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Angiomyolipoma

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https://www.readbyqxmd.com/read/27932168/spindle-cell-lipoma-in-an-end-stage-renal-allograft-case-report
#1
Z Zhao, S Selvarajan, A L S Tiong, T H Lim, L Y Khor
BACKGROUND: Spindle cell lipoma is an uncommon variant of lipoma and usually occurs as a solitary, subcutaneous, and well circumscribed lesion in the posterior neck, shoulders, and back of older men. Primary renal lipomas are rarely reported. Spindle cell lipoma in the kidney has not been previously described in the literature. CASE REPORT: A 60-year-old Chinese man suffered graft failure 10 years after living related donor kidney transplantation. During cancer surveillance, he was found to have a mass in the renal allograft, which increased in size and was suspicious for renal cell carcinoma on computerized tomographic scan...
November 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27907099/analysis-of-a-mouse-skin-model-of-tuberous-sclerosis-complex
#2
Yanan Guo, John R Dreier, Juxiang Cao, Heng Du, Scott R Granter, David J Kwiatkowski
Tuberous Sclerosis Complex (TSC) is an autosomal dominant tumor suppressor gene syndrome in which patients develop several types of tumors, including facial angiofibroma, subungual fibroma, Shagreen patch, angiomyolipomas, and lymphangioleiomyomatosis. It is due to inactivating mutations in TSC1 or TSC2. We sought to generate a mouse model of one or more of these tumor types by targeting deletion of the Tsc1 gene to fibroblasts using the Fsp-Cre allele. Mutant, Tsc1ccFsp-Cre+ mice survived a median of nearly a year, and developed tumors in multiple sites but did not develop angiomyolipoma or lymphangioleiomyomatosis...
2016: PloS One
https://www.readbyqxmd.com/read/27897036/computed-tomography-and-magnetic-resonance-findings-of-fat-poor-angiomyolipomas
#3
Aaron Potretzke, Theodora A Potretzke, Tyler Marques Bauman, B Alexander Knight, Alyssa Park, Jonathan M Mobley, Robert S Figenshau, Cary Siegel
INTRODUCTION: Approximately 5% of angiomyolipomas (AMLs) are classified as "fat poor" due to lack of visually detectable fat on imaging, making them difficult to distinguish from renal cell carcinoma. Recent investigations have proposed computed tomography (CT) and magnetic resonance (MR) imaging features suggestive of fat-poor AML (fp-AML). Herein, we determined the frequency of these features in a cohort of fp-AMLs by retrospective review of preoperative imaging. METHODS: A pathology database query from January 2005 to August 2013 identified 49 renal specimens of AML with available imaging...
November 29, 2016: Journal of Endourology
https://www.readbyqxmd.com/read/27891407/successful-pregnancy-outcome-in-a-patient-with-solitary-kidney-affected-by-angiomyolipoma-a-rare-case
#4
Vineet Vashistha Mishra, Kavita Mistry, Sakshi Nanda, Sumesh Choudhary, Khushali Gandhi
Renal angiomyolipoma is a rare benign tumour and its occurrence during pregnancy is even rare. It is usually diagnosed incidentally. It can increase in size during pregnancy and can present acutely as rupture with retroperitoneal haemorrhage, mechanism of which is still unclear. We present a case of successful pregnancy outcome in a patient with congenital solitary kidney affected by angiomyolipoma, diagnosed incidentally at 19 years of age. The patient had conceived twice. Her antenatal and post partum period was uneventful both the times...
October 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27890423/hepatic-epithelioid-angiomyolipoma-clinical-features-and-imaging-findings-of-contrast-enhanced-ultrasound-and-ct
#5
Y Tan, X Xie, Y Lin, T Huang, G Huang
AIM: To evaluate the clinical features and imaging findings of epithelioid angiomyolipoma (EAML) on contrast-enhanced ultrasound (CEUS) and contrast-enhanced computed tomography (CECT). MATERIALS AND METHODS: During January 2006 to November 2015, six patients with histopathologically proven hepatic EAML were evaluated using both CECT and ultrasonography. Six patients underwent CEUS and four patients had CECT images. Patients' demographics, clinical presentations, laboratory test results, and imaging appearances were collected and analysed...
November 24, 2016: Clinical Radiology
https://www.readbyqxmd.com/read/27865795/hepatic-angiomyolipomas-may-overexpress-tfe3-but-have-no-relevant-genetic-alterations
#6
Naoe Jimbo, Takashi Nishigami, Masayuki Noguchi, Hiroko Iijima, Seiichi Hirota, Takuma Tajiri, Takeshi Inoue, Takanori Hirose, Tomoo Itoh, Yoh Zen
The fusion or amplification of TFE3 has been identified as one of the molecular events underlying tumorigenesis in perivascular epithelioid cell tumors (PEComas). TFE3 rearrangements in PEComas are related to the morphological features of the epithelioid appearance and weaker expression of immunohistochemical muscular markers. This study aimed to clarify whether these genetic alterations are involved in hepatic angiomyolipomas (AMLs), which are a member of the PEComa tumor family. We examined 28 liver specimens (15 biopsies and 13 surgical specimens) of hepatic AMLs obtained from 26 patients...
November 16, 2016: Human Pathology
https://www.readbyqxmd.com/read/27859853/monoexponential-biexponential-and-stretched-exponential-diffusion-weighted-imaging-models-quantitative-biomarkers-for-differentiating-renal-clear-cell-carcinoma-and-minimal-fat-angiomyolipoma
#7
Haojie Li, Lili Liang, Anqin Li, Yao Hu, Daoyu Hu, Zhen Li, Ihab R Kamel
PURPOSE: To determine the utility of various diffusion parameters obtained from monoexponential, biexponential, and stretched exponential diffusion-weighted imaging (DWI) models in differentiating between minimal fat angiomyolipoma (MFAML) and clear cell renal cell carcinoma (ccRCC). MATERIALS AND METHODS: One hundred thirty-one patients with pathologically confirmed MFAML (n = 27) or ccRCC (n = 104) underwent multi-b value DWI (0∼1700 s/mm(2) ) imaging at 3...
November 10, 2016: Journal of Magnetic Resonance Imaging: JMRI
https://www.readbyqxmd.com/read/27853915/renal-cell-carcinoma-applicability-of-the-apparent-coefficient-of-the-diffusion-weighted-estimated-by-mri-for-improving-their-differential-diagnosis-histologic-subtyping-and-differentiation-grade
#8
Yulian Mytsyk, Ihor Dutka, Yuriy Borys, Iryna Komnatska, Iryna Shatynska-Mytsyk, Ammad Ahmad Farooqi, Katarina Gazdikova, Martin Caprnda, Luis Rodrigo, Peter Kruzliak
BACKGROUND: Renal cell carcinoma (RCC) represents the most common malignant epithelial neoplasm of the kidney. Accurate assessment of the renal masses, defining the histologic subtype and the grade of differentiation of the tumor, is vital to ensure an adequate case management as well as for staging and prognosis. Recently, diffusion-weighted imaging (DWI) magnetic resonance imaging (MRI) tends to be increasingly appealing for the clinicians as an imaging procedure of choice for the diagnosis and staging of the RCC, which is predetermined by several advantages over CT...
November 16, 2016: International Urology and Nephrology
https://www.readbyqxmd.com/read/27845698/laparoscopic-surgery-in-great-adrenal-malignant-masses
#9
Miguel Ángel Arrabal-Polo, Paolo Fabiano, Almudena Moreno, María Carmen Cano-García, Jaime Jorge, Rafael Rosado
OBJECTIVE: The aim of this article is to describe the surgical technique of laparoscopic resection in malignant tumors greater than 10 cm. METHODS: We present two cases, a 63 year old woman with a left adrenal heterogeneous mass of 11 cm in maximum diameter and an 80 year old man with a left adrenal heterogeneous mass 13 cm in maximum diameter. In both cases excision was performed laparoscopically using 4 trocars and the Alexis® wound retractor for specimen extraction...
November 2016: Archivos Españoles de Urología
https://www.readbyqxmd.com/read/27845102/re-can-quantitative-ct-texture-analysis-be-used-to-differentiate-fat-poor-renal-angiomyolipoma-from-renal-cell-carcinoma-on-unenhanced-ct-images
#10
https://www.readbyqxmd.com/read/27833825/epidemiology-pathogenesis-and-diagnosis-of-lymphangioleiomyomatosis
#11
Angelo M Taveira-DaSilva, Joel Moss
INTRODUCTION: Lymphangioleiomyomatosis (LAM) is a disease of women characterized by cystic lung destruction, lymphatic involvement, and renal angiomyolipomas. AREAS COVERED: LAM is caused by proliferation of abnormal smooth muscle-like LAM cells containing mutations and perhaps epigenetic modifications of the TSC1 or TSC2 genes, which encode, respectively, hamartin and tuberin, two proteins controlling the mechanistic target of rapamycin (mTOR) signaling pathway...
2016: Expert Opinion on Orphan Drugs
https://www.readbyqxmd.com/read/27833401/comparison-of-biexponential-and-monoexponential-model-of-diffusion-weighted-imaging-for-distinguishing-between-common-renal-cell-carcinoma-and-fat-poor-angiomyolipoma
#12
Yuqin Ding, Mengsu Zeng, Shengxiang Rao, Caizhong Chen, Caixia Fu, Jianjun Zhou
OBJECTIVE: To compare the diagnostic accuracy of intravoxel incoherent motion (IVIM)-derived parameters and apparent diffusion coefficient (ADC) in distinguishing between renal cell carcinoma (RCC) and fat poor angiomyolipoma (AML). MATERIALS AND METHODS: Eighty-three patients with pathologically confirmed renal tumors were included in the study. All patients underwent renal 1.5T MRI, including IVIM protocol with 8 b values (0-800 s/mm(2)). The ADC, diffusion coefficient (D), pseudodiffusion coefficient (D(*)), and perfusion fraction (f) were calculated...
November 2016: Korean Journal of Radiology: Official Journal of the Korean Radiological Society
https://www.readbyqxmd.com/read/27824487/small-4-cm-renal-tumors-with-predominantly-low-signal-intensity-on-t2-weighted-images-differentiation-of-minimal-fat-angiomyolipoma-from-renal-cell-carcinoma
#13
Jung Jae Park, Chan Kyo Kim
OBJECTIVE: The purpose of this study was to retrospectively investigate the utility of multiparametric MRI in differentiating minimal-fat angiomyolipoma (AML) from renal cell carcinoma (RCC) in small renal tumors with predominantly low signal intensity on T2-weighted MR images. MATERIALS AND METHODS: Fifty-six patients with pathologically identified renal tumors (1-4 cm) with predominantly low signal intensity on T2-weighted images without visible fat on unenhanced CT images were enrolled...
November 8, 2016: AJR. American Journal of Roentgenology
https://www.readbyqxmd.com/read/27821035/a-hitherto-undescribed-benign-mesenchymal-polyp-of-the-gallbladder-edematous-angiomyolipoma-like-polyp
#14
R Arcega, J X Wu, S Magaki, T R Donahue, H L Wang
We report a case of two peculiar gallbladder polyps in a sixty-four year old male who presented with symptomatic cholelithiasis. Cholecystectomy was performed, which revealed two polyps measuring 0.6 cm and 1.9 cm, located in the body of the gallbladder. Microscopic examination of the polyps showed composite mesenchymal lesions with vascular proliferation of small-to-medium sized arterioles, myoid stroma, and lipomatous periphery. The myoid component was characterized by wisps of bland smooth muscle fibers loosely separated by proteinaceous and focally myxoid matrix...
July 2016: Acta Gastro-enterologica Belgica
https://www.readbyqxmd.com/read/27809914/efficacy-and-safety-of-everolimus-in-children-with-tsc-associated-epilepsy-pilot-data-from-an-open-single-center-prospective-study
#15
Sharon Samueli, Klaus Abraham, Anastasia Dressler, Gudrun Gröppel, Angelika Mühlebner-Fahrngruber, Theresa Scholl, Gregor Kasprian, Franco Laccone, Martha Feucht
BACKGROUND: Epilepsy occurs in up to 90 % of all individuals with tuberous sclerosis complex (TSC). In 67 % disease onset is during childhood. In ≥ 50 % seizures are refractory to currently available treatment options. The mTOR-Inhibitor Everolimus (Votubia®) was approved for the treatment of subependymal giant cell astrocytoma (SEGA) and renal angiomyolipoma (AML) in Europe in 2011. It's anticonvulsive/antiepileptic properties are promising, but evidence is still limited. Study aim was to evaluate the efficacy and safety of Everolimus in children and adolescents with TSC-associated epilepsies...
November 3, 2016: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/27805457/laparoscopic-radiofrequency-ablation-combined-with-surgical-excision-for-exophytic-renal-angiomyolipoma-a-novel-technique-based-on-tumor-vasculature-features-of-enhancing-renal-masses-toward-hilar-off-clamping-nephron-sparing-surgery
#16
Wei Xiong, Qing Ran, Yangchun Du, Ji Lv, Fang Chen, Shan Zhong, Pu Guo, Ke Dou, Minghan Sun
OBJECTIVES: Symptomatic angiomyolipoma (AML) and asymptomatic AML larger than 4 cm in size are usually treated with nephron-sparing surgery or transarterial embolization. We used radiofrequency ablation to treat the vascular pedicle of exophytic AML with low R.E.N.A.L. nephrometry score and investigated its feasibility for hilar off-clamping nephron-sparing surgery. METHODS: Contrast-enhanced computed tomography (CT) showed enhanced, well-defined lipomatous tumors with a maximum diameter of 4-8 cm in the kidney of 15 patients...
November 2, 2016: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
https://www.readbyqxmd.com/read/27805337/tuberous-sclerosis-with-disseminated-malignancy
#17
Achintya S, Ragesh R, Neeraj Nischal, Manish Soneja, Aanchal Kakkar, Mehar C Sharma, Geethu Anthony, Surabhi Vyas, S K SharmaSenior Professor And Head Department Of Medicine All India Institute Of Medical Sciences Ansari Nagar New Delhi
Tuberous sclerosis is characterized by typical skin and clinical manifestations with predilection to neoplasia. We describe the case of a 42-year old female who presented with a mass and pain in right lumbar region with constitutional symptoms and generalized body aches since last one year. She had adenoma sebaceum, subungual fibromas and hepatomegaly. CT chest, abdomen and MRI revealed mass in the right renal fossa with wide spread extensions in the abdomen, left renal angiomyolipomas (AMLs) and intracerebral lesions...
November 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27803081/renal-neuroendocrine-tumour-and-synchronous-pancreas-metastasis-histopathological-diagnosis-using-prostatic-acid-phosphatase
#18
Keishi Kawasaki, Yoshikuni Kawaguchi, Yoshio Suzuki, Nobutaka Tanaka
A woman aged 56 years developed 2 synchronous tumours: one, 1.2 cm in diameter at the head of the pancreas; and the other, 4.0 cm in diameter, at the left side of her horseshoe kidney. Preoperative differential diagnosis of these hypovascular lesions included pancreatic ductal carcinoma (PDC) with renal metastasis, PDC with renal angiomyolipoma, renal cell carcinoma with pancreatic metastasis or PDC and renal cell carcinoma. Following pancreaticoduodenectomy and left nephrectomy, both specimens were diagnosed as grade 2 neuroendocrine tumours (NETs)...
November 1, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27800047/expanding-living-kidney-donor-criteria-with-ex-vivo-surgery-for-renal-anomalies
#19
Thomas B McGregor, Christie Rampersad, Premal Patel
INTRODUCTION: Renal transplantation remains the gold standard treatment for end-stage renal disease, with living donor kidneys providing the best outcomes in terms of allograft survival. As the number of patients on the waitlist continues to grow, solutions to expand the donor pool are ongoing. A paradigm shift in the eligibility of donors with renal anomalies has been looked at as a potential source to expand the living donor pool. We sought to determine how many patients presented with anatomic renal anomalies at our transplant centre and describe the ex-vivo surgical techniques used to render these kidneys suitable for transplantation...
September 2016: Canadian Urological Association Journal, Journal de L'Association des Urologues du Canada
https://www.readbyqxmd.com/read/27797585/beneficial-effects-of-everolimus-on-autism-and-attention-deficit-hyperactivity-disorder-symptoms-in-a-group-of-patients-with-tuberous-sclerosis-complex
#20
Ayse Kilincaslan, Burcu Ece Kok, Pinar Tekturk, Cengiz Yalcinkaya, Cigdem Ozkara, Zuhal Yapici
OBJECTIVES: Such neuropsychiatric symptoms as autism spectrum disorders, attention-deficit/hyperactivity disorder (ADHD), intellectual disability, aggression, and epilepsy are very common in patients with tuberous sclerosis complex (TSC). Everolimus, a mammalian target of rapamycin (mTOR) inhibitor, is a recent and effective treatment for TSC patients with giant cell astrocytomas and renal angiomyolipoma, and it has been shown to have a potential to reduce tumor volume. However, there is a paucity of studies on the effects of everolimus on neuropsychiatric symptoms...
October 31, 2016: Journal of Child and Adolescent Psychopharmacology
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