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