- Departamento de Patología, Instituto Valenciano de Oncología, Valencia, España
| | - Rafael Estevan
- Servicio de Cirugía, Instituto Valenciano de Oncología, Valencia, España
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19
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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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Nie P, Wu J, Wang H, Zhou R, Sun L, Chen J, Yang G. Primary hepatic perivascular epithelioid cell tumors: imaging findings with histopathological correlation. Cancer Imaging 2019; 19:32. [PMID: 31171030 PMCID: PMC6555711 DOI: 10.1186/s40644-019-0212-x] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/15/2019] [Accepted: 05/13/2019] [Indexed: 12/21/2022] Open
Abstract
Background Hepatic PEComas are very rare. Few systematic reports are available characterizing the imaging and pathological features of hepatic PEComa. The aim of this study was to investigate the imaging findings of primary hepatic perivascular epithelioid cell tumors (PEComa) and its correlation with histopathological features. Methods The CT, MRI and ultrasound images and pathological findings of 22 patients with primary hepatic PEComa were retrospectively reviewed. Results More females (14/22) were affected with the mean age of 47.1 years. Most patients (17/22) were asymptomatic and the routine laboratory tests were normal. More tumors occurred in the right lobe (13/22) with a mean diameter of 76.7 mm. Surgery was performed in 21 patients, and biopsy was performed in 1 patient. Immunohistochemical studies showed the expression rate of HMB-45 and Melan A was 100% (22/22) and 86.4% (19/22) within the tumor cells. The pathology diagnoses were angiomyolipoma (n = 18), lymphangioleiomyoma (n = 2), clear-cell myomelanocytic tumor of falciform ligament/ligamentum teres (n = 1), and not otherwise specified (n = 1). Fifteen cases were classified as uncertain malignant potential (n = 13) or malignant (n = 2). CT, MRI and ultrasound features included well-defined margins (19/22), internal heterogeneity (20/22), arterial enhancement (20/22), dysmorphic vessels (17/22), fat (9/22), hemorrhage (3/22), necrosis (8/22), and calcification (2/22). The diagnostic accuracy was only 27.3% (6/22). No local recurrence or metastasis was found in the follow-up patients (12/22). Conclusions On CT, MRI and ultrasound images, most hepatic PEComas are well-defined, heterogeneous, arterial enhanced masses with dysmorphic vessels, with or without fat, especially in middle-aged females. With the potential to be malignant, timely surgical resection and long-term follow-up may be helpful for improving the prognosis.
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Affiliation(s)
- Pei Nie
- Department of Radiology, the Affiliated Hospital of Qingdao University, Qingdao, 266000, Shandong, China
| | - Jie Wu
- Department of Pathology, the Affiliated Hospital of Qingdao University, Qingdao, 266000, Shandong, China
| | - Hexiang Wang
- Department of Radiology, the Affiliated Hospital of Qingdao University, Qingdao, 266000, Shandong, China
| | - Ruizhi Zhou
- Department of Radiology, the Affiliated Hospital of Qingdao University, Qingdao, 266000, Shandong, China
| | - Lingling Sun
- Department of Pathology, the Affiliated Hospital of Qingdao University, Qingdao, 266000, Shandong, China
| | - Jingjing Chen
- Department of Radiology, the Affiliated Hospital of Qingdao University, Qingdao, 266000, Shandong, China.
| | - Guangjie Yang
- PET-CT Center, the Affiliated Hospital of Qingdao University, Qingdao, 266000, Shandong, China.
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Yang J, Zhang HR, Zhang R, Hua X, Cai P, Yan XC. Hepatic monotypic epithelioid angiomyolipoma with concomitant hepatocellular carcinoma. INTERNATIONAL JOURNAL OF CLINICAL AND EXPERIMENTAL PATHOLOGY 2019; 12:1399-1405. [PMID: 31933955 PMCID: PMC6947045] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Subscribe] [Scholar Register] [Received: 12/19/2018] [Accepted: 01/21/2019] [Indexed: 06/10/2023]
Abstract
Hepatic monotypic epithelioid angiomyolipoma (AML) is a rare lesion in which the predominant population of an epithelioid component can mimic hepatocellular carcinoma (HCC). The hepatic epithelioid AML with concomitant HCC is extremely uncommon. In this study, we present the clinical and pathologic features of a case of hepatic monotypic epithelioid AML with concomitant HCC in a 63-year-old man. Imaging examinations revealed two masses located in the liver, measuring 83×63 mm and 37×27 mm separately, which exhibited an early contrast enhancement and a rapid washout on enhanced computed tomography (CT), so that HCC with intrahepatic metastases was suspected. The small tumor was removed for intraoperative frozen section examination. Grossly, the tumor was solitary, well-circumscribed, and non-encapsulated. Microscopically, it was composed purely of a trabecular arrangement of epithelioid cells with a sinusoidal pattern. Immunohistochemically, it was positive for HMB45, Melan-A, and alpha smooth muscle actin (α-SMA). Interestingly, the large tumor has the histologic features similar to those of the small one. However, it was positive for epithelioid markers and negative for the melanocytic markers. It reminds us that there is a possibility of coexistence of HAML and HCC in the liver. We believe that this might be the first case report of a hepatic monotypic epithelioid AML with concomitant HCC. The patient gave up treatment and died in 6 months after the operation in the follow-up.
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Affiliation(s)
- Jing Yang
- Institute of Pathology, Southwest Hospital, Army Medical University (Third Military Medical University)Chongqing, People’s Republic of China
| | - Hua-Rong Zhang
- Institute of Pathology, Southwest Hospital, Army Medical University (Third Military Medical University)Chongqing, People’s Republic of China
| | - Rong Zhang
- Institute of Pathology, Southwest Hospital, Army Medical University (Third Military Medical University)Chongqing, People’s Republic of China
| | - Xing Hua
- Department of Ultrasound, Southwest Hospital, Army Medical University (Third Military Medical University)Chongqing, People’s Republic of China
| | - Ping Cai
- Department of Radiology, Southwest Hospital, Army Medical University (Third Military Medical University)Chongqing, People’s Republic of China
| | - Xiao-Chu Yan
- Institute of Pathology, Southwest Hospital, Army Medical University (Third Military Medical University)Chongqing, People’s Republic of China
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Sangiorgio V, Daniele L, Gallo T, Rocca R, Fava C, Campisi P, De Rosa G. Ultrasound-guided fine needle aspiration cytology in the diagnosis of hepatic and pancreatic perivascular epithelioid cell tumors: A case series. Diagn Cytopathol 2018; 47:315-319. [DOI: 10.1002/dc.24111] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/12/2017] [Revised: 09/06/2018] [Accepted: 09/28/2018] [Indexed: 02/06/2023]
Affiliation(s)
- Valentina Sangiorgio
- Division of Pathology; European Institute of Oncology, University of Milan; Milan Italy
| | | | - Teresa Gallo
- Radiology Unit; AO Ordine Mauriziano; Torino Italy
| | - Rodolfo Rocca
- Gastroenterology Unit; AO Ordine Mauriziano; Torino Italy
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23
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Kirnap M, Ozgun G, Moray G, Haberal M. Perivascular epithelioid cell tumor outgrowth from the liver. Int J Surg Case Rep 2018; 53:295-298. [PMID: 30453241 PMCID: PMC6240724 DOI: 10.1016/j.ijscr.2018.10.046] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/31/2018] [Revised: 10/09/2018] [Accepted: 10/19/2018] [Indexed: 12/29/2022] Open
Abstract
INTRODUCTION Perivascular epithelioid celltumor (PEComa) is a rare mesenchymal neoplasia and can be found in various body sites. On the other hand, hepatic PEComa is very rare, with only a few studies having reported hepatic malignant PEComa. There is no gold standard regarding the use of diagnostic imaging studies. The diagnosis of hepatic PEComa is made by a positive immunohistochemical staining for HMB45 and Melan A. Herein, we discussed the therapeutic and follow-up process of a symptomatic hepatic PEComa case. PRESENTATION OF CASE A 22-year-old woman presented with a palpable mass in abdomen. A computerized tomographic examination showed a giant hepatic mass of left lobe origin, which was excised surgically. The pathology result was reported as a PEComa. DISCUSSION The diagnostic approach, treatment modalities, and follow-up procedures are not standard. The main treatment modality for PEComa is surgical excision with adequate surgical margin. CONCLUSION A longer follow-up is required for patients with hepatic PEComa because the nature of the disease is not entirely clear.
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Affiliation(s)
- Mahir Kirnap
- Departments of Transplantation, Baskent University, Ankara, Turkey
| | - Gonca Ozgun
- Departments of Pathology, Baskent University, Ankara, Turkey
| | - Gokhan Moray
- Departments of Transplantation, Baskent University, Ankara, Turkey
| | - Mehmet Haberal
- Departments of Transplantation, Baskent University, Ankara, Turkey.
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24
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Ma Y, Huang P, Gao H, Zhai W. Hepatic perivascular epithelioid cell tumor (PEComa): analyses of 13 cases and review of the literature. INTERNATIONAL JOURNAL OF CLINICAL AND EXPERIMENTAL PATHOLOGY 2018; 11:2759-2767. [PMID: 31938393 PMCID: PMC6958297] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Subscribe] [Scholar Register] [Received: 01/19/2018] [Accepted: 02/22/2018] [Indexed: 06/10/2023]
Abstract
Aim: To study the clinicopathologic characteristics of perivascular epithelioid cell tumor (PEComa) in the liver and to improve the understanding and diagnosis of this tumor. Methods and results: 13 cases of primary liver PEComa were retrieved from our hospital from January 2007 to September 2017. The clinicopathologic features and the computer tomographic (CT) or/and magnetic resonance imaging (MRI) tests of each case were retrospectively reviewed. All 13 patients were female, with ages ranging from 22 to 72 years (median, 35 years). The sizes of the tumor ranged from 1.0 to 19.8 cm. Histologically, the tumors were comprised of polygonal cells with clear to granular eosinophilic cytoplasm and were accompanied by positive immunohistochemical expression of HMB-45 and/or Melan-A. 6 cases showed moderate cytological atypia. Hemorrhage was present in 7 of 13 cases, and necrosis in 2 cases. Mitoses were scant, averaged from 0 to 1/50 high-power fields in every case. No vascular invasion was present in any case. Follow-up data were obtained from 9 of 13 cases, and none showed any evidence of tumor recurrence or metastasis. Conclusions: There are no specific symptoms of hepatic PEComa, and the preoperative imaging tests are also insensitive. In view of diverse histological growth patterns with atypical cell features in part of cases, the possibility of PEComa should be considered whenever unfamiliar hepatic tumors are encountered. Immunoreactivity for HMB-45, MelanA, and SMA, especially HMB-45, is very useful for the diagnosis of this tumor.
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Affiliation(s)
- Yihui Ma
- Department of Pathology, 1 Affiliated Hospital, Zhengzhou UniversityZhengzhou, China
- Henan Key Laboratory for Tumor Pathology, Zhengzhou UniversityZhengzhou, China
| | - Pei Huang
- Department of Pathology, 1 Affiliated Hospital, Zhengzhou UniversityZhengzhou, China
- Henan Key Laboratory for Tumor Pathology, Zhengzhou UniversityZhengzhou, China
| | - Hanqing Gao
- Department of Pathology, 1 Affiliated Hospital, Zhengzhou UniversityZhengzhou, China
- Henan Key Laboratory for Tumor Pathology, Zhengzhou UniversityZhengzhou, China
| | - Wenlong Zhai
- Department of Hepatobiliary Surgery, 1 Affiliated Hospital, Zhengzhou UniversityZhengzhou, China
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Kirste S, Kayser G, Zipfel A, Grosu AL, Brunner T. Unresectable hepatic PEComa: a rare malignancy treated with stereotactic body radiation therapy (SBRT) followed by complete resection. Radiat Oncol 2018; 13:28. [PMID: 29463266 PMCID: PMC5819697 DOI: 10.1186/s13014-018-0974-5] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/16/2017] [Accepted: 02/11/2018] [Indexed: 02/07/2023] Open
Abstract
BACKGROUND Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors occurring in various anatomic regions. Although diagnostic criteria and treatment management are not established, current treatment options consist of surgery and chemotherapy including mTOR inhibitors. Stereotactic body radiation therapy (SBRT) is a non-invasive ablative treatment which has shown excellent control rates for more common types of unresectable liver tumors and metastases. In this report we present a rare case of PEComa of the liver that was treated by stereotactic radiotherapy followed by resection. Staging and evaluation of treatment response was done by FDG-PET/CT. This case highlights the potential of SBRT as a neoadjuvant treatment even for rare liver malignancies. It is the first case of liver PEComa treated by SBRT and resection. CASE PRESENTATION A 52-year-old woman presented at an external hospital with abdominal pressure and pain in the right upper abdominal quadrant. A CT scan showed a 700 cm3 liver lesion in segment IV. In repeated biopsy in July 2015 histopathological workup showed a pleomorphic epitheloid tumor with small to medium sized cells expressing vimentin and melan-A while being negative for cytokeratin establishing the diagnosis of PEComa of the liver. To achieve high, ablative doses a stereotactic body radiotherapy (SBRT) technique was chosen consisting of 60Gy (biologically effective dose 105Gy) in 8 fractions of 7.5Gy. Radiotherapy planning was based on MRI resulting in a planning target volume (PTV) of 1944 cm3. Treatment toxicity was limited to a slight elevation of transaminases (grade 1 and 3). A complete resection was performed 21 weeks after radiotherapy confirmed by negative surgical margins. At last follow-up 21 months after therapy, MRI showed neither local nor distant tumor recurrence. The patient was in stable condition (ECOG 1) and without late radiation toxicity. CONCLUSIONS This is the first documented case of liver PEComa treated by SBRT and resection. A favorable post-treatment course demonstrates that SBRT is a potential neoadjuvant treatment that is capable of reducing an inoperable rare liver tumor to a resectable lesion.
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Affiliation(s)
- Simon Kirste
- Department of Radiation Oncology, University Medical Center Freiburg, Robert-Koch-Str. 6, 79106 Freiburg, Germany
- German Cancer Consortium, Partner Site Freiburg (DKTK), Freiburg, Germany
| | - Gian Kayser
- Department of Surgical Pathology, University Medical Center Freiburg, Faculty of Medicine, Freiburg, Germany
| | - Anne Zipfel
- Department of Radiation Oncology, University Medical Center Freiburg, Robert-Koch-Str. 6, 79106 Freiburg, Germany
- German Cancer Consortium, Partner Site Freiburg (DKTK), Freiburg, Germany
| | - Anca-Ligia Grosu
- Department of Radiation Oncology, University Medical Center Freiburg, Robert-Koch-Str. 6, 79106 Freiburg, Germany
- German Cancer Consortium, Partner Site Freiburg (DKTK), Freiburg, Germany
| | - Thomas Brunner
- Department of Radiation Oncology, University Medical Center Freiburg, Robert-Koch-Str. 6, 79106 Freiburg, Germany
- German Cancer Consortium, Partner Site Freiburg (DKTK), Freiburg, Germany
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Han X, Sun MY, Liu JH, Zhang XY, Wang MY, Fan R, Qamar S. Computed tomography imaging features of hepatic perivascular epithelioid cell tumor: A case report and literature review. Medicine (Baltimore) 2017; 96:e9046. [PMID: 29245304 PMCID: PMC5728919 DOI: 10.1097/md.0000000000009046] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/22/2023] Open
Abstract
RATIONALE Perivascular epithelioid cell tumor (PEComa) is a rare tumor which is most frequently found in uterus. The tumor arising from liver is extremely uncommon. PATIENT CONCERNS A 36-year-old female with abdominal distention, cramps, and low-grade fever for over 15 days. The patient had a history of gastric adenocarcinoma with ovarian, celiac lymph nodes, and retroperitoneal lymph nodes metastases. DIAGNOSES Computed tomography (CT) imaging demonstrated an ill-defined heterogeneous hypo-dense mass in segment 8 (S8) of the liver. Contrast-enhanced CT imaging showed marked enhancement in arterial phase, mild-to-moderate enhancement in portal and equilibrium phases. Tumor-feeding artery was demonstrated from the right hepatic artery by the three-dimensional reconstruction images. Biopsy was performed, and a diagnosis of PEComa was rendered. INTERVENTIONS No intervention for this tumor before liver biopsy. LESSONS We present a rare case of hepatic PEComa. The information we provided is useful for summarizing the CT features of this kind of tumors. It should be included in differential diagnoses from common hypervascular neoplasms of liver. The final diagnosis is established on histopathological and immunohistochemical studies that are the "gold standard."
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Affiliation(s)
- Xu Han
- The First Affiliated Hospital of Dalian Medical University, Dalian, Liaoning
| | - Mei-Yu Sun
- The First Affiliated Hospital of Dalian Medical University, Dalian, Liaoning
| | - Jing-Hong Liu
- The First Affiliated Hospital of Dalian Medical University, Dalian, Liaoning
| | | | - Meng-Yao Wang
- The First Affiliated Hospital of Dalian Medical University, Dalian, Liaoning
| | - Rui Fan
- The First Affiliated Hospital of Dalian Medical University, Dalian, Liaoning
| | - Sahrish Qamar
- The Chinese University of Hong Kong, Shatin, Hong Kong, China
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