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https://www.readbyqxmd.com/read/28634560/atypical-lipomatous-tumor-well-differentiated-liposarcoma-developed-in-a-patient-with-progressive-muscular-dystrophy-a-case-report-and-review-of-the-literature
#1
Ryo Miyagi, Toshihiko Nishisho, Shinjiro Takata, Yoshimitsu Shimatani, Shunichi Toki, Koichi Sairyo
BACKGROUND: Atypical lipomatous tumor/well-differentiated liposarcoma (ALT/WDLS) is an intermediate or locally aggressive form of adipocytic soft tissue sarcoma. Muscular dystrophy (MD) is characterized by progressive muscle atrophy and its replacement by adipose and fibrous tissue. Recently, some authors have reported that MD genes are related to neoplastic formation, but there have been no detailed clinical reports of ALT associated with MD. CASE PRESENTATION: A 73-year-old woman with a diagnosis of limb-girdle MD visited our department for recurrence of a huge tumor in her left thigh...
2017: Case Reports in Orthopedics
https://www.readbyqxmd.com/read/28627003/sonographic-screening-for-wilms-tumor-in-children-with-cloves-syndrome
#2
Caitlin M Peterman, R Dawn Fevurly, Ahmad I Alomari, Cameron C Trenor, Denise M Adams, Sophie Vadeboncoeur, Marilyn G Liang, Arin K Greene, John B Mulliken, Steven J Fishman
BACKGROUND: CLOVES syndrome is associated with somatic mosaic PIK3CA mutations and characterized by congenital lipomatous overgrowth, vascular malformations, epidermal nevi, and skeletal anomalies. Wilms tumor (WT) is a malignant embryonal renal neoplasm associated with hemihypertrophy and certain overgrowth disorders. After identifying WT in a child with CLOVES, we questioned whether ultrasonographic screening was necessary in these patients. METHODS: We retrospectively reviewed patients with CLOVES syndrome in our Vascular Anomalies Center at Boston Children's Hospital between 1998 and 2016 to identify those who developed WT...
June 19, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28626403/a-twenty-four-year-old-woman-with-left-flank-lipoma-like-hibernoma
#3
R E Shackelford, M Al Shaarani, J Ansari, E Wei, J Cotelingam
A 24-year-old woman presented with a 5-month history of a left flank mass that was painful on palpation. Magnetic resonance imaging revealed a 10.0 × 6.0 × 2.5 cm mass consistent with lipoma. A fatty lobulated mass was excised and subjected to H&E staining and immunohistochemical analyses. The specimen consisted of mature univacuolated adipocytic cells, with intermixed multivacuolated eosinophilic granular cells. No atypia or hyperchromasia was identified. Most of the cells were S100 positive and Ki-67 immunonegative...
May 2017: Case Reports in Oncology
https://www.readbyqxmd.com/read/28620752/fatty-metaplasia-quantification-and-impact-on-regional-myocardial-function-as-assessed-by-advanced-cardiac-mr-imaging
#4
Tomas Lapinskas, Bernhard Schnackenburg, Marc Kouwenhoven, Rolf Gebker, Alexander Berger, Remigijus Zaliunas, Burkert Pieske, Sebastian Kelle
OBJECTIVE: This study aimed to investigate the advantages of recently developed cardiac imaging techniques of fat-water separation and feature tracking to characterize better individuals with chronic myocardial infarction (MI). MATERIALS AND METHODS: Twenty patients who had a previous MI underwent CMR imaging. The study protocol included routine cine and late gadolinium enhancement (LGE) technique. In addition, mDixon LGE imaging was performed in every patient. Left ventricular (LV) circumferential (EccLV) and radial (ErrLV) strain were calculated using dedicated software (CMR(42), Circle, Calgary, Canada)...
June 15, 2017: Magma
https://www.readbyqxmd.com/read/28616637/calcaneal-cysts-and-lipomas-a-common-pathogenesis
#5
REVIEW
Jacques Malghem, Frédéric Lecouvet, Bruno Vande Berg
Calcaneal cysts and lipomas are relatively rare, benign bone lesions. They are similar in many ways, including in their location and radiological appearance, but their content differs. Cysts contain fluid whereas lipomas contain fat, although some lesions may exhibit a mixed content. The pathogenesis of the two entities is the subject of controversy. The theory that calcaneal cysts may result from lipomatous necrosis has been widely suggested in the literature, but no such progression has ever been shown. The contrary hypothesis has also been considered, i...
June 14, 2017: Skeletal Radiology
https://www.readbyqxmd.com/read/28601782/neuropraxia-following-resection-of-a-retroperitoneal-liposarcoma
#6
Stevenson Tsiao, Nail Aydin, Subhasis Misra
BACKGROUND: This is a unique case of neuropraxia of femoral nerve seen after resection of retroperitoneal liposarcoma which has not been reported before in the literature. INTRODUCTION: Neuropraxia, a transient paralysis due to blockage of nerve conduction, commonly associated with athletes and orthopedic procedures, has not been previously reported as a complication following resection of retroperitoneal sarcoma. CASE: This is an 81-year-old female who, on CT for evaluation of her atherosclerosis, was found to have an incidental right-sided retroperitoneal mass extending from the right renal capsule inferiorly through the inguinal canal...
2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28577738/-pik3ca-related-overgrowth-syndrome-pros
#7
Quitterie Venot, Guillaume Canaud
This review presents an overview of a recently characterized spectrum of overgrowth syndrome: phosphoinositide-3 kinase (PI3K)-related overgrowth spectrum (PROS). This spectrum encompasses overgrowth syndromes associated with somatic mosaic activating PIK3CA mutations such as megalencephaly-capillary malformation (MCAP) syndrome, dysplatic megalencephaly (DMEG), congenital lipomatous asymmetric overgrowth of the trunk, lymphatic, capillary, venous, and combined-type vascular malformations, epidermal nevi, skeletal and spinal anomalies (CLOVES) syndrome, hemihyperplasia-multiple lipomatosis (HHML), fibroadipose overgrowth and Klippel-Trenaunay syndrome...
April 2017: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/28573464/imaging-features-of-mammary-type-myofibroblastoma-of-soft-tissue-a-case-series-with-literature-review
#8
Gokhan Kuyumcu, Brian P Rubin, Carl Winalski
Mammary-type myofibroblastoma (MTM) is a rare, benign neoplasm that is histologically identical to myofibroblastoma of the breast, but occurring in an extramammary site. The masses have been reported in superficial and deep soft tissue sites and visceral locations with the inguinal area and lower extremities are the most common. Most previous reports of MTMs have concentrated on clinical and pathological findings, with very limited information regarding imaging characteristics. Here, we describe three cases of MTM diagnosed at our institution, with a focus on imaging findings...
June 1, 2017: Skeletal Radiology
https://www.readbyqxmd.com/read/28571234/cloves-syndrome-severe-neonatal-presentation
#9
Silvana Acosta, Viviana Torres, María Paulos, Ignacio Cifuentes
Congenital Lipomatous Overgrowth, Vascular Malformations, Epidermal Nevi and Spinal Abnormalities (CLOVES syndrome) is a newly described and rare overgrowth disorder with serious morbidity. The course of this disease is not well understood and few cases have been reported among neonates. Moreover, not all of the signs of this syndrome are present at birth, making a high index of suspicion necessary. We present a cohort of three newborns with CLOVES syndrome who died due to septic and hemodynamic complications directly related to extensive vascular malformations...
April 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28553400/proteus-syndrome-with-neurological-manifestations-a-rare-presentation
#10
Pallavi Sachdeva, Priyanka Minocha, Rohit Jain, Sadasivan Sitaraman, Manisha Goyal
Proteus syndrome (PS) is an extremely rare and complex disorder. Approximately 200 cases have been reported, and it seems to affect people of all ethnic and racial groups. PS is characterized by segmental overgrowth of multiple tissues and organs including vascular malformations, lipomatous overgrowth, hyperpigmentation, and various types of nevi. We hereby present a 7-year-old boy who presented with seizures and overgrowth of one-half of the body. Although classical physical features have been described, epilepsy and other neurological manifestations are rarely reported features of PS...
January 2017: Journal of Pediatric Neurosciences
https://www.readbyqxmd.com/read/28507957/arthroscopic-synovectomy-in-bilateral-lipoma-arborescens
#11
Paolo Fornaciari, Pascal A Schai, Michael O Kurrer, G Ulrich Exner
INTRODUCTION: Lipoma arborescens (LA) is an uncommon condition that consists of a villous lipomatous proliferation of the synovial membrane. Open synovectomy has been previously selected as a curative treatment option. In recent years, some authors have published good results with arthroscopic interventions. We describe a well-documented case of bilateral LA of the knees treated with staged arthroscopic synovectomy. CASE REPORT: A 48-year-old North American woman without a history of trauma presented with recurrent effusions and mild pain in both knee joints for many years...
November 2016: Journal of Orthopaedic Case Reports
https://www.readbyqxmd.com/read/28502730/overgrowth-syndromes-caused-by-somatic-variants-in-the-phosphatidylinositol-3-kinase-akt-mammalian-target-of-rapamycin-pathway
#12
REVIEW
Gozde Akgumus, Fengqi Chang, Marilyn M Li
Somatic variants have been well described in tumorigenesis; however, they are only recently appreciated in other human disorders, such as mosaic overgrowth syndromes. Although overgrowth is a manifestation in many genetic syndromes, not all overgrowth syndromes are inherited. Mosaic somatic variants have been lately described in several overgrowth disorders, such as Proteus syndrome, CLOVES (congenital, lipomatous, overgrowth, vascular malformations, epidermal nevi, and spinal/skeletal anomalies and/or scoliosis) syndrome, megalencephaly-polymicrogyria-polydactyly-hydrocephalus syndrome, and megalencephaly-capillary malformation-polymicrogyria syndrome...
May 11, 2017: Journal of Molecular Diagnostics: JMD
https://www.readbyqxmd.com/read/28502725/molecular-diagnosis-of-mosaic-overgrowth-syndromes-using-a-custom-designed-next-generation-sequencing-panel
#13
Fengqi Chang, Liu Liu, Erica Fang, Guangcheng Zhang, Tiansheng Chen, Kajia Cao, Yanchun Li, Marilyn M Li
Recent studies have discovered a group of overgrowth syndromes, such as congenital lipomatous overgrowth with vascular, epidermal, and skeletal anomalies (CLOVES) syndrome, Proteus syndrome, and megalencephaly-capillary malformation-polymicrogyria (MCAP) syndrome, are caused by somatic activating variants in the genes involved in the phosphatidylinositol 3-kinase/AKT/mechanistic target of rapamycin pathway. Because of the low-abundance nature of these pathogenic variants, Sanger sequencing often yields negative results...
May 11, 2017: Journal of Molecular Diagnostics: JMD
https://www.readbyqxmd.com/read/28501824/the-vanishing-duodenal-polyp-mesenteric-invagination-presenting-as-duodenal-pseudopolyp
#14
Bernadette S de Bakker, Saffire S K S Phoa, Mohammed Kara, Sabaa Al-Eryani, Marieke E Gielen, Cyriel I J Ponsioen, Henri M de Bakker, Thomas M van Gulik
Duodenal polypoid masses are an uncommon finding mainly diagnosed incidentally at endoscopy or surgery. We report a 39-year-old female patient with symptoms of intermittent stabbing pain in the upper right abdominal quadrant and an iron deficiency anaemia, without complaints of weight loss, haematemesis or melaena. A duodenal polyp and acute duodenitis have been described during endoscopic examinations and CT and ultrasound. Surgical excision of the polyp was advised. Intraoperatively, an elongated duodenum was remarkable; however, at duodenotomy, no polyp was found, nor during intraoperative endoscopy...
May 13, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28485184/lipomatous-hypertrophy-of-the-interatrial-septum
#15
Lindsay Alpert, Tatjana Antic
No abstract text is available yet for this article.
May 1, 2017: International Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28474576/imaging-review-of-lipomatous-musculoskeletal-lesions
#16
Ashley M Burt, Brady K Huang
Lipomatous lesions are common musculoskeletal lesions that can arise within the soft tissues, bone, neurovascular structures, and synovium. The majority of these lesions are benign, and many of the benign lesions can be diagnosed by radiologic evaluation. However, radiologic differences between benign and malignant lipomatous lesions may be subtle and pathologic correlation is often needed. The use of sonography, computed tomography (CT), and magnetic resonance imaging (MRI) is useful not only in portraying fat within the lesion, but also for evaluating the presence and extent of soft tissue components...
2017: SICOT-J
https://www.readbyqxmd.com/read/28466126/uncommon-cranial-meningioma-key-imaging-features-on-conventional-and-advanced-imaging
#17
REVIEW
Nader Zakhari, Carlos Torres, Mauricio Castillo, Thanh B Nguyen
Given the high incidence of intracranial meningiomas encountered in clinical practice, it is not uncommon to find rare subtypes of meningioma, with unusual imaging findings. These commonly represent a diagnostic challenge. In this article, we review the imaging appearance of typical meningioma on conventional and advanced imaging as well as the key imaging features of multiple uncommon subtypes: cystic, microcystic, lipomatous, chordoid, angiomatous, intraosseous, extracranial, atypical/malignant, and tumor-to-tumor metastasis (also known as collision tumors)...
May 2, 2017: Clinical Neuroradiology
https://www.readbyqxmd.com/read/28438867/magnetic-resonance-imaging-assessment-of-lipomatous-soft-tissue-tumors
#18
Alessandro Coran, Paolo Ortolan, Shady Attar, Enrico Alberioli, Egle Perissinotto, Anna Lisa Tosi, Maria Cristina Montesco, Carlo Riccardo Rossi, Saveria Tropea, Marco Rastrelli, Roberto Stramare
AIM: To establish the accuracy of magnetic resonance imaging (MRI) in distinguishing between benign and malignant lipomatous tumors; to evaluate the reproducibility of the MRI interpretation assessing the agreement between judgments of two radiologists with the same experience in soft-tissue sarcomas; to identify an association among MRI findings (size, depth, septa, nodules, signal homogeneity) and nature of the lesion. MATERIALS AND METHODS: A total of 54 patients (28 men and 26 women), with a mean age of 56 (range=27-84) were included years...
May 2017: In Vivo
https://www.readbyqxmd.com/read/28423686/clinical-features-and-prognosis-for-intraventricular-liponeurocytoma
#19
Nini Xu, Jinxiu Cai, Jiang Du, Rong Yang, Huachen Zhu, Peiyi Gao, Jian Zhou, Xiaofeng Li
Cerebellar liponeurocytoma is a rare central nervous system tumor, we investigate its biological behaviors and clinical prognosis to improve the understanding of this tumor. We retrospectively analyzed the clinical, radiological and histopathological findings as well as follow-up data of two patients with intraventricular liponeurocytomas in Beijing Tiantan Hospital between July 2000 and July 2016. The main clinical manifestations of the two patients were headache. The supratentorial intraventricular liponeurocytoma appeared as isodense to slight hyperdense on CT scan and heterogeneous intensity on T1-weighted imaging (T1WI) and T2-weighted imaging (T2WI)...
March 8, 2017: Oncotarget
https://www.readbyqxmd.com/read/28423445/-comment-on-the-case-report-atypical-lipomatous-tumour-of-the-hand
#20
Marcus Lehnhardt
No abstract text is available yet for this article.
February 2017: Handchirurgie, Mikrochirurgie, Plastische Chirurgie
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