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Orphanet J Rare Dis 2021 Jun 2;16(1):250. Epub 2021 Jun 2.

Epilepsy Center Frankfurt Rhine-Main, Department of Neurology, Goethe-University Frankfurt, Schleusenweg 2-16, 60528, Frankfurt am Main, Germany.

Background: Tuberous sclerosis complex (TSC) is a monogenetic, multisystem disorder characterized by benign growths due to TSC1 or TSC2 mutations. This German multicenter study estimated the costs and related cost drivers associated with organ manifestations in adults with TSC.

Methods: A validated, three-month, retrospective questionnaire assessed the sociodemographic and clinical characteristics, organ manifestations, direct, indirect, out-of-pocket (OOP), and nursing care-level costs among adult individuals with TSC throughout Germany from a societal perspective (costing year: 2019). Read More

Front Oncol 2021 12;11:691996. Epub 2021 May 12.

Department of Urology, Osaka University Graduate School of Medicine, Osaka, Japan.

Tuberous sclerosis complex is a genetic disorder characterized by facial angiofibromas, intellectual disability, epilepsy, and tumor formation in multiple organs, including the kidney. Renal cell carcinoma occurs in 2%-4% of patients with tuberous sclerosis complex, often developing multiply and bilaterally. Renal cell carcinoma associated with this genetic disorder may include complex tumor heterogeneity caused by the spatially different mutational landscape. Read More

Radiol Clin North Am 2021 Jul;59(4):631-646

Department of Radiology, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, 500 Hofstra Boulevard, Hempstead, NY 11549, USA.

Renal masses are commonly encountered on cross-sectional imaging examinations performed for nonrenal indications. Although most can be dismissed as benign cysts, a subset will be either indeterminate or suspicious; in many cases, imaging cannot be used to reliably differentiate between benign and malignant masses. On-going research in defining characteristics of common renal masses on advanced imaging shows promise in offering solutions to this issue. Read More

Urol Case Rep 2021 Sep 2;38:101693. Epub 2021 May 2.

University of Uyo Teaching Hospital, Uyo, Nigeria.

Giant Hydronephrosis in the adult can reach elephantoid proportions, containing several liters of fluid. It can be associated with rare renal neoplasms. We present a 27 year old man with progressive painless abdominal distention whose imaging investigations (ultrasound and CT scan) disclosed a left GH. Read More

J Int Med Res 2021 May;49(5):3000605211016193

Department of Radiology, School of Medicine, Juntendo University, Tokyo, Japan.

Objective: This study investigated the efficacy and safety of superselective transcatheter arterial embolization for angiomyolipoma at the renal hilum.

Methods: Between August 2012 and January 2015, 13 patients with 16 angiomyolipomas at the renal hilum underwent initial, prophylactic, superselective transcatheter arterial embolization. The patients were followed by computed tomography or magnetic resonance imaging, and volume-reduction ratios after embolization were measured. Read More

Ann R Coll Surg Engl 2021 Jun 6;103(6):e184-e188. Epub 2021 May 6.

Maulana Azad Medical College, New Delhi, India.

Angiomyolipoma is a benign solid renal neoplasm. A giant angiomyolipoma is more than 10cm by size, but it can grow to huge proportions. Our case appears to be the third largest angiomyolipoma and the largest among bilateral giant renal angiomyolipoma in the indexed literature. Read More

Arch Esp Urol 2021 May;74(4):457-458

Servicio de Radiodiagnóstico. Hospital Universitario Central de Asturias. Oviedo. Asturias. España.

Surg Case Rep 2021 May 3;7(1):110. Epub 2021 May 3.

Department of Surgery, Tohoku Rosai Hospital, 4-3-21, Dainohara, Aobaku, Sendai, Miyagi, 981-8563, Japan.

Background: Angiomyolipoma is a benign mesenchymal tumor that develops commonly in the kidney and rarely in other organs. The involvement of the spleen in angiomyolipoma is extremely rare, and only one such case has been reported in the English literature.

Case Presentation: A 27-year-old man presented with adenoid hyperplasia and bilateral palatal tonsillar hyperplasia. Read More

Urol Ann 2021 Jan-Mar;13(1):67-72. Epub 2021 Jan 19.

Department of Internal Medicine, Division of Nephrology, University of Mississippi Medical Center, Jackson, Mississippi, USA.

Background: Renal angiomyolipoma (AML) is the most frequent mesenchymal tumor of the kidney. Although there is a rare possibility of malignant transformation of AML, this risk has not been studied in immunosuppressed patients. The safety of donors with AML and their kidney transplant recipients has not been well established. Read More

Urol Case Rep 2021 Sep 19;38:101645. Epub 2021 Mar 19.

Department of Urology, San Luca Hospital, Via Francesco Cammarota 1, 84078, Vallo della Lucania, Italy.

A male patient, 40 years of age, arrived at our Institute with diffuse abdominal tenderness, right flank pain, hematuria and early stage of hemorrhagic shock with anemia and initial hypotension. The immediate clinical history revealed no significant previous trauma, only subsequently was reported inconstant pain in the right flank for 4-5 days with pallor and asthenia, signs and symptoms that the patient had not investigated. Abdominal CT scan with angiographic evaluation was performed showing right kidney mass and perirenal fluid collection by blood component. Read More

BMC Nephrol 2021 Apr 9;22(1):127. Epub 2021 Apr 9.

Department of Urology, Peking Union Medical College Hospital, Chinese Academy of Science, Beijing, China.

Background: Renal tumors during pregnancy are rare and the treatment requires evaluation of both the patient and the fetus. No consensus or guidelines has been proposed or verified in this field. We successfully managed three renal tumor cases during pregnancy and reviewed the relative literature. Read More

Balkan J Med Genet 2020 Nov 23;23(2):93-98. Epub 2021 Mar 23.

University Clinic of Nephrology, Skopje, Republic of North Macedonia.

Tuberous sclerosis complex (TSC) is an autosomal-dominant multi system disorder. The genetic basis of the disorder is mutations in the or gene, which leads to over activation of the mammalian target of rapamycin (mTOR) protein complex and results in development of benign tumors in different body systems such as brain, skin, lungs and kidney. The mTOR inhibitors are presently the main treatment option for patients with TSC. Read More

J Int Med Res 2021 Mar;49(3):3000605211001710

Department of Urology, The First Hospital of Jilin University, Changchun, Jilin, P.R. China.

Angiomyolipoma (AML) is a benign tumor that mainly occurs in the kidneys. Simultaneous involvement of the kidney and local regional lymph nodes is very rare and might be misdiagnosed as a metastasizing malignant cancer. In the present study, a 50-year-old woman was referred to our hospital after a routine health screening ultrasound. Read More

Urol Ann 2020 Oct-Dec;12(4):392-393. Epub 2020 Oct 15.

Department of Urology, Pakistan Kidney and Liver Institute and Research Centre, Lahore, Pakistan.

Wunderlich syndrome is a rarely entity. We report our case of a 60-year-old female, who presented in the emergency medicine department with acute right flank pain, tender mass right upper quadrant abdomen, hypotension, and visible hematuria. Urgent computerized tomogram confirmed bleeding in the right renal angiomyolipoma. Read More

World J Clin Cases 2021 Mar;9(8):1931-1939

Department of Radiology, Shandong Medical Imaging Research Institue, Cheeloo College of Medicine, Shandong University, Jinan 250021, Shandong Province, China.

Background: Angiomyolipomas (AMLs), belonging to the family of mesenchymal tumors, are considered benign lesions that occur mostly in the kidney or as a part of tuberous sclerosis. Epithelioid AML (EAML) is a rare type of AML that appears to have malignant potential. Extrarenal AMLs usually occur in the liver according to the retrieved literature reports. Read More

Am J Emerg Med 2021 Feb 12. Epub 2021 Feb 12.

Department of Emergency Medicine, Naval Medical Center San Diego, 34800 Bob Wilson Drive, San Diego, CA 92134, United States.

While primarily a respiratory illness, infection with the novel coronavirus (COVID-19) is associated with pathologic changes in coagulation, characterized by both thromboembolic and bleeding events. We present the case of a 22-year-old female diagnosed with renal angiomyolipoma (AML) rupture 2 weeks after COVID-19 infection, ultimately requiring admission for hemorrhage control via endovascular embolization. Emergency medicine physicians should maintain a high index of suspicion for renal AML rupture and other spontaneous bleeding events in patients with recent COVID-19 infection due to a possible correlation between the two. Read More

BMJ Case Rep 2021 Mar 18;14(3). Epub 2021 Mar 18.

Urology, Sengkang General Hospital, Singapore.

Synovial sarcomas are most commonly localised in extremities, especially in the lower thigh and knee areas. Comprising less than 1% of all malignancies, retroperitoneal synovial sarcoma is very rare with primary synovial sarcoma of the kidney being even more infrequent and difficult to diagnose. We describe a case report of a renal synovial sarcoma in a young adult who was initially managed as a case of Wunderlich's syndrome secondary to what was believed to be a ruptured renal angiomyolipoma. Read More

BMJ Case Rep 2021 Mar 16;14(3). Epub 2021 Mar 16.

Department of Internal Medicine, School of Medicine, Texas Tech University Health Sciences Center, Odessa, Texas, USA.

Renal angiomyolipomas (AMLs) were first described in the early 1900s by Gravitz, but it was not until 1951 that they were named renal AML. These kidney tumours are rare, occurring in 0.13%-0. Read More

Abdom Radiol (NY) 2021 Mar 6. Epub 2021 Mar 6.

Department of Radiology, Hospital Clínic, Villarroel 170, 08036, Barcelona, Spain.

Purpose: To identify specific contrast-enhanced CT (CECT) findings and develop a predictive model with logistic regression to differentiate fat-poor angiomyolipomas (fpAML) from papillary renal cell carcinomas (pRCC).

Methods: This is a single-institution retrospective study that assess CT features of histologically proven 67 pRCC and 13 fpAML. CECT variables were studied by means of univariate logistic regression. Read More

Korean J Radiol 2021 05 9;22(5):735-741. Epub 2021 Feb 9.

Department of Radiology, Chungnam National University Hospital, Daejeon, Korea.

Objective: To evaluate circularity as a quantitative shape factor of small renal tumor on computed tomography (CT) in differentiating fat-poor angiomyolipoma (AML) from renal cell carcinoma (RCC).

Materials And Methods: In 257 consecutive patients, 257 pathologically confirmed renal tumors (either AML or RCC less than 4 cm), which did not include visible fat on unenhanced CT, were retrospectively evaluated. A radiologist drew the tumor margin to measure the perimeter and area in all the contrast-enhanced axial CT images. Read More

Abdom Radiol (NY) 2021 Mar 3. Epub 2021 Mar 3.

School of Information and Electronics, Beijing Institute of Technology, Beijing, 100081, China.

Purpose: With advancements in medical imaging, more renal tumors are detected early, but it remains a challenge for radiologists to accurately distinguish subtypes of renal parenchymal tumors. We aimed to establish a novel deep convolutional neural network (CNN) model and investigate its effect on identifying subtypes of renal parenchymal tumors in T2-weighted fat saturation sequence magnetic resonance (MR) images.

Methods: This retrospective study included 199 patients with pathologically confirmed renal parenchymal tumors, including 77, 46, 34, and 42 patients with clear cell renal cell carcinoma (ccRCC), chromophobe renal cell carcinoma (chRCC), angiomyolipoma (AML), and papillary renal cell carcinoma (pRCC), respectively. Read More

Pediatr Neurol 2021 Apr 26;117:21-26. Epub 2020 Dec 26.

Division of Pediatric Nephrology, University of Iowa Stead Family Department of Pediatrics, University of Iowa, Iowa City, Iowa.

Background: Kidney disease has historically been the primary source of early mortality in adults with tuberous sclerosis complex (TSC). Kidney imaging surveillance promotes early detection of lesions requiring intervention. We describe kidney imaging frequency in relationship to patient-level characteristics for commercially insured patients with TSC in the United States. Read More

Actas Urol Esp (Engl Ed) 2021 May 23;45(4):264-272. Epub 2021 Feb 23.

Unidad de Uro-Oncología, Servicio Urología, Hospital Universitario Puerta del Mar, INIBICA (Instituto de Investigación e Innovación Biomédica), Cádiz, España.

Introduction: Renal angiomyolipoma is a frequent manifestation of Tuberous Sclerosis Complex (TSC), for which everolimus therapy has been recently established as a novel non-invasive therapeutic option. As there are limited real life and long-term data, the analysis of our experience provides added value in terms of safety and efficacy.

Material And Methods: Descriptive analysis of our experience in patients with giant bilateral renal angiomyolipomas, in the context of TSC, treated with 10 mg oral everolimus daily, during a median of 71. Read More

Mol Clin Oncol 2021 Mar 21;14(3):52. Epub 2021 Jan 21.

Department of Urology, The Fifth Affiliated Hospital of Guangzhou Medical University, Guangzhou, Guangdong 510700, P.R. China.

Renal angiomyolipoma (RAML), also referred to as renal hamartoma, is a rare benign tumor. There are two types of RAML, which include the tuberous sclerosis complex (TSC)-associated type and the sporadic type. TSC is an autosomal dominant genetic disease characterized by the growth of benign tumors in the skin, brain, kidneys, lung and heart. Read More

Seizure 2021 Mar 30;86:82-84. Epub 2021 Jan 30.

Department of Pediatric Neurology, Fukuoka Children's Hospital, Fukuoka, Japan.

Eur Radiol 2021 Feb 9. Epub 2021 Feb 9.

Department of Diagnostic Imaging and Nuclear Medicine, Tokyo Women's Medical University, 8-1, Kawada-cho, Shinjuku-ku, Tokyo, 162-8666, Japan.

Objectives: To retrospectively evaluate whether the early dark cortical band (EDCB) on CT can be a predictor to differentiate clear cell renal cell carcinoma (ccRCC) from fat poor angiomyolipoma (Fp-AML) and to detect peritumoral pseudocapsules in ccRCC.

Methods: The EDCBs, which are comprised of unenhanced thin lines at the tumor-renal cortex border in the corticomedullary phase, on the CT images of 342 patients who underwent partial nephrectomy were evaluated. Independent predictors among the clinical and CT findings for differentiating ccRCC from Fp-AML were identified using multivariate analyses. Read More

Radiol Case Rep 2021 Apr 30;16(4):858-862. Epub 2021 Jan 30.

Department of Radiology, Keio University School of Medicine, 35, Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan.

In the 2016 World Health Organization renal tumor classification, the mixed epithelial and stromal tumor family was introduced as a new entity. This family encompasses a spectrum of tumors, ranging from predominantly cystic tumors (adult cystic nephromas) to tumors that are variably solid (mixed epithelial and stromal tumors). The majority of previous studies incorporating "mixed epithelial and stromal tumor" in the titles were actually reports of imaging findings of adult cystic nephroma. Read More

Quant Imaging Med Surg 2021 Feb;11(2):676-684

Department of Radiology, The First Hospital of China Medical University, Shenyang, China.

Background: This study aimed to use the stretched-exponential nonlinear regression analysis model to explore the value of the energy spectral curve in the differential diagnosis of clear cell renal cell carcinoma (ccRCC), minimal fat renal angiomyolipoma (RAML), and hypovascular renal cell carcinoma.

Methods: Sixty-five cases with renal tumors were enrolled retrospectively who had undergone a preoperative multiphase spectral CT scan of the kidney in pre-enhance and double-phase enhanced scanning. The normalized iodine concentrations (NIC) of these lesions, normal renal cortex, and psoas major were measured and calculated. Read More

BMC Nephrol 2021 01 31;22(1):47. Epub 2021 Jan 31.

Department of Urology, University Hospital Basel, Spitalstrasse 21, CH-4031, Basel, Switzerland.

Background: Tuberous Sclerosis Complex (TSC) is a genetic disorder, with renal manifestations like angiomyolipoma (AML) occurring in 70-80% of patients. AML usually cause more complications in TCS patients than in non-TSC patients. However, AML patients are not routinely investigated for TSC. Read More

Pan Afr Med J 2020 2;37:210. Epub 2020 Nov 2.

Department of Pathology and Laboratory Medicine, All India Institute of Medical Sciences, Rishikesh, India.

Angiomyolipoma of the kidney is a common benign mesenchymal neoplasm of kidney. A rare variant, epithelioid angiomyolipoma, however, may show malignant behavior. We report a case of epithelioid angiomyolipoma in a patient not having tuberous sclerosis which was initially misdiagnosed as renal cell carcinoma. Read More