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Boccatonda A, Marcellini MM, Ruggeri E, Felicani C, Brighenti A, Loiacono R, Ercolani G, Serra C. Ceus features of liver pecoma: a case report and literature review. J Ultrasound 2025; 28:261-268. [PMID: 39557792 PMCID: PMC11947360 DOI: 10.1007/s40477-024-00973-x] [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: 09/28/2024] [Accepted: 10/24/2024] [Indexed: 11/20/2024] Open
Abstract
Perivascular epithelioid cell neoplasms (PEComas) and epithelioid angiomyolipomas (EAMLs) are two different denominations for the same "mesenchymal tumor composed of histologically and immunohistochemically distinctive perivascular epithelioid cells". Hepatic PEComa/EAML is a very rare neoplasm, and only 29 case reports of hepatic PEComa and 25 of hepatic EAML have been reported in the current literature. A clear female predominance with a mean age at diagnosis of 42.5 years old can be observed by literature review. Ultrasound (US) examination was the first-line diagnostic technique in most of the cases of hepatic PEComa, but it was documented in very few cases of hepatic EAML. A great variability in the ultrasonographic B-mode, color Doppler and contrast-enhanced ultrasonography (CEUS) features of hepatic PEComa/EAML emerges. Computed tomography and magnetic resonance were the most common used techniques to confirm the nature of the hepatic lesion, even if the anatomo-pathological examination was the only technique to display a certainty diagnosis and to differentiate hepatic PEComa/EAML from benign and malignant hepatic lesions. The great majority of hepatic PEComas/EAMLs are surgically treated without any adjuvant therapy.
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Affiliation(s)
- Andrea Boccatonda
- Diagnostic and Therapeutic Interventional Ultrasound Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Policlinico Sant'Orsola-Malpighi, via Massarenti n 9, 40138, Bologna, Italy.
| | | | - Eugenio Ruggeri
- Diagnostic and Therapeutic Interventional Ultrasound Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Policlinico Sant'Orsola-Malpighi, via Massarenti n 9, 40138, Bologna, Italy
| | - Cristina Felicani
- Medicina ad Indirizzo Metabolico Nutrizionale. Policlinico di Modena, AOU Modena, Modena, Italy
| | - Alice Brighenti
- Diagnostic and Therapeutic Interventional Ultrasound Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Policlinico Sant'Orsola-Malpighi, via Massarenti n 9, 40138, Bologna, Italy
| | - Rossella Loiacono
- Diagnostic and Therapeutic Interventional Ultrasound Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Policlinico Sant'Orsola-Malpighi, via Massarenti n 9, 40138, Bologna, Italy
| | - Giorgio Ercolani
- General and Oncologic Surgery, Morgagni-Pierantoni Hospital, AUSL Romagna, Via Forlanini 34, 47121, Forlì, Italy
- Department of Medical and Surgical Sciences, University of Bologna, Via Zamboni 33, 40126, Bologna, Italy
| | - Carla Serra
- Diagnostic and Therapeutic Interventional Ultrasound Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Policlinico Sant'Orsola-Malpighi, via Massarenti n 9, 40138, Bologna, Italy
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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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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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Li G, Wang X, He X. Hepatic perivascular epithelioid cell tumor with the constipation symptom: A rare case report. Asian J Surg 2024; 47:1628-1630. [PMID: 38143173 DOI: 10.1016/j.asjsur.2023.12.079] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/26/2023] [Accepted: 12/08/2023] [Indexed: 12/26/2023] Open
Affiliation(s)
| | - Xin Wang
- Gannan Medical University, China
| | - Xiao He
- First Affiliated Hospital of Gannan Medical University, China.
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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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Yazıcı C, Gündoğdu E. Very Rare Liver Tumor: PEComa Case Report with and a Review of Literature. Indian J Radiol Imaging 2024; 34:172-176. [PMID: 38106859 PMCID: PMC10723948 DOI: 10.1055/s-0043-1774392] [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] [Indexed: 12/19/2023] Open
Abstract
Perivascular epithelioid cell tumors (PEComas) are very rare mesenchymal tumors. In addition to being rare in general, they are even rarer in the liver. There are various subtypes and there is only one case of liver PEComa reported in the clear cell type in the literature to date. We present the second clear cell type liver PEComa in this case by reviewing the literature data. The hypodense, smooth-edged, ovoid lesion was detected on computed tomography (CT) for performing abdominal pain in a 41-year-old female patient. Magnetic resonance imaging (MRI) was then performed for lesion characterization. The lesion was hypointense on T1-weighted imaging (T1WI), hyperintense on T2-weighted imaging (T2WI). In dynamic phases, it showed marked enhancement on the arterial phase and capsular enhancement with central washout on the portal and late venous phases. The posterior branch of the right portal vein extended into the mass. The lesion was excised and the pathological result was epithelioid clear cell subtype of PEComa. Although the imaging findings are generally nonspecific and certain diagnosis is made histopathologically, radiologists should consider PEComa in the differential diagnosis in the presence of intensely enhanced lesion on the right lobe in female patients. Also, the "large vessel sign" may help in the diagnosis.
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Affiliation(s)
- Celal Yazıcı
- Department of Radiology, Eskişehir Osmangazi University, Faculty of Medicine, Eskişehir, Turkey
| | - Elif Gündoğdu
- Department of Radiology, Eskişehir Osmangazi University, Faculty of Medicine, Eskişehir, Turkey
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Yang W, Sun Q, Shang M, Li S, Hu X, Hu X. Multimodal imaging study of hepatic perivascular epithelioid cell tumors: a case report. Front Med (Lausanne) 2023; 10:1322048. [PMID: 38173942 PMCID: PMC10762310 DOI: 10.3389/fmed.2023.1322048] [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: 10/15/2023] [Accepted: 11/27/2023] [Indexed: 01/05/2024] Open
Abstract
Hepatic perivascular epithelioid cell tumors (PEComas) are rare interstitial tumors that are often misdiagnosed as hepatocellular carcinomas due to their unique vascular enhancement patterns. Herein, we present a case of a 61-year-old man who was incidentally found to have a lesion in the left medial segment of the liver during a chest computed tomography (CT) examination performed 4 days prior to his presentation for chest discomfort. Imaging revealed solid components with density similar to that of normal liver tissue and areas of low-density adipose tissue within the lesion. The solid components exhibited increased uptake of fluorine-18 fluorodeoxyglucose on positron emission tomography/CT. Magnetic resonance imaging demonstrated areas with unevenly high signal intensity in both T1-weighted imaging (T1WI) in-phase and T2-weighted imaging (T2WI) sequences, while T2WI in the opposite phase displayed areas with unevenly low signal intensity, indicating the presence of fatty components. Contrast-enhanced T1WI displayed a "fast in and fast out" enhancement pattern. These distinct imaging features contribute to the diagnosis of hepatic PEComas and distinguish it from hepatocellular carcinoma.
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Affiliation(s)
| | | | | | | | | | - Xianwen Hu
- Department of Nuclear Medicine, Affiliated Hospital of Zunyi Medical University, Zunyi, China
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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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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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Yang G, Hwang S, Ahn CS, Kim KH, Moon DB, Ha TY, Song GW, Jung DH, Park GC, Hong SM. Clinicopathological correlation and post-resection outcomes of hepatic angiomyolipoma. Ann Hepatobiliary Pancreat Surg 2021; 25:215-220. [PMID: 34053924 PMCID: PMC8180398 DOI: 10.14701/ahbps.2021.25.2.215] [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: 09/24/2020] [Revised: 09/29/2020] [Accepted: 10/01/2020] [Indexed: 01/12/2023] Open
Abstract
Backgrounds/Aims Angiomyolipoma is a rare neoplasm of mesenchymal origin derived from perivascular epithelioid cells. Due to rarity, hepatic angiomyolipoma (HAML) has been often misdiagnosed as hepatocellular carcinoma (HCC) or other hypervascular liver tumors based on imaging studies. This study investigated the clinicopathological correlation and post-resection outcomes of HAML. Methods This retrospective observational study included 40 patients who underwent hepatic resection (HR) for HAML between 2008 and 2018. Results Mean age of the patients was 42.6±11.4 years and there were 30 (75.0%) females. Hepatitis B and C virus infection was present in 8 patients (20.0%) and 1 patient (2.5%), respectively. Preoperative diagnoses on imaging studies were HCC in 23 (57.5%) patients, HAML in 14 (35.0%) patients, focal nodular hyperplasia in 2 (5.0%) patients, and hepatic adenoma in 1 (2.5%) patient. Percutaneous liver biopsy was performed in 10 (25.0%) patients and HAML was diagnosed in all patients. Only 3 patients (7.5%) showed a slight elevation in the level of liver tumor markers. Major HR was performed in 10 (25.0%). Laparoscopic HR was performed in 9 (22.5%). The mean tumor size was 4.8±3.9 cm and single tumor was present in 38 (95.0%) patients. Currently, all the patients are alive without tumor recurrence during the follow-up observation period of 75.7±37.3 months. Conclusions HAML is a rare form of primary liver tumor and is often misdiagnosed as HCC or other hypervascular tumors. Although HAML is benign in nature, it has malignant potential, thus resection is indicated if the tumor grows or malignancy cannot be excluded.
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Affiliation(s)
- Geunhyeok Yang
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Shin Hwang
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Chul-Soo Ahn
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Ki-Hun Kim
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Deok-Bog Moon
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Tae-Yong Ha
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Gi-Won Song
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Dong-Hwan Jung
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Gil-Chun Park
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Seung-Mo Hong
- Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
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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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Li MY, Lin JC, Liou TC. A Rare Hepatic Tumor. Clin Gastroenterol Hepatol 2018; 16:e27-e28. [PMID: 28529165 DOI: 10.1016/j.cgh.2017.05.011] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/10/2017] [Accepted: 05/13/2017] [Indexed: 02/07/2023]
Affiliation(s)
| | - Jiunn-Chang Lin
- Department of Surgery, Taipei, Taiwan; Liver Medical Center, Taipei, Taiwan; Mackay Junior College of Medicine, Nursing, and Management, Taipei, Taiwan
| | - Tai-Cherng Liou
- Department of Gastroenterology, Mackay Memorial Hospital, and MacKay Medical College, Taipei, Taiwan
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13
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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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14
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Tsai HY, Kao Y, Chuang CK, Lee KH. Huge retroperitoneal epithelioid angiomyolipoma: A case report. Urol Case Rep 2017; 17:10-11. [PMID: 29276686 PMCID: PMC5737944 DOI: 10.1016/j.eucr.2017.11.016] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/09/2017] [Revised: 11/08/2017] [Accepted: 11/21/2017] [Indexed: 01/27/2023] Open
Affiliation(s)
- Han-Yu Tsai
- Urology, Chang Gung Memorial Hospital, 5, Fushing Street, Kweishan, Taoyuan, Taiwan
| | - Yuting Kao
- School of Medicine, Chang Gung University, Chang Gung Memorial Hospital, 5, Fushing Street, Kweishan, Taoyuan, Taiwan
| | - Cheng-Keng Chuang
- Division of Urology, Department of Surgery, Chang Gung Memorial Hospital, Chang Gung University, Taoyuan, Taiwan
| | - Kun-Han Lee
- School of Medicine, Chang Gung University, Chang Gung Memorial Hospital, 5, Fushing Street, Kweishan, Taoyuan, Taiwan
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15
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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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16
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Okamoto S, Komura M, Terao Y, Kurisaki-Arakawa A, Hayashi T, Saito T, Togo S, Shiokawa A, Mitani K, Kobayashi E, Kumasaka T, Takahashi K, Seyama K. Pneumothorax caused by cystic and nodular lung metastases from a malignant uterine perivascular epithelioid cell tumor (PEComa). Respir Med Case Rep 2017; 22:77-82. [PMID: 28706850 PMCID: PMC5496452 DOI: 10.1016/j.rmcr.2017.06.011] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/20/2017] [Accepted: 06/20/2017] [Indexed: 12/03/2022] Open
Abstract
Perivascular epithelioid cell tumors (PEComas) are mesenchymal neoplasms with immunoreactivity for both melanocytic and smooth muscle markers. PEComas occur at multiple sites, and malignant PEComas can undergo metastasis, recurrence and aggressive clinical courses. Although the lung is a common metastatic site of PEComas, they usually appear as multiple nodules but rarely become cystic or cavitary. Here, we describe a female patient whose lungs manifested multiple cystic, cavity-like and nodular metastases 3 years after the resection of uterine tumors tentatively diagnosed as epithelioid smooth muscle tumors with uncertain malignant potential. This patient's subsequent pneumothorax necessitated video-assisted thoracoscopic surgery, and examination of her resected lung specimens eventually led to correcting the diagnosis, i.e., to a PEComa harboring tuberous sclerosis complex 1 (TSC1) loss-of-heterozygosity that originated in the uterus and then metastasized to the lungs. The administration of a gonadotropin-releasing hormone analogue later stabilized her clinical course. To the best of our knowledge, the present case is the first in the literature that associates PEComas with a TSC1 abnormality. Additionally, the pulmonary manifestations, including imaging appearance and pneumothorax, somewhat resembled those of lymphangioleiomyomatosis, a representative disease belonging to the PEComa family. Although PEComas are rare, clinicians, radiologists and pathologists should become aware of this disease entity, especially in the combined clinical setting of multiple cystic, cavity-like, nodular lesions on computed tomography of the chest and a past history of the tumor in the female reproductive system.
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Key Words
- CAPUs, clinically aggressive PEComas of the uterine corpus
- CT, computed tomography
- Cystic lung disease
- ESS, endometrial stromal sarcoma
- GnRH, gonadotropin-releasing hormone analogue
- HPF, high-power fields
- LAM, lymphangioleiomyomatosis
- LOH, loss of heterozygosity
- Loss of heterozygosity
- Multiple lung nodules
- PEComa
- PEComa, perivascular epithelioid cell tumor
- PEComa-NOS, PEComa not otherwise specified
- Pneumothorax
- Pulmonary metastasis
- TFE3, transcription factor E3
- TSC, tuberous sclerosis complex
- mTOR, mammalian target of rapamycin
- α-SMA, α-smooth muscle actin
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Affiliation(s)
- Shouichi Okamoto
- Division of Respiratory Medicine, Juntendo University Faculty of Medicine and Graduate School of Medicine, 3-1-3 Hongo, Bunkyo-ku, Tokyo, 113-8431, Japan.,The Study Group for Pneumothorax and Cystic Lung Diseases, 4-8-1 Seta, Setagaya-ku, Tokyo, 158-0095, Japan
| | - Moegi Komura
- Division of Respiratory Medicine, Juntendo University Faculty of Medicine and Graduate School of Medicine, 3-1-3 Hongo, Bunkyo-ku, Tokyo, 113-8431, Japan
| | - Yasuhisa Terao
- Department of Gynecology and Obstetrics, Juntendo University Faculty of Medicine and Graduate School of Medicine, 3-1-3 Hongo, Bunkyo-ku, Tokyo, 113-8431, Japan
| | - Aiko Kurisaki-Arakawa
- Department of Human Pathology, Juntendo University Faculty of Medicine and Graduate School of Medicine, 3-1-3 Hongo, Bunkyo-ku, Tokyo, 113-8431, Japan
| | - Takuo Hayashi
- Department of Human Pathology, Juntendo University Faculty of Medicine and Graduate School of Medicine, 3-1-3 Hongo, Bunkyo-ku, Tokyo, 113-8431, Japan.,The Study Group for Pneumothorax and Cystic Lung Diseases, 4-8-1 Seta, Setagaya-ku, Tokyo, 158-0095, Japan
| | - Tsuyoshi Saito
- Department of Human Pathology, Juntendo University Faculty of Medicine and Graduate School of Medicine, 3-1-3 Hongo, Bunkyo-ku, Tokyo, 113-8431, Japan
| | - Shinsaku Togo
- Division of Respiratory Medicine, Juntendo University Faculty of Medicine and Graduate School of Medicine, 3-1-3 Hongo, Bunkyo-ku, Tokyo, 113-8431, Japan
| | - Akira Shiokawa
- Department of Clinical Diagnostic Pathology, Showa University Fujigaoka Hospital, 1-30, Fujigaoka Aoba-ku, Yokohama, Kanagawa, 227-8501, Japan
| | - Keiko Mitani
- Department of Human Pathology, Juntendo University Faculty of Medicine and Graduate School of Medicine, 3-1-3 Hongo, Bunkyo-ku, Tokyo, 113-8431, Japan.,The Study Group for Pneumothorax and Cystic Lung Diseases, 4-8-1 Seta, Setagaya-ku, Tokyo, 158-0095, Japan
| | - Etsuko Kobayashi
- Division of Respiratory Medicine, Juntendo University Faculty of Medicine and Graduate School of Medicine, 3-1-3 Hongo, Bunkyo-ku, Tokyo, 113-8431, Japan.,The Study Group for Pneumothorax and Cystic Lung Diseases, 4-8-1 Seta, Setagaya-ku, Tokyo, 158-0095, Japan
| | - Toshio Kumasaka
- Department of Pathology, Japanese Red Cross Medical Center, 4-1-22 Hiroo, Shibuya-ku, Tokyo, 150-8935, Japan.,The Study Group for Pneumothorax and Cystic Lung Diseases, 4-8-1 Seta, Setagaya-ku, Tokyo, 158-0095, Japan
| | - Kazuhisa Takahashi
- Division of Respiratory Medicine, Juntendo University Faculty of Medicine and Graduate School of Medicine, 3-1-3 Hongo, Bunkyo-ku, Tokyo, 113-8431, Japan
| | - Kuniaki Seyama
- Division of Respiratory Medicine, Juntendo University Faculty of Medicine and Graduate School of Medicine, 3-1-3 Hongo, Bunkyo-ku, Tokyo, 113-8431, Japan.,The Study Group for Pneumothorax and Cystic Lung Diseases, 4-8-1 Seta, Setagaya-ku, Tokyo, 158-0095, Japan
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17
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Ortiz S, Tortosa F. Epithelioid angiomyolipoma of the liver: Clinicopathological correlation in a series of 4 cases. REVISTA ESPANOLA DE ENFERMEDADES DIGESTIVAS 2017; 108:27-30. [PMID: 26765232 DOI: 10.17235/reed.2015.3947/2015] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
Hepatic angiomyolipoma is a rare neoplasm that can be difficult to diagnose in cases of ecoguide biopsy. We studied 4 cases of female patients with a mean age of 51 years. None of the patients presented specific abdominal symptoms, or other tumour masses detected by chance. One of them had relevant personal history: Renal cell carcinoma. This same patient was diagnosed with a contralateral renal angiomyolipoma. None of the patients showed evidence of tuberous sclerosis. Three tumours have been diagnosed by biopsy and only in one patient was decided to completely remove the tumour surgically. None of the patients had evidence of recurrence of disease or an increase in tumour size over an average period of 45 months. Histologically, the tumours have been sub classified into angiomyolipomas epithelioid. In 50% of the cases, extramedullary haematopoiesis was observed. The first diagnostic impression using imaging methods included: Focal nodular hyperplasia, hepatocellular adenoma, hepatocellular carcinoma and metastasis. By immunohistochemistry, it has been demonstrated that all tumours expressing melanocytic markers (HMB45 and Melan A) and markers of smooth muscle cells (smooth muscle actin). The diagnosis of these tumours is increasing due to programmes for early detection of hepatocellular carcinoma in patients with liver cirrhosis. It must be taken into account their broad spectrum of morphological diversity to avoid incorrect diagnosis of a malignant neoplasm.
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Affiliation(s)
- Santiago Ortiz
- Anatomía Patológica, Centro Hospitalario Lisboa Norte, Portugal
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18
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Jung DH, Hwang S, Hong SM, Kim KH, Ahn CS, Moon DB, Alshahrani AA, Lee SG. Clinico-pathological correlation of hepatic angiomyolipoma: a series of 23 resection cases. ANZ J Surg 2017; 88:E60-E65. [PMID: 28122404 DOI: 10.1111/ans.13880] [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: 10/22/2016] [Revised: 11/15/2016] [Accepted: 11/16/2016] [Indexed: 12/12/2022]
Abstract
BACKGROUND Angiomyolipomas are rare neoplasms of mesenchymal origin and are derived from perivascular epithelioid cells. They usually develop in the kidney and rarely in the liver. Due to their rarity, most hepatic angiomyolipomas have been misinterpreted as hepatocellular carcinoma (HCC) or other hypervascular liver tumours on imaging studies. We aimed to assess the clinico-pathological correlation of hepatic angiomyolipoma. METHODS We identified 23 patients with hepatic angiomyolipoma through an institutional database search. RESULTS Of 5680 cases of primary liver tumours, 23 (0.4%) had angiomyolipomas (mean age, 43.6 ± 12.4 years; 16 female patients). Hepatitis B virus infection was noted in four patients, whereas a liver mass was incidentally detected on routine health screening in 13 patients. The preoperative diagnoses, before liver biopsy, included HCC in 14, angiomyolipoma in six, focal nodular hyperplasia in two and hepatic adenoma in one patient. Eventually, the preoperative diagnoses were changed to HCC in 12 and hepatic angiomyolipoma in 11 patients. The tumour size was 5.3 ± 4.6 cm, and 22 patients had a single tumour. All tumours exhibited positive findings for human melanoma black-45 and smooth muscle actin staining. During a follow-up period of 52.2 ± 23.7 months, none of the patients exhibited tumour recurrence or mortality. CONCLUSIONS Hepatic angiomyolipoma is a rare form of primary liver tumour and is often misdiagnosed as other hypervascular tumours. Although angiomyolipoma is benign in nature, it also has malignant potential; hence, resection is indicated if the tumour grows or malignancy cannot be excluded. Surgical resection is a definitive curative treatment of hepatic angiomyolipoma.
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Affiliation(s)
- Dong Hwan Jung
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Shin Hwang
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Seung Mo Hong
- Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Ki Hun Kim
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Chul Soo Ahn
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Deok Bog Moon
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Abdulwahab A Alshahrani
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
| | - Sung Gyu Lee
- Department of Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea
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19
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Tang D, Wang J, Tian Y, Li Q, Yan H, Wang B, Xiong L, Li Q. Hepatic perivascular epithelioid cell tumor: Case report and brief literature review. Medicine (Baltimore) 2016; 95:e5572. [PMID: 28002331 PMCID: PMC5181815 DOI: 10.1097/md.0000000000005572] [Citation(s) in RCA: 8] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/07/2023] Open
Abstract
RATIONAL Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm which expresses both myogenic and melanocytic markers. PEComas are found in a variety locations in the body, but up to now only approximately 30 cases about hepatic perivascular epithelioid cell tumor are reported in English language worldwide. PATIENT CONCERNS A 32-year-old woman was admitted in our hospital with intermittent right upper quadrant pain for 1 month and recent (1 day) progressive deterioration. DIAGNOSES Based on the results of the laboratory examinations and the findings of the computed tomography, the diagnosis of hepatic hamartoma or the hepatocecullar carcinoma with hemorrhage was made. INTERVENTIONS The patient underwent a segmentectomy of the liver, and the finally diagnosis of hepatic PEComa was made with immunohistochemical confirmation with HMB-45 and SMA. OUTCOMES There is no clinical or radiographic evidence of recurrence 9 months after surgery. LESSONS This kind of tumor is extremely rare and the natural history of PEComa is uncertain, as the treatment protocol for hepatic PEComa has not reached a consensus. But the main treatment of the disease may be surgical resection. Only after long term follow-up can we know whether the tumor is benign or malignant. It appears that longer clinical follow-up is necessary in all patients with hepatic PEComas.
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Affiliation(s)
- Da Tang
- Department of General Surgery, Second Xiangya Hospital, Central South University
| | - Jianmin Wang
- Department of General Surgery, Second Xiangya Hospital, Central South University
| | - Yuepeng Tian
- Department of General Surgery, Second Xiangya Hospital, Central South University
| | - Qiuguo Li
- Department of General Surgery, the First Hospital of Hunan University of Chinese Medicine, Changsha, Hunan, P.R. China
| | - Haixiong Yan
- Department of General Surgery, Second Xiangya Hospital, Central South University
| | - Biao Wang
- Department of General Surgery, Second Xiangya Hospital, Central South University
| | - Li Xiong
- Department of General Surgery, Second Xiangya Hospital, Central South University
| | - Qinglong Li
- Department of General Surgery, Second Xiangya Hospital, Central South University
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20
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Du H, Zhou J, Xu L, Yang C, Zhang L, Liang C. Pigmented perivascular epithelioid cell tumor (PEComa) arising from kidney: A case report. Medicine (Baltimore) 2016; 95:e5248. [PMID: 27858882 PMCID: PMC5591130 DOI: 10.1097/md.0000000000005248] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/26/2022] Open
Abstract
INTRODUCTION Perivascular epithelioid cell tumor (PEComa) is a mesenchymal neoplasm composed of perivascular epithelioid cells with clear to eosinophilic cytoplasm. Pigmented PEComa arising from kidney is extraordinarily rare and sometimes can exhibit aggressive biological behavior. CASE REPORT We present here a rare case of pigmented renal PEComa in a 46-year-old female. The patient had complained of lumbago complicated with nausea and vomiting for 2 weeks and therefore was referred to our department. An enhanced computed scan revealed a 4 × 3 × 3 cm round-like mass in the lower pole of right kidney with inhomogeneous enhancement. The tumor cells immunestained was positive for HMB-45, focally positive for c-Kit (CD117), and negative for vimentin, S-100, AE1/AE3, CK-7, CK-18, CD-10, RCC antigen, CgA, DOG-1, EMA, smooth muscle actin, and synaptophysin. We successfully performed 3-dimensional laparoscopic resection of the neoplasm, which was then diagnosed as pigmented PEComa by postoperative pathology. No further growing lesion or metastasis was observed during a 1-year follow-up. CONCLUSION This case report shows that pigmented renal PEComa is often presented as a renal mass with nonspecific symptoms and imaging features. The gold diagnosis of renal pigmented PEComa is mainly based on the combination of histopathology and immunohistochemistry. Complete resection by 3-dimensional laparoscopic nephron-sparing surgery can be an effective therapeutic management.
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Affiliation(s)
| | | | | | | | | | - Chaozhao Liang
- Department of Urology, the First Affiliated Hospital of Anhui Medical University and Institute of Urology, Anhui Medical University, Hefei, Anhui, China
- Correspondence: Chaozhao Liang, Department of Urology, the First Affiliated Hospital of Anhui Medical University and Institute of Urology, Anhui Medical University, Hefei, China (e-mail: )
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21
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Wang ZS, Xu L, Ma L, Song MQ, Wu LQ, Zhou X. Hepatic falciform ligament clear cell myomelanocytic tumor: A case report and a comprehensive review of the literature on perivascular epithelioid cell tumors. BMC Cancer 2015; 15:1004. [PMID: 26698563 PMCID: PMC4690247 DOI: 10.1186/s12885-015-1992-4] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/02/2015] [Accepted: 12/11/2015] [Indexed: 12/11/2022] Open
Abstract
Background The objective of the study was to explore the clinical expression, radiological and pathological features, differential diagnosis, and biological behavior of a clear cell myomelanocytic tumor. In a case involving a clear cell myomelanocytic tumor located in the hepatic falciform ligament, we evaluated clinical expression, radiological characteristics, histopathology, immunohistochemistry, and biological behavior; we also reviewed the relevant literature. Case presentation Clear cell myomelanocytic tumor is a benign soft-tissue neoplasm that often occurs in women, and is expressed as a painless mass. The falciform ligament is its most frequent site of occurrence. The imaging characteristics of this lesion were uneven enhancement in the arterial phase, continuing to strengthen in the venous phase, and equal density in the balance phase. Histological and immunohistochemical analysis revealed the main transparent epithelioid cells and smooth muscle spindle cells to be HMB-45(+), smooth muscle actin(+), and melan-A (+). Conclusion Hepatic vascular epithelioid cell tumors are very rare mesenchymal neoplasms. Few studies have investigated this tumor in the hepatic falciform ligament; consequently, its diagnosis and the selection of an appropriate treatment and follow-up protocol are challenging. Treatment outcome remains unpredictable. Therefore, clear cell myomelanocytic tumor should be viewed as a tumor with uncertain malignant potential requiring long-term follow-up.
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Affiliation(s)
- Zu-Sen Wang
- Department of Hepatobiliary Surgery, Affiliated Hospital of Qingdao University, Qingdao, Shandong, 266003, China.
| | - Lin Xu
- Department of Hepatobiliary Surgery, Affiliated Hospital of Qingdao University, Qingdao, Shandong, 266003, China.
| | - Lin Ma
- Department of General Surgery, Qingdao Eighth People's Hospital, Qingdao, Shandong, 266003, China.
| | - Meng-Qi Song
- Department of Hepatobiliary Surgery, Affiliated Hospital of Qingdao University, Qingdao, Shandong, 266003, China.
| | - Li-Qun Wu
- Department of Hepatobiliary Surgery, Affiliated Hospital of Qingdao University, Qingdao, Shandong, 266003, China.
| | - Xuan Zhou
- Department of Pathology, Affiliated Hospital of Qingdao University, Qingdao, Shandong, 266003, China.
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PEComa in a Young Patient with Known Li-Fraumeni Syndrome. Case Rep Med 2015; 2015:906981. [PMID: 25821471 PMCID: PMC4364120 DOI: 10.1155/2015/906981] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/19/2014] [Revised: 01/14/2015] [Accepted: 02/22/2015] [Indexed: 02/08/2023] Open
Abstract
Perivascular epithelioid cells neoplasms (PEComas) constitute a family of rare tumours which have been reported virtually in all anatomic sites. The histological clarification of the malignant potential of these tumours is still problematic despite the proposed risk stratification systems. Li-Fraumeni syndrome (LFS) is caused by a germline mutation in the TP53 tumour suppressor gene. It is a rare but well-characterized cancer predisposition syndrome leading to the development of a variety of different tumour types. To the best of our knowledge, an association between this syndrome and PEComas has not been previously documented. A 24-year-old lady with known LFS presented with two uncertain-in-nature lesions, one within the right part of the liver and one within the upper pole of the right kidney. The patient underwent an uncomplicated open simultaneous right partial nephrectomy and resection of segment 7 of the liver. The morphological and immunohistochemical features of both lesions were of epithelioid angiomyolipoma (PEComa). Although the obvious scenario was that the liver lesion was a metastasis from the renal lesion, the assessment of their malignant potential according to the existing risk stratification systems was rather in favour of two synchronous primary PEComas, pointing out that the histological assessment of malignant potential of PEComas is still problematic.
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Shi H, Cao Q, Li H, Zhen T, Lai Y, Han A. Malignant perivascular epithelioid cell tumor of the kidney with rare pulmonary and ileum metastases. INTERNATIONAL JOURNAL OF CLINICAL AND EXPERIMENTAL PATHOLOGY 2014; 7:6357-6363. [PMID: 25337291 PMCID: PMC4203262] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Subscribe] [Scholar Register] [Received: 07/30/2014] [Accepted: 08/23/2014] [Indexed: 06/04/2023]
Abstract
AIMS To report one case of malignant perivascular epithelioid cell tumor (PEComa) of the kidney with rare pulmonary and ileum metastases and analyze its clinicopathological features. METHODS We analyzed the clinicopathological features of one case of malignant PEComa of the kidney with pulmonary and ileum metastases. Immunohistochemistry staining was performed. RESULTS The patient was a 48-year-old man with a renal mass approximately 14 cm × 11 cm × 8 cm in size. Microscopically, the tumor was mainly composed of polygonal epithelioid cells with dense eosinophilic cytoplasm and round nuclei with small nucleoli. Focal tumor cells showed pleomorphism with multinucleated giant cells and prominent nucleoli. The tumor cells nests were surrounded by thick-walled irregular blood vessels. Focal fat cells were found within the tumor. Hemorrhage and coagulative necrosis were also present. The tumor cells were positive for vimentin, HMB45, and Melan-A, and focally positive for SMA and S-100 protein. After 5 years and 5.6 years of nephrectomy, the tumor metastasized to the right lung and ileum, respectively. CONCLUSION We first reported one case of malignant PEComa of the kidney with pulmonary and ileum metastases. Metastatic PEComa of the lung and ileum should differentiate from primary carcinoma, metastatic carcinoma, malignant melanoma, and gastrointestinal stromal tumor.
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Affiliation(s)
- Huijuan Shi
- Department of Pathology, The First Affiliated Hospital, Sun Yat-Sen University Guangzhou, China
| | - Qinghua Cao
- Department of Pathology, The First Affiliated Hospital, Sun Yat-Sen University Guangzhou, China
| | - Hui Li
- Department of Pathology, The First Affiliated Hospital, Sun Yat-Sen University Guangzhou, China
| | - Tiantian Zhen
- Department of Pathology, The First Affiliated Hospital, Sun Yat-Sen University Guangzhou, China
| | - Yingrong Lai
- Department of Pathology, The First Affiliated Hospital, Sun Yat-Sen University Guangzhou, China
| | - Anjia Han
- Department of Pathology, The First Affiliated Hospital, Sun Yat-Sen University Guangzhou, China
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