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Hepatic angiomyolipoma

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https://www.readbyqxmd.com/read/29666741/a-novel-mutation-in-tsc2-gene-a-34-year-old-female-with-pulmonary-lymphangioleiomyomatosis-with-concomitant-hepatic-lesions
#1
Mehdi Nadiri, Mortaza Raeisi, Seyed Ali Mousavi Aghdas
Tuberous sclerosis complex (TSC) is an autosomal dominant disease resulting from mutation(s) in TSC1 or TSC2 genes. TSC is associated with the formation of hamartomas in the brain, heart, eyes, skin, kidneys, and lymphangioleiomyomatosis (LAM) of the lungs. LAM is almost restricted to women in reproductive age. Different mutations in TSC1 and TSC2 genes have been reported in the literature. Here, we present a female patient with TSC-LAM with a novel mutation in TSC2 gene. The patient also had multiple hepatic angiomyolipomas, which is a relatively less-reported manifestation of the disease...
2018: Case Reports in Pulmonology
https://www.readbyqxmd.com/read/29594465/interpreting-body-mri-cases-classic-findings-in-abdominal-mri
#2
REVIEW
Leann Kania, Flavius Guglielmo, Donald Mitchell
Few things in radiology are "pathognomonic" in their appearance or presentation. However, having an awareness of those findings which are specific to a certain entity is important when interpreting imaging studies. These classic findings can be identified with many imaging modalities, but no modality provides as many recognizable observations as an MRI. This results from the large variety of pulse sequences that provide high contrast resolution, prior to and following contrast administration. In this article, the most classically recognized abdominal findings are presented including the following: Liver: Cyst, hemangioma, focal nodular hyperplasia, hepatic adenoma, hemosiderosis, hepatocellular carcinoma...
March 28, 2018: Abdominal Radiology
https://www.readbyqxmd.com/read/29578870/hepatic-epithelioid-angiomyolipoma-and-18f-fdg-pet-ct
#3
Yiqiu Zhang, Beilei Li, Jun Hou, Haojun Yu, Hongcheng Shi
Hepatic epithelioid angiomyolipoma (AML) is rare. Although generally epithelioid AML shows benign biological behavior, it can occasionally have malignant potential. In this study, we report a case of 2 hepatic epithelioid AMLs with different F-FDG uptake; one is intensely FDG avid, and the other is non-FDG avid.
March 24, 2018: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/29512829/hepatic-angiomyolipoma-mutation-analysis-and-immunohistochemical-pitfalls-in-diagnosis
#4
Zhen Yan, James P Grenert, Nancy M Joseph, Chuanli Xu, Xin Chen, Nafis Shafizadeh, Sanjay Kakar
AIMS: Hepatic angiomyolipoma (AML) often shows epithelioid morphology with inconspicuous fat. Epithelioid component can mimic hepatocellular adenoma (HCA) or carcinoma (HCC). The aims were to examine the expression of commonly used markers for HCA or HCC in hepatic AML, and highlight pitfalls in diagnosis. METHODS AND RESULTS: Resected hepatic AML (n=16) were reviewed; reticulin stain, immunohistochemistry for GS, β-catenin, LFABP were performed along with Sanger sequencing of exon 3 of CTNNB1 and next generation sequencing (NGS)...
March 7, 2018: Histopathology
https://www.readbyqxmd.com/read/29392859/hepatic-angiomyolipoma-detected-using-computed-tomography
#5
Yoshihiro Miyazaki, Takashi Kokudo, Amane Takahashi, Katsumi Amikura, Hirohiko Sakamoto
No abstract text is available yet for this article.
February 1, 2018: ANZ Journal of Surgery
https://www.readbyqxmd.com/read/29392818/hepatic-angiomyolipoma-presenting-as-an-arterially-enhancing-liver-lesion
#6
Chun Yuet Khoo, Wei Keat Wan, Brian K P Goh
No abstract text is available yet for this article.
February 1, 2018: ANZ Journal of Surgery
https://www.readbyqxmd.com/read/29361814/angiomyolipoma-of-the-liver-without-a-fat-component-mimicking-a-hepatocellular-carcinoma
#7
Yong Moon Woo, Soo Hyung Ryu, Jeong Wha Min, Mi Ryeong Kim, Tae Young Park, Jeong Seop Moon, Yun Kyung Kang
Angiomyolipoma (AML) is a rare benign mesenchymal tumor in the liver, which is composed of blood vessels, smooth muscle, and adipose cells. The proportion of each component varies, making a diagnosis difficult. This paper reports a case of AML in the liver without adipose tissue, mimicking a hepatocellular carcinoma (HCC), which was diagnosed by a surgical tissue biopsy. A 65-year-old woman was admitted for an evaluation of a hepatic mass that had been detected by ultrasonography. The serologic markers of viral hepatitis B and C were negative...
January 25, 2018: Korean Journal of Gastroenterology, Taehan Sohwagi Hakhoe Chi
https://www.readbyqxmd.com/read/29279500/hepatic-angiomyolipoma-staining-in-the-post-vascular-phase-of-contrast-enhanced-ultrasound-due-to-the-presence-of-macrophages
#8
Kei Endo, Hidekatsu Kuroda, Keisuke Kakisaka, Takayoshi Oikawa, Kei Sawara, Kazuyuki Ishida, Tamotsu Sugai, Yasuhiro Takikawa
A 47-year-old Japanese man was referred to hospital after the detection of a liver tumor. Dynamic computed tomography and Gd-EOB-DTPA-enhanced magnetic resonance imaging were consistent with a diagnosis of hepatocellular carcinoma (HCC). No perfusion defect was observed in the post-vascular phase of contrast-enhanced ultrasound (CEUS). Histopathological staining of the tumor cells was positive for antibodies against HMB-45 and CD68, confirming the diagnosis of hepatic angiomyolipoma (HAML). These findings indicated the presence of macrophages in HAML...
December 27, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29249553/liver-angiomyolipomas-in-tuberous-sclerosis-complex-their-incidence-and-course
#9
Sergiusz Jóźwiak, Krzysztof Sadowski, Julita Borkowska, Dorota Domańska-Pakieła, Dariusz Chmielewski, Elżbieta Jurkiewicz, Maciej Jaworski, Małgorzata Urbańska, Magdalena Ogrodnik, Monika Słowińska, Katarzyna Kotulska
BACKGROUND: The purpose of this study was to evaluate the epidemiology and clinical significance of hepatic angiomyolipomas in patients with tuberous sclerosis complex. METHODS: We performed a retrospective analysis of clinical and imaging data from 187 patients with tuberous sclerosis complex. The prevalence, progression, and potential relationship between liver lesions and other clinical findings, including genetic associations, were assessed. RESULTS: Twenty-eight of 187 patients (14...
January 2018: Pediatric Neurology
https://www.readbyqxmd.com/read/29241297/selecting-a-suitable-surgical-treatment-for-hepatic-angiomyolipoma-a-retrospective-analysis-of-92-cases
#10
Xianwei Yang, Chuanfen Lei, Yiwen Qiu, Shu Shen, Changli Lu, Lunan Yan, Wentao Wang
BACKGROUND: Hepatic angiomyolipoma (HAML) is a rare and difficult-to-diagnose liver tumour. The aim of this study was to summarize experiences in the management of HAML and to recommend a practical treatment strategy. METHODS: We retrospectively studied 92 patients who were diagnosed with HAML and analysed the clinical presentation, histopathological features and treatment of the tumours encountered at our institute from May 2009 to June 2016. RESULTS: The patients included 67 females and 25 males who underwent at least one radiographic examination...
December 14, 2017: ANZ Journal of Surgery
https://www.readbyqxmd.com/read/29102941/angiomyolipoma-of-the-liver-a-rare-benign-tumor-treated-with-a-laparoscopic-approach-for-the-first-time
#11
Christos Damaskos, Nikolaos Garmpis, Anna Garmpi, Afroditi Nonni, Stratigoula Sakellariou, Georgios-Antonios Margonis, Eleftherios Spartalis, Dimitrios Schizas, Nikolaos Andreatos, Eleni Magkouti, Alexandros Grivas, Konstantinos Kontzoglou, Matthew J Weiss, Efstathios A Antoniou
BACKGROUND/AIM: Epithelioid angiomyolipoma of the liver is a rare benign mesenchymal tumor that usually presents in adult female patients. It most frequently occurs in the kidney, with the liver being the second most common site of involvement. Angiomyolipoma belongs to a family of tumors arising from perivascular epithelioid cells, but in rare cases may also have cystic features. We report our experience via the first case of hepatic angiomyolipoma treated by laparoscopic approach. PATIENTS AND METHODS: We present the case of a 50-year-old female patient complaining of abdominal pain...
November 2017: In Vivo
https://www.readbyqxmd.com/read/28809731/the-histopathological-features-and-ct-mri-imaging-performances-in-hepatic-angiomyolipoma-patients
#12
Huiguo Ding, Hongtao Wei, Hui Liu, Yuhan Chen, Xiaowei Xue, Honglei Weng
AIM: To evaluate the diagnostic value of dynamic contrast-enhanced computed tomography (CT) and magnetic resonance imaging (MRI) in the differential diagnosis of hepatic angiomyolipoma (HAML) and hepatocellular carcinoma (HCC) and to clarify the relationship between histopathological features and CT or MRI imaging performances in HAML. MATERIAL AND METHODS: Six HAML and 33 non-cirrhotic HCC patients confirmed by histopathology were retrospectively analyzed. The serum biomarkers, CT and MRI examinations were conventionally performed before the confirmatory histological diagnosis...
September 2017: Annals of Hepatology
https://www.readbyqxmd.com/read/28449573/a-case-of-ruptured-hepatic-angiomyolipoma-in-a-young-male
#13
Sun Hwa Kim, Tae Wook Kang, Kyunghee Lim, Hyun Sung Joh, Jiseok Kang, Dong Hyun Sinn
A 31-year-old male visited a local hospital due to sudden-onset severe abdominal pain. Abdominal computed tomography revealed a solid cystic mass with a size of approximately 12 cm and exhibiting both hemorrhage and fluid collection in the pelvic cavity. Emergency angiography and embolization were performed, and a large hepatic tumor was subsequently surgically resected. The tumor cells stained positive for human melanoma black-45 and smooth-muscle actin, and the pathologic diagnosis was hepatic angiomyolipoma...
June 2017: Clinical and Molecular Hepatology
https://www.readbyqxmd.com/read/28327196/primary-hepatic-angiomyolipoma-immunohistochemistry-and-electron-microscopic-observations-a-case-report
#14
Hidefumi Kubo, Hitoshi Yamazaki, Takemichi Okada, Yoshihito Takahashi, Yatsushi Nishi, Hiroaki Yokomori
BACKGROUND: Hepatic angiomyolipomas are a rare, benign group of mesenchymal tumors in the liver. Hepatic angiomyolipoma is sometimes misdiagnosed as hepatocellular carcinoma, and there is the possibility of a malignant transformation. Hence, the accurate diagnosis of this disorder is necessary. CASE PRESENTATION: A 64-year-old Japanese man was observed to have a space-occupying lesion of 15-mm diameter in the liver during a follow-up examination for a previously resected cecal carcinoma...
March 22, 2017: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/28301898/epithelioid-angiomyolipoma-of-the-liver-a-case-report
#15
Soo Yeon Lee, Baek-Hui Kim
Epithelioid angiomyolipoma (EAML) of liver is a rare neoplasm. Hepatic EAML is often misdiagnosed as other neoplasms such as hepatocellular carcinoma due to non-specific clinical and radiologic features. The morphologic features under microscope and immunohistochemistry staining profile are important in the diagnosis EAML. Here, we report a case of 52-year-old man who found 1.2 cm mass in liver by routine checkup. On the impression of hepatocellular carcinoma, lateral sectionectomy of the liver was done. Microscopically, the tumor is composed of predominant epithelioid cells with vascular component and foamy cells...
March 2017: Clinical and Molecular Hepatology
https://www.readbyqxmd.com/read/28246920/primary-perivascular-epithelioid-cell-tumors-of-the-liver-ct-mri-findings-and-clinical-outcomes
#16
Martin E O'Malley, Tanya P Chawla, Lisa P Lavelle, Sean Cleary, Sandra Fischer
OBJECTIVES: The purpose of our study was to describe the CT and MRI features of primary PEComas of the liver and to document the associated clinical outcomes. METHODS: Retrospective study included 20 patients with primary hepatic perivascular epithelioid cell tumors (PEComa) with pathology and clinical outcomes for correlation. RESULTS: Study group included 20 patients: 16 women, 4 men; mean age 53 (range 35-77) years. Initial pathology diagnoses were classic angiomyolipoma (AML) (n = 11), epithelioid AML (n = 7), and PEComa not otherwise specified (n = 2)...
June 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/28202028/management-of-everolimus-associated-adverse-events-in-patients-with-tuberous-sclerosis-complex-a-practical-guide
#17
REVIEW
Mark Davies, Anurag Saxena, John C Kingswood
Tuberous sclerosis complex (TSC) is a genetic disorder characterised by highly variable comorbid dysfunction and subsequent morbidity. The mTOR inhibitor everolimus is indicated for the treatment of adult TSC patients with renal angiomyolipomas (AMLs) and for subependymal giant astrocytoma (SEGA) in both adults and children, based on data from the EXIST-1 and EXIST-2 trials. However, due to the historical predominance of everolimus in the oncology setting, some physicians who treat TSC patients may be unfamiliar with everolimus-associated adverse events (AEs) and appropriate management strategies...
February 15, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28177188/management-of-hepatic-angiomyolipoma-a-systematic-review
#18
REVIEW
Anne J Klompenhouwer, Danielle Verver, Shiromani Janki, Wichor M Bramer, Michail Doukas, Roy S Dwarkasing, Robert A de Man, Jan N M IJzermans
Hepatic Angiomyolipoma (HAML) is a rare mesenchymal liver tumour assumed to be predominantly benign, although incidental cases with malignant behaviour such as invasive growth, recurrence after resection and metastases have been reported. The aim of this systematic review was to assess the biological behaviour, estimate the risk of HAML related mortality and recommend on a justifiable management strategy. We performed a systematic literature search in Embase, Medline, Web-of-Science, Scopus, Pubmed Publisher, Cochrane and Google Scholar...
February 8, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28122404/clinico-pathological-correlation-of-hepatic-angiomyolipoma-a-series-of-23-resection-cases
#19
Dong Hwan Jung, Shin Hwang, Seung Mo Hong, Ki Hun Kim, Chul Soo Ahn, Deok Bog Moon, Abdulwahab A Alshahrani, Sung Gyu Lee
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...
January 25, 2017: ANZ Journal of Surgery
https://www.readbyqxmd.com/read/28049244/false-positive-diagnosis-of-hepatocellular-carcinoma-in-liver-resection-patients
#20
Hongeun Lee, Jeong Hee Yoon, Hyeyoung Kim, Nam Joon Yi, Suk Kyun Hong, Kyung Chul Yoon, Hyo Sin Kim, Sung Woo Ahn, Jin Young Choi, Youngrok Choi, Hae Won Lee, Ju Yeon Yi, Kyoung Bun Lee, Kwang Woong Lee, Kyung Suk Suh
The diagnosis of hepatocellular carcinoma (HCC) is based on imaging studies particularly in high-risk patients without histologic confirmation. This study evaluated the prevalence and characteristics of false-positively diagnosed HCC in a liver resection cohort for HCC. A retrospective review was performed of 837 liver resection cases for clinically diagnosed HCC between 2005 and 2010 at our institute. High-risk patients with tumors > 1 cm with one or two image findings consistent with HCC and tumors < 1 cm with two or more image findings consistent with HCC with persistently increased serum alpha-fetoprotein (AFP) levels above the normal range with underlying inhibited hepatitis activity underwent liver resection...
February 2017: Journal of Korean Medical Science
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