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J Nandhini, S Ramasamy, Ronak Nazir Kaul, Ravi David Austin
Angiofibroma is a locally advancing immensely vascular tumor that essentially arises from the nasopharynx. The clinical characteristics of extranasopharyngeal angiofibroma (ENA) do not accord to that of nasopharyngeal angiofibroma and can present a diagnostic confront. We describe a case of primary juvenile ENA in a 19-year-old patient who presented with a rapidly enlarging mass of the cheek region. The case is unusual because of its anatomic location. The diagnostic and management particulars are sketched.
January 2018: Journal of Oral and Maxillofacial Pathology: JOMFP
Kareem O Tawfik, Jeffrey J Harmon, Zoe Walters, Ravi Samy, Alessandro de Alarcon, Shawn M Stevens, Todd Abruzzo
OBJECTIVES: To describe a case of the rare complication of facial palsy following preoperative embolization of a juvenile nasopharyngeal angiofibroma (JNA). To illustrate the vascular supply to the facial nerve and as a result, highlight the etiology of the facial nerve palsy. METHODS: The angiography and magnetic resonance (MR) imaging of a case of facial palsy following preoperative embolization of a JNA is reviewed. RESULTS: A 13-year-old male developed left-sided facial palsy following preoperative embolization of a left-sided JNA...
February 1, 2018: Annals of Otology, Rhinology, and Laryngology
Sonia Nath, Jayant Prakash, Narendra Nath Singh, Virendra Kumar Prajapati
Tuberous sclerosis complex (TSC) is an autosomal dominant, multisystem genetic disorder. It is characterised by formation of benign hamartomas, neurofibromas, and angiofibromas located in different organs. We describe a case of a 13-year boy who complained of gingival enlargement. Clinical examination showed distinctive dermatological signs like hypopigmented macules, shagreen plaques, miliary fibromas, fibrous plaques and multiple angiofibromas. Oral manifestation included localised gingival enlargement. Gingivectomy was performed and the excised tissue was submitted for histopathological examination...
March 2018: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
Claiton Heitz, Alexandre Weber, Leandro Dini, Guilherme Pivatto Louzada, Eduardo Lombardo
Juvenile nasopharyngeal angiofibroma (JNA) is a rare benign tumor that occurs predominantly in young males. The authors report the case of a 22-year-old male patient who presented with a painless, exophytic tumor mass protruding through the right nostril, with anterior lateral extension associated with severe posterior involvement, erosion of the sphenoid bone to the right of the pterygoid process, and significant epistaxis. The preoperative evaluation, surgical approach, postoperative results, and a review of the literature are presented...
February 23, 2018: Journal of Craniofacial Surgery
Maulik M Dhandha, Elaine C Siegfried, Alan P Knutsen
The association between guttate psoriasis and infection with group A Streptococcus (GAS) has been well established in the medical literature. However, responses to treatments aimed at GAS eradication such as systemic antibiotics or tonsillectomy are inconsistent. Further complicating treatment recommendations for a disease with a suspected microbial trigger, the standard therapy for severe psoriasis is with systemic immunosuppressant medications. This case report illustrates the role of GAS as a trigger for acute onset severe psoriasis in a child whose skin disease initially worsened with a trial of methotrexate...
August 15, 2017: Dermatology Online Journal
G K Kundu, S Ahmed, S Akhter, M T Islam, T Dwa, A M Sabbir
Tuberous sclerosis complex (TSC) is a common neurocutaneous disorder characterized by hamartomatous changes in the lungs, brain, kidneys, skin, heart, and other organs. This retrospective study was done to see the clinical presentation and neuro imaging pattern of TSC in a tertiary care centre of Bangladesh and was conducted at Pediatric Neurology Unit of Bangabandhu Sheikh Mujib Medical University (BSMMU), Dhaka, Bangladesh from January 2013 to December 2013. Among total 10 patients male-female ratio was 3:2...
January 2018: Mymensingh Medical Journal: MMJ
M S Begum, U K Sarker, M A Islam, M A Sangma, P Paul, M A Rahman
Sinonasal mass is the abnormal growth of tissue from nasal cavity and mucosa of the paranasal sinuses. The growth may be benign or malignant. The benign lesion grows slowly and does not metastasize. The malignant lesion grows rapidly and metastasizes early. The aim of this study is to evaluate and diagnose the various types of sinonasal masses with MRI and its correlation with histopathological findings. This cross sectional descriptive study was carried out for a period of 02 years, from July 2015 to June 2017...
January 2018: Mymensingh Medical Journal: MMJ
Ritu Gupta, Satya Prakash Agarwal
Juvenile angiofibroma is a rare, highly vascular, histologically benign but locally invasive tumour, affecting predominantly male adolescents. To describe our experience in diagnosis of angiofibroma and treatment by combined endoscopic and transpalatal route. We analysed a case series of 50 patients of nasopharyngeal angiofibroma in Fisch stages I and II who presented at our institute during a period of four years 2011-2014, their clinical presentation, their treatment by combined transpalatal and endoscopic approach, intraoperative and post operative course, complications and tumour recurrence...
March 2018: Indian Journal of Otolaryngology and Head and Neck Surgery
Patrick O McGarey, Abel P David, Spencer C Payne
Nasopharyngeal angiofibroma (NA) is a benign, highly vascularised tumour of the nasopharynx, which typically occurs in young males aged 14-25 years. We report an interesting case of an NA arising de novo in a 32-year-old male. He was referred to our facility for severe nasal haemorrhage after biopsy of a left nasopharyngeal mass. In the operating room, extensive bleeding was noted, and an endoscopic medial maxillectomy was performed, and the left internal maxillary artery was ligated allowing for near total resection of the lesion...
February 8, 2018: BMJ Case Reports
Ahmad Safadi, Alberto Schreiber, Dan M Fliss, Piero Nicolai
Juvenile angiofibroma (JA) is a benign, highly vascular tumor which is diagnosed on the basis of clinical and imaging features. It has a characteristic pattern of spread commonly involving the pterygopalatine fossa and pterygoid base. The mainstay of treatment is surgery, while radiotherapy is rarely used for the treatment of recurrent lesion. Endoscopic endonasal surgery is currently the treatment of choice for small to intermediate size JAs, and is feasible even for advanced lesions; however, this should only be practiced in well-experienced centers...
February 2018: Journal of Neurological Surgery. Part B, Skull Base
Melissa A LoPresti, Jonathan N Sellin, Franco DeMonte
Objectives  To review developmental surgical anatomy and technical nuances related to pediatric skull base surgery. Design  Retrospective, single-center case series with literature review. Setting  MD Anderson Cancer Center. Participants  Patients undergoing pediatric skull base surgery. Main Outcome Measures  Review developmental anatomy of the pediatric skull base as it relates to technical nuance of various surgical approaches and insight gained from a 25-year institutional experience with this unique patient population...
February 2018: Journal of Neurological Surgery. Part B, Skull Base
Xiaole Song, Dehui Wang, Xicai Sun, Jingjing Wang, Zhuofu Liu, Quan Liu, Yurong Gu
BACKGROUND: To determine the learning curve with cumulative sum analysis for endoscopic resection of juvenile nasopharyngeal angiofibroma (JNA) and investigate whether the surgeon's expertise is a risk factor for recurrence. MATERIALS AND METHODS: We reviewed the medical records of patients with JNA who underwent endoscopic or endoscopic-assisted surgery between 2006 and 2015. We used cumulative sum (Cusum) analysis to plot the learning curve for operation time versus chronological sequence, and verified the Cusum curve by risk-adjusted Cusum (RA-Cusum) analysis...
January 24, 2018: Surgical Endoscopy
Ana Brandão, Sara Campos, João Fraga, Teresa Silva, Fernanda Águas
Cellular angiofibroma is a mesenchymal tumor, described in 1997, without gender preference, that usually appears at age 40. The vulvovaginal area is the most common site in women, mimicking vulvar benign tumors, like Bartholin gland cyst. However, there are a few described cases of a deep or extra-pelvic angiofibroma. Excision is the treatment of choice and the recurrence rate appears to be low. We present the case of a woman with a heterogeneous tumor in the right adnexial region. At the surgery, a retroperitoneal tumor was excised and the histopathological tissue analysis revealed a cellular angiofibroma...
December 29, 2017: Acta Médica Portuguesa
Aoife J McCarthy, Runjan Chetty
This is a review of the morphological spectrum of fatty tumours containing a component of spindle cells, highlighting the immunohistochemical and cytogenetic workup that is now mandatory for accurate diagnosis, with the goal of providing a practical approach for practising surgical pathologists. There have been significant advances in recent years in classifying and understanding the pathogenesis of fatty tumours with spindle cells, based on the correlation of histological, immunohistochemical and cytogenetic/molecular findings...
January 22, 2018: Journal of Clinical Pathology
M Zhao, Z R Yang, Y B Wang, Y Chen, G W Qi, Y J Yan, W J Xu, G Q Ru, X L He
Objective: To investigate the clinicopathologic characteristics, immunophenotypes, and differential diagnostic features of extra-pleural solitary fibrous tumor (SFT) with uncommon histology. Methods: Seven cases of extra-pleural SFT with uncommon histology were collected during January 2015 and December 2016 in Zhejiang Provincal People's Hospital; the clinical and radiologic features, histomorphology, immunophenotype and prognosis were analyzed. EnVision method was used for immunohistochemical staining of STAT6, CD34 and other differential diagnosis associated markers...
January 8, 2018: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Meryem Altin Ekin, Seyda Karadeniz Ugurlu, Fulya Cakalagaoglu
Giant cell angiofibroma (GCA) is a recently reported rare soft-tissue tumor that can develop in various sites including orbit. Orbital GCAs were mainly located in the eyelid or extraconal regions such as lacrimal gland and conjunctiva. We report an atypical case of a GCA arising in the intraconal area of the orbit in a 65-year-old male patient. The tumor was excised in total by lateral orbitotomy. Histological and immunohistochemical features were consistent with the diagnosis of GCA. No recurrence was observed during the follow-up of over 2 years...
January 2018: Indian Journal of Ophthalmology
Oyetewa Oyerinde, Danielle Buccine, Alison Treichel, Claire Hong, Chyi-Chia Richard Lee, Joel Moss, Thomas N Darling
BACKGROUND: Fibrous cephalic plaques (FCPs) stereotypically develop on the forehead of tuberous sclerosis complex (TSC) patients. They constitute a major feature for TSC diagnosis, and may present before other TSC-related cutaneous hamartomas. OBJECTIVE: To describe the clinical characteristics of FCPs in TSC. METHODS: 113 patients with TSC were enrolled in an observational cohort study. Retrospective analysis of medical records and skin photography was performed...
December 16, 2017: Journal of the American Academy of Dermatology
C Chiaverini
The year 2017 in pediatric dermatology was marked by several consensus recommendations and meta analyzes on childhood psoriasis, atopic dermatitis or PHACE syndrome, case series on the role of anti-JAK treatment in adolescent with alopecia areata, sirolimus for vascular malformations, ivermectine for rosacea, inhibitors of MEK for type 1 neurofibromatosis or on the side effects of the oral isotretinoin for acne or propranolol for immature hemangioma. Only few randomized controlled studies have been published on the interest of adalimumab in the treatment of psoriasis or topical sirolimus for angiofibroma in tuberous sclerosis complex for example...
December 2017: Annales de Dermatologie et de Vénéréologie
Manuel Valdebran, Brent Martin, Kristen M Kelly
Notable milestones in the treatment of vascular lesions have been achieved over the past century. Many cutaneous vascular lesions can be successfully treated with lightbased devices. In this review, we will discuss the treatment of port-wine birthmarks, lymphatic malformations, infantile hemangiomas, rosacea, venous lakes, pyogenic granulomas, cherry angiomas, and angiofibromas using lasers, total reflection amplification of spontaneous emission of radiation, intense pulsed light, and photodynamic therapy. In addition, for several of these diagnoses, we will review medical therapies that can be combined with light-based devices to provide enhanced results...
December 2017: Seminars in Cutaneous Medicine and Surgery
Michael Lause, Alisha Kamboj, Esteban Fernandez Faith
The skin serves as a window for clinicians to understand, diagnose, and monitor endocrine disease. Dermatologic manifestations of endocrinopathies contribute significantly to an individual's health and quality of life. In this review, we outline various disorders of the hypothalamic-pituitary axis, thyroid gland, pancreas, adrenal gland, and androgen axis as well as hereditary endocrine syndromes. In acromegaly, glycosaminoglycan deposition contributes to a thickening of skin and soft tissue, which manifests as coarsening and enlargement of facial and acral structures...
October 2017: Translational pediatrics
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