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Angiofibroma

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https://www.readbyqxmd.com/read/28199045/nasal-juvenile-angiofibroma-current-perspectives-with-emphasis-on-management
#1
Fernando López, Asterios Triantafyllou, Carl H Snyderman, Jennifer L Hunt, Carlos Suárez, Valerie J Lund, Primož Strojan, Nabil F Saba, Iain J Nixon, Kenneth O Devaney, Isam Alobid, Manuel Bernal-Sprekelsen, Ehab Y Hanna, Alessandra Rinaldo, Alfio Ferlito
Juvenile angiofibroma is an uncommon, benign, locally aggressive vascular tumor. It is found almost exclusively in young men. Common presenting symptoms include nasal obstruction and epistaxis. More advanced tumors may present with facial swelling and visual or neurological disturbances. The evaluation of patients with juvenile angiofibroma relies on diagnostic imaging. Preoperative biopsy is not recommended. The mainstay of treatment is resection combined with preoperative embolization. Endoscopic surgery is the approach of choice in early stages, whereas, in advanced stages, open or endoscopic approaches are feasible in expert hands...
February 15, 2017: Head & Neck
https://www.readbyqxmd.com/read/28193293/loss-of-chromosome-13-material-in-cellular-angiofibromas-indicates-pathogenetic-similarity-with-spindle-cell-lipomas
#2
Ioannis Panagopoulos, Ludmila Gorunova, Bodil Bjerkehagen, Kristin Andersen, Marius Lund-Iversen, Sverre Heim
BACKGROUND: Cellular angiofibroma is a rare benign mesenchymal neoplasm with morphological and immunohistochemical similarities to spindle cell lipoma. Karyotypic information on cellular angiofibroma is restricted to one case only which showed loss of material from chromosomes 13 and 16. A few other studies using fluorescence in situ hybridization showed deletions of the RB1 and FOXO1 loci, both of which are located in chromosome band 13q14. We present here cytogenetic data on two cellular angiofibromas with an abnormal karyotype...
February 13, 2017: Diagnostic Pathology
https://www.readbyqxmd.com/read/28181194/topical-rapamycin-for-facial-angiofibromas-in-a-child-with-tuberous-sclerosis-complex-tsc-a-case-report-and-long-term-follow-up
#3
Daniel Ebrahimi-Fakhari, Cornelia Sigrid Lissi Müller, Sascha Meyer, Marina Flotats-Bastardas, Thomas Vogt, Claudia Pföhler
Tuberous sclerosis complex (TSC) is a genetic multisystem disorder with prominent skin involvement including facial angiofibromas that often appear in early childhood. Here we report the case of a 12-year-old girl with widespread disfiguring facial angiofibromas that were successfully treated with topical rapamycin, a mTOR inhibitor. A sustained remission of skin lesions was documented in detail over a 3-year follow-up. This case highlights the fact that topical rapamycin is a useful option in treating TSC-associated skin lesions...
February 8, 2017: Dermatology and Therapy
https://www.readbyqxmd.com/read/28180045/juvenile-nasal-angiofibromas-a-comparison-of-modern-staging-systems-in-an-endoscopic-era
#4
Nicholas R Rowan, Nathan T Zwagerman, Molly E Heft-Neal, Paul A Gardner, Carl H Snyderman
Objectives To compare the clinical utility of four juvenile nasal angiofibroma (JNA) staging systems in a large cohort of patients. Design Retrospective case series. Setting Tertiary referral academic center. Participants Pediatric patients undergoing surgical resection of JNAs between January 2008 and June 2015. Main Outcome Measures Intraoperative blood loss and transfusions, number of staged operations, postoperative residual disease, and recurrent disease. Results In total, 34 patients were identified; all underwent preoperative embolization followed by surgery...
February 2017: Journal of Neurological Surgery. Part B, Skull Base
https://www.readbyqxmd.com/read/28175976/efficacy-of-preoperative-transcatheter-arterial-embolization-for-nasopharyngeal-angiofibroma-a-comparative-study
#5
Guosheng Tan, Zhenjiang Ma, Weiqing Long, Liangshuai Liu, Bing Zhang, Wei Chen, Jianyong Yang, Heping Li
OBJECTIVE: This study aimed to retrospectively evaluate the efficacy and safety of preoperative transcatheter arterial embolization (pTAE) for treating nasopharyngeal angiofibroma (NPAF). METHODS: Seventy-four NPAF patients were hospitalized for elective surgical treatment with pTAE (pTAE group, n = 32) or surgical treatment alone (non-pTAE group, n = 42) between January 1990 and December 2013. The following outcome measures were retrospectively analyzed and compared: intraoperative bleeding volume, surgery time (ST), duration of postoperative hospital stay (PHS), and disease recurrence...
February 7, 2017: Cardiovascular and Interventional Radiology
https://www.readbyqxmd.com/read/28153757/sinonasal-hemangioma-diagnosis-treatment-and-follow-up-of-37-patients-at-a-single-center
#6
Jong Seung Kim, Sam Hyun Kwon
PURPOSE: Hemangioma is a common benign vascular lesion of the head and neck region. It rarely involves the mucous membranes of the nasal cavity and paranasal sinuses. It should be added to the differential diagnosis of nasal cavity masses when the presenting symptoms are epistaxis or nasal obstruction. MATERIALS AND METHODS: A retrospective chart review was conducted of a histologic diagnosis of lobular capillary hemangioma or cavernous hemangioma of the sinonasal mucous membranes treated at the Chonbuk National University Hospital from January 1995 through December 2015...
January 5, 2017: Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28153134/distinctive-head-and-neck-bone-and-soft-tissue-neoplasms
#7
REVIEW
Bibianna Purgina, Chi K Lai
Benign and malignant primary bone and soft tissue lesions of the head and neck are rare. The uncommon nature of these tumors, combined with the complex anatomy of the head and neck, pose diagnostic challenges to pathologists. This article describes the pertinent clinical, radiographic, and pathologic features of selected bone and soft tissue tumors involving the head and neck region, including angiofibroma, glomangiopericytoma, rhabdomyosarcoma, biphenotypic sinonasal sarcoma, chordoma, chondrosarcoma, and osteosarcoma...
March 2017: Surgical Pathology Clinics
https://www.readbyqxmd.com/read/28123735/lipomatous-angiomyofibroblastoma-of-the-vulva-a-case-report-and-review-of-the-literature
#8
Susumu Matsukuma, Ayano Koga, Ryohei Suematsu, Hiroaki Takeo, Kimiya Sato
Lipomatous angiomyofibroblastoma is extremely rare, with only 16 cases reported to date. We herein describe an additional case arising in the left vulvar region of a 49-year-old woman. The resected 5.3-cm yellowish tumor predominantly consisted of fat cells (85% of the tumor), together with medium- and small-sized vessels, multifocal fibrotic areas and pseudoangiomatous spaces. Spindle, rounded, and/or epithelioid tumor cells proliferated in a nest and/or cord-like pattern, or singly within perivascular fibrous tissues and between fat cells...
January 2017: Molecular and Clinical Oncology
https://www.readbyqxmd.com/read/28073205/rare-presentation-of-a-testicular-angiofibroma-treated-with-testis-sparing-surgery
#9
Luca Leone, Paola Fulvi, Giulia Sbrollini, Alessandra Filosa, Enrico Caraceni, Angelo Marronaro, Andrea B Galosi
INTRODUCTION: Testicular benign tumors are very rare (< 5%). Testicular Angiofibroma (AF) is one of those, however the gold standard of treatment and follow-up is still unclear. CASE REPORT: A 47 years-old man with only one functioning testis was referred to our clinic for a palpable right testicular mass and atrophic contralateral testis. Patient underwent testis-sparing surgery with inguinal approach and intraoperative frozen sections examination with diagnosis of AF...
December 30, 2016: Archivio Italiano di Urologia, Andrologia
https://www.readbyqxmd.com/read/28073203/testicular-sparing-surgery-in-small-testis-masses-a-multinstitutional-experience
#10
Andrea B Galosi, Paola Fulvi, Andrea Fabiani, Lucilla Servi, Alessandra Filosa, Luca Leone, Angelo Marronaro, Enrico Caraceni, Rodolfo Montironi
INTRODUCTION: The incidence of benign testicular tumors is increasing in particular in small lesion incidentally found at scrotal ultrasonography. Primary aim of this study was to perform radical surgery in malignant tumor. Secondary aim was to verify the efficacy of the diagnostic-therapeutic pathway recently adopted in management of small masses with testis sparing surgery in benign lesions. MATERIALS AND METHODS: In this multicenter study, we reviewed all patients with single testis lesion less than 15 mm at ultrasound as main diameter...
December 30, 2016: Archivio Italiano di Urologia, Andrologia
https://www.readbyqxmd.com/read/28057044/tuberous-sclerosis-registry-to-increase-disease-awareness-tosca-baseline-data-on-2093-patients
#11
John C Kingswood, Guillaume B d'Augères, Elena Belousova, José C Ferreira, Tom Carter, Ramon Castellana, Vincent Cottin, Paolo Curatolo, Maria Dahlin, Petrus J de Vries, Martha Feucht, Carla Fladrowski, Gabriella Gislimberti, Christoph Hertzberg, Sergiusz Jozwiak, John A Lawson, Alfons Macaya, Rima Nabbout, Finbar O'Callaghan, Mirjana P Benedik, Jiong Qin, Ruben Marques, Valentin Sander, Matthias Sauter, Yukitoshi Takahashi, Renaud Touraine, Sotiris Youroukos, Bernard Zonnenberg, Anna C Jansen
BACKGROUND: Tuberous sclerosis complex (TSC) is a rare autosomal dominant genetic disorder. Many gaps remain in the understanding of TSC because of the complexity in clinical presentation. The TuberOus SClerosis registry to increase disease Awareness (TOSCA) is an international disease registry designed to address knowledge gaps in the natural history and management of TSC. Here, we present the baseline data of TOSCA cohort. METHODS: Patients of any age diagnosed with TSC, having a documented visit for TSC within the preceding 12 months, or newly diagnosed individuals were included...
January 5, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28049994/facial-angiofibromas-and-periungual-fibromas-in-tuberous-sclerosis
#12
Hiraku Tsujimoto, Hiroya Takeoka
No abstract text is available yet for this article.
2017: Internal Medicine
https://www.readbyqxmd.com/read/28002623/comprehensive-screening-for-med12-mutations-in-gynecological-mesenchymal-tumors-identified-morphologically-distinctive-mixed-epithelial-and-stromal-tumors
#13
Chang-Tsu Yuan, Wen-Chih Huang, Cheng-Han Lee, Ming-Chieh Lin, Chen-Hui Lee, Yu-Chien Kao, Hsuan-Ying Huang, Kuan-Ting Kuo, Jen-Chieh Lee
BACKGROUND: MED12 exon 2 mutations have been identified in most uterine leiomyomas and mammary fibroepithelial tumors. MED12 has not been genotyped in most other gynecological mesenchymal tumors. DESIGN: 68 uncommon gynecological mesenchymal tumors were genotyped for MED12 exon 2, including 27 müllerian adenosarcomas (including 3 tentatively diagnosed as "variant adenosarcomas"), 6 cellular angiofibromas, 6 aggressive angiomyxomas, 5 angiomyofibroblastomas, 5 superficial myofibroblastomas, 5 atypical polypoid adenomyomas, and 14 endometrial stromal sarcomas...
December 21, 2016: Histopathology
https://www.readbyqxmd.com/read/27994429/odontogenic-myxofibroma-of-gingiva-in-a-pediatric-patient-with-tuberous-sclerosis-a-rare-case-report
#14
Nidhi Bhoyar, Sunita Gupta, Sujoy Ghosh
Tuberous sclerosis complex (TSC) is a rare multisystem genetic disease, with an estimated incidence of 1 in 6000-1 in 10,000. TSC is an autosomal dominant syndrome involving heart, kidneys, lungs, and skin. The classic triad of TSC is seizures, mental retardation, and angiofibromas; this triad occurs in only 29% of patients. The clinical diagnostic guidelines on TSC are prepared based on clinical features, radiographic findings. The most common oral manifestations of TSC are fibroma, gingival hyperplasia, and enamel hypoplasia...
October 2016: Contemporary Clinical Dentistry
https://www.readbyqxmd.com/read/27957369/angiofibroma-originating-outside-the-nasopharynx-a-management-dilemma
#15
Ashraf Nabeel Mahmood, Rashid Sheikh, Hamad Al Saey, Sarah Ashkanani, Shanmugam Ganesan
Background. Angiofibroma is a benign tumor, consisting of fibrous tissue with varying degrees of vascularity, characterized by proliferation of stellate and spindle cells around the blood vessels. It most commonly arises from the nasopharynx, although it may rarely arise in extranasopharyngeal sites. Case Report. A 46-year-old male presented with left side nasal obstruction and epistaxis for one month. Clinical nasal examination revealed left sided polypoidal mass arising from the vestibular region of the lateral nasal wall...
2016: Case Reports in Otolaryngology
https://www.readbyqxmd.com/read/27957307/ten-years-of-experience-in-clinicopathologic-characteristics-treatment-and-outcome-of-patients-with-nasopharyngeal-pathologies-in-yazd-iran
#16
Fariba Binesh, Ahmad Shajari, Somayeh Abdollahi, Nasim Behniafard
INTRODUCTION: Although there are some studies regarding the clinical characteristics and outcome of nasopharyngeal tumors, one such study from Iran has not been reported to date. This study aimed to evaluate the clinical features, treatment, and consequences of nasopharyngeal lesions. METHODS: In this cross sectional study, we conducted a retrospective review of patients who were diagnosed with nasopharyngeal pathologies and treated at Shahid Sadoughi Hospital and Shahid Ramazanzadeh Radiotherapy Center in Yazd, Iran, over a period of 10 years (from 2005 to 2014)...
October 2016: Electronic Physician
https://www.readbyqxmd.com/read/27922925/update-on-nonmalignant-lesions-of-the-inferior-turbinate
#17
Jared M Goldfarb, David Goldfarb, Marc R Rosen
PURPOSE OF REVIEW: The inferior turbinates are routinely examined by otolaryngologists on anterior rhinoscopy and nasal endoscopy. Most lesions of the inferior turbinate are benign but can often be confused with malignancy. This review highlights the broad differential of nonmalignant lesions of the inferior turbinates and their management. RECENT FINDINGS: A variety of infectious, inflammatory, neoplastic, and vascular lesions may affect the inferior turbinates...
February 2017: Current Opinion in Otolaryngology & Head and Neck Surgery
https://www.readbyqxmd.com/read/27907099/analysis-of-a-mouse-skin-model-of-tuberous-sclerosis-complex
#18
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/27837336/perivaginal-benign-masses-diagnosis-and-therapy-in-a-series-of-66-women
#19
Anna-Lena Liaci, Hans Boesmueller, Markus Huebner, Sara Yvonne Brucker, Christl Reisenauer
PURPOSE: Benign perivaginal masses (PVM) are relatively rare. The aim of this study is, to create a higher awareness for these entities and to point out reliable diagnostics and an accurate treatment. METHODS: The medical records of the Department of Obstetrics and Gynecology Tuebingen were searched for number and type of urogynecological surgery in general, and a surgery, which took place particularly owing to benign PVM, over a period of 5 years. Diagnostics, treatment, histology and postoperative management were summarized and analyzed...
November 12, 2016: Archives of Gynecology and Obstetrics
https://www.readbyqxmd.com/read/27837201/efficacy-and-safety-of-topical-sirolimus-therapy-for-facial-angiofibromas-in-the-tuberous-sclerosis-complex-a-randomized-clinical-trial
#20
Mari Wataya-Kaneda, Ayumi Nakamura, Mari Tanaka, Misa Hayashi, Shoji Matsumoto, Koji Yamamoto, Ichiro Katayama
Importance: Inhibitors of mammalian target of rapamycin complex 1, such as sirolimus, effectively target skin lesions in tuberous sclerosis complex (TSC). However, systemic treatment causes adverse effects, and topical sirolimus has shown promise in the treatment of facial angiofibromas. Objective: To evaluate the efficacy, safety, and optimal concentration of a topical sirolimus gel vs placebo for treatment of facial angiofibromas in TSC. Design, Setting, and Participants: A double-blind, placebo-controlled, parallel-group, dose-escalation, phase 2 randomized clinical trial using 3 sirolimus gel concentrations was performed at Osaka University Hospital, Osaka, Japan...
January 1, 2017: JAMA Dermatology
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