Jnas originate from the posterior choanal tissues and rapidly extend into the surrounding regions, including the nasopharynx, the orbits, and even the intracranial cavity. Expression on endothelial cells in juvenile nasopharyngeal angiofibroma. Juvenile nasopharyngeal angiofibroma is a rare benign tumour of vascular origin found in adolescent males, originating around the sphenopalatine foramen. Angiofibroma nasofaring belia pdf juvenile nasopharyngeal angiofibroma is a pathologically benign yet locally aggressive and destructive. As the tumor is largely spaceoccupying and highly vascular, patients typically. Robotic surgery of the skull base for jna is something to expect for the future. Study the changing trend basically in the northeastern region of southeast asia and its. The juvenile nasopharyngeal angiofibroma has a characteristic growth in all directions from its origin. In some situations, depending on where the tumor has spread, complete removal may not be possible. The tumor contains many blood vessels, spreads within the area in which it.
Nasoangiofibroma youth is a highly vascularized tumor almost exclusively male adolescents presentation. Juvenile nasopharyngeal angiofibroma jna is a rare locally invasive neoplasm composed of cavernous vascular channels set in an abundant myxoid stroma of fibroblasts and myofibroblasts. Jnas occur almost exclusively in the nasopharynx of adolescent. What are the treatment options for juvenile nasopharyngeal angiofibroma. First line therapy is usually surgery, with the goal of removing as much of the tumor as possible. However, the extensions of the tumor seem to be independent, each one with distinct behavior. The typical patient is an adolescent male with a clinical history of recurrent epistaxis and nasal obstruction. Song, xiaole yang, chenhe zhang, huankang wang, jingjing sun, xicai hu, li liu, zhuofu and wang, dehui 2018. Two types of lateral extension in juvenile nasopharyngeal angiofibroma. Starting with a brief introduction to the history, etiology and relevant anatomy, the book goes on to discuss the pathology, clinical features and treatment of this disease in depth, concluding with a summary of current controversies. Variable expression of molecular markers in juvenile. Pdf juvenile nasopharyngeal angiofibroma researchgate. It has been shown to contain cytosolic androgen receptors and to regress with estrogen therapy. Endoscopic approach to juvenile nasopharyngeal angiofibroma.
Jul 16, 2019 the diseasefree period ranged from 10 to 36 months postoperatively. In 1906 chareau revived the interest in the study of juvenile nasopharyngeal angiofibroma. Recently a changing trend has been seen in its epidemiology, pathogenesis, diagnosis, medical management, preoperative care, surgical management and postoperative care including radiotherapy. Sep 01, 2008 juvenile nasopharyngeal angiofibroma is a rare, benign tumor that occurs most often in adolescent males. Juvenile nasopharyngeal angiofibroma jna is a rare benign tumor arising predominantly in the nasopharynx of adolescent males. Exclusively endoscopic removal of juvenile nasopharyngeal. Juvenile angiofibroma is a rare benign lesion originating from the pterygopalatine fossa with distinctive epidemiologic features and growth patterns. Radiologists prior knowledge on the tumors characteristics aids in establishing a diagnosis. Ppt journal juvenile nasopharynx angiofibroma free download as powerpoint presentation. Juvenile nasopharyngeal angiofibroma knowledge for.
Juvenile angiofibroma is a rare benign lesion originating from the. Ppt juvenile nasopharyngeal angiofibroma powerpoint. Juvenile nasopharyngeal angiofibroma jna onset most commonly is in the second decade. Juvenile angiofibroma ja is a rare benign vascular lesion of the skull. Finally, with the advent of imrt and an imageguided robotic radiotherapy delivery. Surgical management of juvenile nasopharyngeal angiofibroma. Design retrospective study of 20 patients, with a mean followup of 22 months. Oct 04, 2019 epidemiology juvenile nasopharyngeal angiofibromas. Review article nasopharyngeal angiofibroma ashutosh hota s. Treatments for juvenile nasopharyngeal angiofibroma boston. Staging of juvenile nasopharyngeal angiofibromas is performed with crosssectional imaging and relies on the identification of local tumor extent, and invasion of adjacent spaces. Available formats pdf please select a format to send. Juvenile nasopharyngeal angiofibroma jna is a rare tumor that exhibits a predictable spreading pattern.
The contents of this presentation do not have any intended commercial use. Jul 15, 2015 juvenile nasopharyngeal angiofibroma jna is a benign neoplasm of the nasopharynx. Juvenile nasopharyngeal angiofibroma knowledge for medical. Adolescents and young adults between 14 and 25 years are affected, and there is a distinct male predominance. Ppt journal juvenile nasopharynx angiofibroma radiation. The diseasefree period ranged from 10 to 36 months postoperatively. Treatments for juvenile nasopharyngeal angiofibroma. Juvenile nasopharyngeal angiofibromas jna are a rare benign but locally aggressive vascular tumor. It can grow slowly, spreading into the area around the brain and into the orbits of the eye. Home acta otorrinolaringologica espanola comprar tratamiento quirurgico del angiofibroma nasofaringeo juvenil en pacientes pediatricos. The tumor contains many blood vessels, spreads within the area in which it started locally invasive, and can cause bone damage. Common practice is to excise the tumor with open or endoscopic surgery.
Juvenile angiofibroma is a noncancerous growth that causes bleeding in the nose and sinuses. Mar 07, 2020 it most commonly affects adolescent males and may grow into fissures of the. The juvenile nasopharyngeal angiofibroma is a benign neoplasm of the posterior nasopharynx it occurs almost exclusively in adolescent males, with an average age at diagnosis of 15 years old. Although the exact pathogenesis of the tumour is not yet known, natural history and growth patterns can be predicted.
Juvenile nasopharyngeal angiofibroma with orbital extension angiofibroma nasofaringeo juvenil con extension orbitaria. Plain radiographs no longer play a role in the workup of a suspected juvenile nasopharyngeal angiofibroma, however they may still be obtained in some instances during the assessment of nasal obstruction, or symptoms of sinus obstructions. Juvenile nasopharyngeal angiofibroma jna is a rare and benign but locally aggressive fibrovascular tumor arising from the posterolateral wall of the. These tumors are highly aggressive and are associated with significant morbidity and mortality due to its tendency to bleed. Juvenile angiofibroma rads see also with virtual slide. Variations in molecular markers in juvenile nasopharyngeal angiofibroma in an indian population were investigated and compared with global reports. Juvenile nasopharyngeal angiofibromas staging radiology.
Juvenile nasopharyngeal angiofibroma electronic journal of. Fisch classification, however, is currently accepted. It also may be referred to as juvenile nasopharyngeal angiofibroma jna. The juvenile nasopharyngeal angiofibroma is a benign neoplasm of the posterior. Jun 28, 2019 angiofibroma nasofaring belia pdf juvenile nasopharyngeal angiofibroma is a pathologically benign yet locally aggressive and destructive vascular lesion of head and neck. Nasopharyngeal angiofibroma is usually encapsulated and composed of vascular tissue and fibrous stroma with coarse or fine collagen fibers. It is a rare, benign, vascular neoplasm that accounts for less than 0. Nasal cavity, paranasal sinuses, nasopharynx nasopharyngeal angiofibroma.
Juvenile angiofibroma occurs most commonly among adolescent boys. Infact hippocrates goes on to describe a polyp in the nose which weeped blood. The pictures used in this presentation have been obtained from a number of sources. Juvenile nasopharyngeal angiofibromas are benign yet aggressive anterior skull base tumors that occur almost exclusively in male adolescents. Juvenile nasopharyngeal angiofibroma jna is a rare and benign but locally in the nasopharynx and nasal cavity, leading to manifestations such as nasal. Given its vascularity, prominent flow voids are seen on mri leading to a salt and pepper appearance. It is the most common benign tumor of the nasopharnyx but only accounts for less than 0. Visual acuity was 2020 in both eyes and intraocular pressure iop 24 mmhg in left eye le without limitations in ocular movement. Juvenile nasopharyngeal angiofibroma evaluation and workup return to. Juvenile angiofibromas childrens health issues merck. Juvenile nasopharyngeal angiofibroma has been documented since the time of hippocrates 4 bc.
Sep 16, 2015 juvenile nasopharyngeal angiofibroma is a very rare, highly vascular, uncapsulated and locally invasive tumor. Juvenile nasopharyngeal angiofibroma is a rare, highly luiz a cr, romualdo s lt, fava a s. Common presenting symptoms include nasal obstruction and epistaxis. Shaheen in 1930 reported the first female patient with juvenile nasopharyngeal. Juvenile nasopharyngeal angiofibromas jna are a rare benign, but locally aggressive, vascular tumour. Juvenile nasopharyngeal angiofibroma is a very rare, highly vascular, uncapsulated and locally invasive tumor. Juvenile nasopharyngeal angiofibroma with orbital extension.
The age range for this disease is 7 to 19 years old, with most people being diagnosed between 10 and 19 years old. Vessels are thinwalled, lack elastic fibers, have absent or incomplete smooth muscle, and vary in appearance from stellate or staghorn to barely conspicuous because of stromal compression. Juvenile nasopharyngeal angiofibroma is a rare, benign tumor that occurs most often in adolescent males. Juvenile angiofibroma is an uncommon, benign, locally aggressive vascular tumor. Juvenile nasopharyngeal angiofibroma jna is a rare tumor that predominantly occurs in adolescent males, the average age of occurence being 15. Juvenile nasopharyngeal angiofibroma radiology reference. Juvenile nasopharyngeal angiofibroma a free powerpoint ppt presentation displayed as a flash slide show on id. Juvenile nasopharyngeal angiofibroma jna is a benign neoplasm of the nasopharynx. Angiofibroma nasofaring juvenile free download as pdf file. Juvenile nasopharyngeal angiofibroma iowa head and neck. Introduction uncommon, benign and extremely vascular tumour up to 0. The juvenile nasopharyngeal angiofibroma is a benign neoplasm of. Juvenile nasopharyngeal angiofibroma carver college of medicine.
May 16, 2018 nasopharyngeal angiofibroma is usually encapsulated and composed of vascular tissue and fibrous stroma with coarse or fine collagen fibers. None of these patients required postoperative nasal packing, and all were discharged the day after surgery. Angiofibroma is a benign noncancerous nasal cavity tumor that almost exclusively affects adolescent boys. Although the use of nonsurgical therapies is described in the literature, surgery is currently considered the ideal treatment for. We aimed to report the characteristic magnetic resonance imaging mri findings and the spread patterns of jna. Angiofibroma juvenile nasopharyngeal angiofibroma upmc. Juvenile nasopharyngeal angiofibroma is a tumor with a predilection for adolescent boys. Extensive investigation has been unable to settle this issue in. It most commonly affects adolescent males because it is a hormonesensitive tumor. Jnas represent less than 1 % of head and neck tumors. Their use is purely for academic and teaching purposes. Review article nasopharyngeal angiofibroma ashutosh hota. Nasopharyngeal angiofibroma is a histologically benign but locally aggressive vascular tumor of the nasopharynx that arises from the superior margin of the sphenopalatine foramen and grows in the back of the nasal cavity. A male, 21 years of age, without relevant history, presented with left exophthalmos and diplopia in extreme gaze positions with 2 weeks evolution, without clinic of epistaxis or nasal obstruction.
It is an aggressive neoplasm and shows a propensity for destructive local spread often extending to the base of the skull and into the cranium. Variable expression of molecular markers in juvenile nasopharyngeal angiofibroma volume 1 issue 9 a mishra, a pandey, s c mishra. Microsurgery in juvenile nasopharyngeal angiofibroma. Juvenile angiofibroma jna is a benign tumor that tends to bleed and occurs in the nasopharynx of prepubertal and adolescent males. Juvenile nasopharyngeal angiofibroma jna is a rare, benign, but locally aggressive tumor occurring almost exclusively in adolescent males. We report the case of a 17yearold male who presented in 1995 with a mass filling the left posterior nasal cavity.
It most commonly affects adolescent males and may grow into fissures of the. Juvenile nasopharyngeal angiofibroma jna springerlink. Genetic evidence that juvenile nasopharyngeal angiofibroma. Epidemiology juvenile nasopharyngeal angiofibromas. Typically, the tumor causes a stuffy nose or headache, often with nosebleeds, which are sometimes very severe.
These red erythematous elevated skin lesions papules are tumors madeup of fibrous tissue angiofibroma and occur with tuberous sclerosis. Juvenile angiofibroma information mount sinai new york. Juvenile nasopharyngeal angiofibromas are a rare benign, but locally aggressive, vascular tumors that occur almost exclusively in young men. For a discussion of this entity please refer to the parent article. Nasopharyngeal angiofibroma introduction juvenile nasopharyngeal angiofibroma jna is a benign, but locally aggressive and extremely vascular head and neck neoplasm, occurring almost exclusively in the nasopharynx of adolescent males. Get a printable copy pdf file of the complete article 2. Jun 29, 2019 juvenile nasopharyngeal angiofibroma jna is a rare benign tumor arising predominantly in the nasopharynx of adolescent males. Objective to determine the feasible conditions for exclusive endoscopic resection of juvenile nasopharyngeal angiofibroma. On imaging, they present as a vividly enhancing softtissue masses centered on the sphenopalatine foramen. Surgical resection of juvenile angiofibroma vula university of.
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