Juvenile angiofibroma is a rare, benign, rich vascular tumor, and approximately one new case is diagnosed in denmark each year. The use of intensity modulated radiotherapy for the treatment. This is a concise presentation for medical students especially from india. However, the extensions of the tumor seem to be independent, each one with distinct behavior. Juvenile nasopharyngeal angiofibroma is a pathologically benign yet locally aggressive and destructive vascular lesion of head and neck region typically affecting adolescent boys. Juvenile nasopharyngeal angiofibroma is the most common. Females with juvenile nasopharyngeal angiofibroma jna should undergo genetic testing. Although considered the standard of care by most, an endoscopic approach may not be sufficient for extensive tumours, as exemplified by the current case of a young man. Jna can spread into a variety of compartments in the head including the sinuses, the orbit where the eye is located, and the brain which can make. Juvenile nasopharyngeal angiofibroma jna is rare in patients older than 25 years. Juvenile nasopharyngeal angiofibroma jna is a locally aggressive benign vascular tumour exclusively amongst adolescence males. Juvenile nasopharyngeal angiofibroma stages i and ii.
Flutamide as a preoperative treatment in juvenile angiofibroma ja with intracranial invasion. Juvenile nasopharyngeal angiofibroma jna is a benign vascular tumor that appears in the nasal cavity. The choice of a surgical approach is designed to provide access to routes of tumor extension and control of the vascular supply of the tumor. Graph of dose volume histogram demonstrating doses delivered to the normal structures and juvenile angiofibroma target. Hippocrates described the tumor in the 5th century bc, but friedberg first used the term angiofibroma in 1940. As the name implies, jna is a disease of the young and occurs almost exclusively in adolescent males. Jul 15, 2015 juvenile nasopharyngeal angiofibroma jna is a benign neoplasm of the nasopharynx. Extensive investigation has been unable to settle this issue in. Juvenile nasopharyngeal angiofibroma jna is a rare, benign, highly vascular, and locally aggressive tumor that predominantly occurs in adolescent males. Nasopharyngeal angiofibroma an overview sciencedirect topics. To accomplish this goal we first describe our institutions experience with radiotherapy for jna and then use this data set as a framework for explaining the role of radiotherapy in the treatment of this uncommon tumor. Ppt juvenile nasopharyngeal angiofibroma powerpoint. In usa, this lesion represents the most frequent head and neck tumor of adolescence with one new case per 5000 to 50,000 patients referred to an otolaryngologist.
Juvenile nasopharyngeal angiofibroma jna is a rare, benign, but locally aggressive tumor occurring almost exclusively in adolescent males. Primary radiation therapy for juvenile nasopharyngeal angiofibroma. A new endoscopic staging system for angiofibromas oncology. Mean age at diagnosis is 14 years may regress in late teens but may persist into adulthood rare in patients older than 25 years. Juvenile nasopharyngeal angiofibroma knowledge for. Juvenile nasopharyngeal angiofibroma jna is a locally invasive benign tumor which almost exclusively.
Juvenile nasopharyngeal angiofibroma is a tumor with a predilection for adolescent boys. Juvenile nasopharyngeal angiofibroma for medical students. The juvenile nasopharyngeal angiofibroma has a characteristic growth in all directions from its origin. Study design retrospective study of 14 cases of jna resection at the department of otorhinolaryngology, university of innsbruck innsbruck, austria. Introduction uncommon, benign and extremely vascular tumour up to 0.
The goals of treatment of juvenile nasopharyngeal angiofibroma are complete surgical excision with minimal morbidity and avoidance of radiation therapy. Juvenile nasopharyngeal angiofibroma jna is a benign neoplasm of the nasopharynx. Historical treatment of jna has included embolization, surgical resection, and radiation. In this video, we are going to see about juvenile nasopharyngeal angiofibroma. Juvenile nasopharyngeal angiofibroma linkedin slideshare. Nasopharyngeal angiofibroma an overview sciencedirect. Ppt journal juvenile nasopharynx angiofibroma free download as powerpoint presentation. Objective to determine the feasible conditions for exclusive endoscopic resection of juvenile nasopharyngeal angiofibroma design retrospective study of 20 patients, with a mean followup of 22 months setting six academic referral hospitals interventions all patients had a preoperative computed tomographic or magnetic resonance imaging scan and at least 1 follow. They are exclusively found in males and if diagnosed in a female patient, genetic testing. Given its vascularity, prominent flow voids are seen on mri leading to a salt and pepper appearance. The typical patient is an adolescent male with a clinical history of recurrent epistaxis and nasal obstruction.
Radiotherapy for juvenile nasopharyngeal angiofibroma. Juvenile nasopharyngeal angiofibroma knowledge for medical. Craniofacial resection of advanced juvenile nasopharyngeal. 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. Juvenile nasopharyngeal angiofibromas jna are a rare benign, but locally aggressive, vascular tumor. Jan 11, 2011 juvenile nasopharyngeal angiofibroma is a pathologically benign yet locally aggressive and destructive vascular lesion of head and neck region typically affecting adolescent boys. Juvenile angiofibroma is a rare, benign, rich vascular tumor, and approximately one new case is diagnosed in denmark each. Two patients received radiotherapy as primary therapy, and the.
However, this case is not typical as it doesnt invade the nasal fossae. Juvenile nasopharyngeal angiofibromas occur almost exclusively in males and usually in adolescence 15 years. Juvenile nasopharyngeal angiofibroma jna onset most commonly is in the second decade. Juvenile nasopharyngeal angiofibroma radiology case. Reddy ka, mendenhall wm, amdur rj, stringer sp, cassisi nj. Mar 07, 2020 it most commonly affects adolescent males and may grow into fissures of the. Juvenile angiofibroma is a noncancerous growth that causes bleeding in the nose and sinuses. Nov 17, 2011 juvenile angiofibroma ja is a benign vascular neoplasm which affects young males between 9 and 19 years of age and accounts for 0. Although the use of nonsurgical therapies is described in the literature, surgery is currently considered the ideal. Juvenile angiofibroma free download as powerpoint presentation. Although it is histologically benign, it may be locally invasive and show considerable growth, eroding through bone and vital structures 3, 5, 17. The management of juvenile nasopharyngeal angiofibroma has undergone a significant evolution, with more surgeons moving towards the minimal invasive endoscopic approaches. A, axial t2weighted repetition time tr, 2500 milliseconds. Juvenile nasopharyngeal angiofibroma jna is a benign tumor that tends to bleed and occurs in the nasopharynx of prepubertal and adolescent males.
Management of juvenile nasopharyngeal angiofibroma. Common presenting symptoms include nasal obstruction, epistaxis and a nasal mass. Juvenile nasopharyngeal angiofibroma jna is a rare, benign neoplasm that accounts for less than 0. There is extension of the patients juvenile angiofibroma into the posterior ethmoid air cells and sphenoid sinus arrows. Design retrospective study of 20 patients, with a mean followup of 22 months. 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. Juvenile nasopharyngeal angiofibroma evaluation and workup return to. Juvenile nasopharyngeal angiofibromas are a rare benign, but locally aggressive, vascular tumors that occur almost exclusively in young men on imaging, they present as a vividly enhancing softtissue masses centered on the sphenopalatine foramen. Journal of clinical imaging science imaging in the. Nasoangiofibroma youth is a highly vascularized tumor almost exclusively male adolescents presentation. May 16, 2018 patterns of vascularization and surgical morbidity in juvenile nasopharyngeal angiofibroma. To explain the concepts that radiation oncologists need to understand to manage patients with juvenile nasopharyngeal angiofibroma jna.
Juvenile angiofibroma rads see also with virtual slide. Their use is purely for academic and teaching purposes. It most commonly affects adolescent males and may grow into fissures of the. Grossly, juvenile nasopharyngeal angiofibroma is a lobulated, red and blue tumor with a firm to spongy, focally cystic, and hemorrhagic cut surface. Juvenile angiofibroma ja is a benign vascular neoplasm which affects young males between 9 and 19 years of age and accounts for 0. Angiofibroma nasofaring juvenile free download as pdf file. Mar 22, 20 juvenile nasopharyngeal angiofibroma jna is a rare, benign neoplasm that accounts for less than 0. Jnas originate from the posterior choanal tissues and rapidly extend into the surrounding regions, including the nasopharynx, the orbits, and even the intracranial cavity. General characteristics, management and outcomes of 11 cases of jna presented to our centre between 2000 till. These features are compatible with juvenile angiofibroma, a benign but locally invasive tumor. Juvenile nasopharyngeal angiofibroma 420 introduction juvenile nasopharyngeal angiofibroma jna represents 0. Usually, the presenting symptom is a painless nasal obstruction or epistaxis.
A concise classification system and appropriate treatment options. Angiofibroma nasofaring belia pdf juvenile nasopharyngeal angiofibroma is a pathologically benign yet locally aggressive and destructive vascular lesion of head and neck. Introduction juvenile angiofibroma represents less than 0. Longterm results of radiation therapy for juvenile nasopharyngeal angiofibroma.
Jna occurs commonly in the nasopharynx of adolescent males. Juvenile nasopharyngeal angiofibroma iowa head and neck. Several reports suggest that jna regresses over time. Juvenile angiofibroma human head and neck clinical. Imaging in the diagnosis of juvenile nasopharyngeal. This is a histologically benign lesion with very aggressive local behavior.
Juvenile nasopharyngeal angiofibroma jna is a pathologically benign yet locally aggressive and destructive tumor that develops in the choana and nasopharynx. Juvenile nasopharyngeal angiofibroma boston childrens. It most commonly affects adolescent males because it is a hormonesensitive tumor. Patterns of vascularization and surgical morbidity in juvenile nasopharyngeal angiofibroma. May 16, 2018 juvenile nasopharyngeal angiofibroma jna occurs exclusively in males. It has been shown to contain cytosolic androgen receptors and to regress with estrogen therapy. Sirolimus for the treatment of juvenile nasopharyngeal. Juvenile nasopharyngeal angiofibroma radiology reference. Download fulltext pdf juvenile nasopharyngeal angiofibroma article pdf available in indian journal of dental research 161. It is almost exclusicely encountered in adolescent males 1. Endoscopic surgery for juvenile nasopharyngeal angiofibroma where are. To provide an analysis of nine patients with juvenile angiofibroma treated with radiotherapy between june 1975 and june 1987 methods and materials. Juvenile angiofibroma human head and neck clinical medicine. Ppt journal juvenile nasopharynx angiofibroma radiation.
Objective to determine the feasible conditions for exclusive endoscopic resection of juvenile nasopharyngeal angiofibroma. Juvenile angiofibroma is a rare benign lesion originating from the pterygopalatine fossa with distinctive epidemiologic features and growth patterns. Though it is a benign tumor, it is locally invasive and can invade the. The tumor contains many blood vessels and spreads within the area in which it started locally invasive. Juvenile nasopharyngeal angiofibroma is a rare benign tumour of vascular origin found in adolescent males, originating around the sphenopalatine foramen. A, axial unenhanced t1weighted repetition time tr, 600 milliseconds. A 10yearold boy with juvenile nasopharyngeal angiofibroma. Exclusively endoscopic removal of juvenile nasopharyngeal.
It was friedberg who first used the term angiofibroma in 1940. Juvenile nasopharyngeal angiofibroma boston childrens hospital. Imaging in the diagnosis of juvenile nasopharyngeal angiofibroma. Download fulltext pdf download fulltext pdf juvenile angiofibroma. Adolescents and young adults between 14 and 25 years are affected, and there is a distinct male predominance.
The most common presenting symptom is painless nasal obstruction or epistaxis. 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. Although it is nonmalignant not cancerous, it can expand quickly and extensively. Although the use of nonsurgical therapies is described in the literature, surgery is currently considered the ideal treatment for. Juvenile angiofibroma is an uncommon, benign, locally aggressive vascular tumor.
It was first described by hippocrates in the 5 th century bc. Cheek swelling, proptosis, visual changes, aural fullness, decreased hearing, or cranial nerve findings suggest a more advanced lesion. Juvenile nasopharyngeal angiofibroma jna is a rare and benign but locally aggressive fibrovascular tumor arising from the posterolateral wall of the. Section from treatment planning program demonstrating doses delivered to normal structures and juvenile angiofibroma. Juvenile nasopharyngeal angiofibroma with orbital extension. A yearold boy with juvenile nasopharyngeal angiofibroma with skull base extension and invasion of the left cavernous sinus.
This is a classical presentation of juvenile nasopharyngeal angiofibroma. The present article is a retrospective study of surgically treated patients of juvenile nasopharyngeal angiofibroma over a period of 5 years. Surgical management and outcome of juvenile nasopharyngeal. Juvenile angiofibroma is an uncommon benign neoplasm that presents in adolescent males at a median age of 14 years. Juvenile nasopharyngeal angiofibroma is an uncommon benign tumour of the nasopharynx which arises in the region of the sphenopalatine foramen and it grows insidiously invading surrounding structures.
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