Juvenile nasopharyngeal angiofibroma jna is a pathologically benign yet locally aggressive and destructive tumor that develops in the choana and nasopharynx. Juvenile angiofibroma is an uncommon benign neoplasm that presents in adolescent males at a median age of 14 years. Imaging in the diagnosis of juvenile nasopharyngeal angiofibroma. The goals of treatment of juvenile nasopharyngeal angiofibroma are complete surgical excision with minimal morbidity and avoidance of radiation therapy. Sirolimus for the treatment of juvenile nasopharyngeal. 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.
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. Mean age at diagnosis is 14 years may regress in late teens but may persist into adulthood rare in patients older than 25 years. Hippocrates described the tumor in the 5th century bc, but friedberg first used the term angiofibroma in 1940. Juvenile angiofibroma ja is a benign vascular neoplasm which affects young males between 9 and 19 years of age and accounts for 0.
Mar 22, 20 juvenile nasopharyngeal angiofibroma jna is a rare, benign neoplasm that accounts for less than 0. Section from treatment planning program demonstrating doses delivered to normal structures and juvenile angiofibroma. The management of juvenile nasopharyngeal angiofibroma has undergone a significant evolution, with more surgeons moving towards the minimal invasive endoscopic approaches. The tumor contains many blood vessels and spreads within the area in which it started locally invasive. It was friedberg who first used the term angiofibroma in 1940. Juvenile nasopharyngeal angiofibromas jna is an uncommon histologically benign but locally aggressive vascular tumour, accounting for 0. Juvenile nasopharyngeal angiofibromas occur almost exclusively in males and usually in adolescence 15 years. Ppt journal juvenile nasopharynx angiofibroma free download as powerpoint presentation. Although the exact pathogenesis of the tumour is not yet known, natural history and growth patterns can be predicted. Juvenile nasopharyngeal angiofibroma radiology case. 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 is a tumor with a predilection for adolescent boys. Cheek swelling, proptosis, visual changes, aural fullness, decreased hearing, or cranial nerve findings suggest a more advanced lesion. It was first described by hippocrates in the 5 th century bc. Juvenile nasopharyngeal angiofibroma jna is a rare, benign, but locally aggressive tumor occurring almost exclusively in adolescent males. Though it is a benign tumor, it is locally invasive and can invade the. Two patients received radiotherapy as primary therapy, and the.
Design retrospective study of 20 patients, with a mean followup of 22 months. It is almost exclusicely encountered in adolescent males 1. Although it is nonmalignant not cancerous, it can expand quickly and extensively. Several reports suggest that jna regresses over time. May 16, 2018 juvenile nasopharyngeal angiofibroma jna occurs exclusively in males. The juvenile nasopharyngeal angiofibroma is a benign neoplasm of the posterior. Although it is histologically benign, it may be locally invasive and show considerable growth, eroding through bone and vital structures 3, 5, 17. Juvenile nasopharyngeal angiofibroma radiology reference. 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. There is extension of the patients juvenile angiofibroma into the posterior ethmoid air cells and sphenoid sinus arrows. Ppt juvenile nasopharyngeal angiofibroma powerpoint. A 10yearold boy with juvenile nasopharyngeal angiofibroma.
Radiotherapy for juvenile nasopharyngeal angiofibroma. 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. Usually, the presenting symptom is a painless nasal obstruction or epistaxis. Although the use of nonsurgical therapies is described in the literature, surgery is currently considered the ideal treatment for. The most common presenting symptom is painless nasal obstruction or epistaxis. 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. Common presenting symptoms include nasal obstruction, epistaxis and a nasal mass. Juvenile nasopharyngeal angiofibroma is a pathologically benign yet locally aggressive and destructive vascular lesion of head and neck region typically affecting adolescent boys. In this video, we are going to see about juvenile nasopharyngeal angiofibroma. Juvenile nasopharyngeal angiofibroma 420 introduction juvenile nasopharyngeal angiofibroma jna represents 0.
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. These features are compatible with juvenile angiofibroma, a benign but locally invasive tumor. Adolescents and young adults between 14 and 25 years are affected, and there is a distinct male predominance. Objective to determine the feasible conditions for exclusive endoscopic resection of juvenile nasopharyngeal angiofibroma. Juvenile nasopharyngeal angiofibroma linkedin slideshare. 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. 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. Disease definition juvenile nasopharyngeal angiofibroma jna is a rare and benign but locally aggressive fibrovascular tumor nwsal from the posterolateral wall of the nasopharynx, which affects mainly young and adolescent males onset usually occurring between years of age and that presents as a mass in the nasopharynx and nasal cavity.
Introduction juvenile angiofibroma represents less than 0. Nasoangiofibroma youth is a highly vascularized tumor almost exclusively male adolescents presentation. Juvenile angiofibroma is an uncommon, benign, locally aggressive vascular tumor. Flutamide as a preoperative treatment in juvenile angiofibroma ja with intracranial invasion. Nasopharyngeal angiofibroma an overview sciencedirect topics. Juvenile nasopharyngeal angiofibroma jna is a rare, benign, highly vascular, and locally aggressive tumor that predominantly occurs in adolescent males. Grossly, juvenile nasopharyngeal angiofibroma is a lobulated, red and blue tumor with a firm to spongy, focally cystic, and hemorrhagic cut surface. Exclusively endoscopic removal of juvenile nasopharyngeal. Study design retrospective study of 14 cases of jna resection at the department of otorhinolaryngology, university of innsbruck innsbruck, austria. However, this case is not typical as it doesnt invade the nasal fossae.
Jna occurs commonly in the nasopharynx of adolescent males. Download fulltext pdf download fulltext pdf juvenile angiofibroma. General characteristics, management and outcomes of 11 cases of jna presented to our centre between 2000 till. 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. 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. Longterm results of radiation therapy for juvenile nasopharyngeal angiofibroma. The present article is a retrospective study of surgically treated patients of juvenile nasopharyngeal angiofibroma over a period of 5 years. A yearold boy with juvenile nasopharyngeal angiofibroma with skull base extension and invasion of the left cavernous sinus.
It most commonly affects adolescent males because it is a hormonesensitive tumor. Juvenile angiofibroma free download as powerpoint presentation. Juvenile nasopharyngeal angiofibroma with orbital extension. Juvenile angiofibroma is a rare, benign, rich vascular tumor, and approximately one new case is diagnosed in denmark each year. Juvenile nasopharyngeal angiofibroma jna is rare in patients older than 25 years. Juvenile nasopharyngeal angiofibroma is the most common. Juvenile nasopharyngeal angiofibroma jna is a locally aggressive benign vascular tumour exclusively amongst adolescence males. Juvenile nasopharyngeal angiofibroma jna is a rare and benign but locally aggressive fibrovascular tumor arising from the posterolateral wall of the. It has been shown to contain cytosolic androgen receptors and to regress with estrogen therapy. Management of juvenile nasopharyngeal angiofibroma. Mar 07, 2020 it most commonly affects adolescent males and may grow into fissures of the. Females with juvenile nasopharyngeal angiofibroma jna should undergo genetic testing. Juvenile nasopharyngeal angiofibroma jna is a benign vascular tumor that appears in the nasal cavity. Extensive investigation has been unable to settle this issue in.
Juvenile nasopharyngeal angiofibroma evaluation and workup return to. Juvenile angiofibroma human head and neck clinical medicine. Download fulltext pdf juvenile nasopharyngeal angiofibroma article pdf available in indian journal of dental research 161. Imaging in the diagnosis of juvenile nasopharyngeal.
It most commonly affects adolescent males and may grow into fissures of the. Juvenile nasopharyngeal angiofibroma boston childrens hospital. Craniofacial resection of advanced juvenile nasopharyngeal. Ppt journal juvenile nasopharynx angiofibroma radiation. Jul 15, 2015 juvenile nasopharyngeal angiofibroma jna is a benign neoplasm of the nasopharynx. 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. Juvenile angiofibroma human head and neck clinical.
Reddy ka, mendenhall wm, amdur rj, stringer sp, cassisi nj. Graph of dose volume histogram demonstrating doses delivered to the normal structures and juvenile angiofibroma target. Juvenile nasopharyngeal angiofibroma jna is a benign neoplasm of the nasopharynx. Given its vascularity, prominent flow voids are seen on mri leading to a salt and pepper appearance. Although the use of nonsurgical therapies is described in the literature, surgery is currently considered the ideal. As the name implies, jna is a disease of the young and occurs almost exclusively in adolescent males. 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. Juvenile nasopharyngeal angiofibroma knowledge for medical. Angiofibroma nasofaring juvenile free download as pdf file. Juvenile nasopharyngeal angiofibroma jna is a benign tumor that tends to bleed and occurs in the nasopharynx of prepubertal and adolescent males. This is a histologically benign lesion with very aggressive local behavior.
The pictures used in this presentation have been obtained from a number of sources. Juvenile nasopharyngeal angiofibroma jna is a locally invasive benign tumor which almost exclusively. 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. The use of intensity modulated radiotherapy for the treatment.
The juvenile nasopharyngeal angiofibroma has a characteristic growth in all directions from its origin. A new endoscopic staging system for angiofibromas oncology. Primary radiation therapy for juvenile nasopharyngeal angiofibroma. Nasopharyngeal angiofibroma an overview sciencedirect. Juvenile nasopharyngeal angiofibroma jna jnas are wellvascularized benign lesions typically occurring in adolescent males.
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. Historical treatment of jna has included embolization, surgical resection, and radiation. Juvenile nasopharyngeal angiofibroma for medical students. Surgical management and outcome of juvenile nasopharyngeal. Journal of clinical imaging science imaging in the. The use of intensity modulated radiotherapy for the. Juvenile angiofibroma rads see also with virtual slide. Endoscopic surgery for juvenile nasopharyngeal angiofibroma where are. Juvenile nasopharyngeal angiofibroma stages i and ii. Juvenile nasopharyngeal angiofibroma boston childrens. This is a classical presentation of juvenile nasopharyngeal angiofibroma. A, axial unenhanced t1weighted repetition time tr, 600 milliseconds. To provide an analysis of nine patients with juvenile angiofibroma treated with radiotherapy between june 1975 and june 1987 methods and materials.
Introduction uncommon, benign and extremely vascular tumour up to 0. Juvenile nasopharyngeal angiofibroma is a rare benign tumour of vascular origin found in adolescent males, originating around the sphenopalatine foramen. They are exclusively found in males and if diagnosed in a female patient, genetic testing. Juvenile angiofibroma is a rare, benign, rich vascular tumor, and approximately one new case is diagnosed in denmark each. Juvenile nasopharyngeal angiofibroma jna is a rare, benign neoplasm that accounts for less than 0. Evolution of management article pdf available in international journal of pediatrics 201222. The typical patient is an adolescent male with a clinical history of recurrent epistaxis and nasal obstruction.