Shaheen in 1930 reported the first female patient with juvenile nasopharyngeal. Seven cases of biopsyproved juvenile nasopharyngeal angiofibroma are presented. Juvenile nasopharyngeal angiofibroma is a pathologically benign yet locally aggressive and destructive vascular lesion of head and neck region typically affecting adolescent boys. Oct 18, 2018 juvenile nasopharyngeal angiofibroma jna is an uncommon, benign tumor of the nose that is generally seen in boys. Juvenile nasopharyngeal angiofibroma jna is a benign vascular tumor that appears in the nasal cavity. Longterm results of radiation therapy for juvenile nasopharyngeal angiofibroma. Juvenile nasopharyngeal angiofibroma jna is a relatively rare tumor of the nasopharynx, comprising only 0. Intricate mixture of stellate and staghorn blood vessels with variable vessel wall thickness ranging from single layer of endothelium to variable smooth muscle coat. 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. Mean age at diagnosis is 14 years may regress in late teens but may persist into adulthood rare in patients older than 25 years. There is an enhancing mass expanding and extending into the posterior nasal air space with erosion of the base of the right pterygoid plate and lateral extension to the. In this video, we are going to see about juvenile nasopharyngeal angiofibroma.
Their use is purely for academic and teaching purposes. Although a histopathologically benign tumor of vascular origin, jna behaves. Genetic evidence that juvenile nasopharyngeal angiofibroma is. It most commonly affects adolescent males because it is a hormonesensitive 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 case. Examination under anesthesia and tomography and angiography were very rewarding in determining the full extent of tumor. The study was approved by the institutional ethical committee. Ppt juvenile nasopharyngeal angiofibroma powerpoint. Nasopharyngeal angiofibroma definition of nasopharyngeal. Juvenile angiofibroma ja is a benign vascular neoplasm which affects young males between 9 and 19 years of age and accounts for 0. 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. Origin of the tumor is superior posterior margin of the sphenopalatine foramen and intracranial invasion appears in 10%to. This fact translates into the need to combine gentle movements of.
In some situations, depending on where the tumor has spread, complete removal may. Juvenile nasopharyngeal angiofibroma jna is a benign tumor that presents almost exclusively in males, usually in the early adolescent years. May 16, 2018 patterns of vascularization and surgical morbidity in juvenile nasopharyngeal angiofibroma. Accounting for less that 1% of all head and neck neoplasms, jna typically originates from a site along the posterolateral wall of the nasal cavity fig.
Juvenile nasal angiofibroma jna is a rare sinonasal tumor that is found almost exclusively in adolescent and young adult males. Juvenile angiofibroma ja is a benign, highly vascular tumor which is. This presentation was atypical given the acuity of presentation and. Longterm followup of juvenile nasopharyngeal angiofibromas. Juvenile nasopharyngeal angiofibroma is a rare, highly vascular, and histologically benign tumor, generally observed in male adolescents. 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 nasopharyngeal angiofibroma jna is a rare benign tumor arising predominantly in the nasopharynx of adolescent males. The swelling was reduced with administration of dexamethasone and the jna was surgically resected within 48 hours. Angiofibroma nasofaring adalah suatu tumor jinak nasofaring yang secara histologik jinak dan secara klinis bersifat ganas, karena mempunyai kemampuan mendestruksi tulang dan meluas ke jaringan sekitarnya, seperti ke sinus paranasal, pipi, mata dan tengkorak, serta sangat mudah berdarah yang sulit dihentikan. In addition, the term nasopharyngeal is often used instead of nasal because angiofibromas that occur. Juvenile nasopharyngeal angiofibroma has been documented since the time of hippocrates 4 bc. Angiofibroma nasofaring belia pdf juvenile nasopharyngeal angiofibroma is a pathologically benign yet locally aggressive and destructive vascular lesion of head and neck. Juvenile angiofibroma rads see also with virtual slide.
What are the treatment options for juvenile nasopharyngeal angiofibroma. Although it is a rare neoplasm, juvenile nasopharyngeal angiofibroma jna is associated with high rates of morbidity and mortality, with the potential for. They often grow quickly during puberty, then slow down or stop growing entirely after adolescence. Abstrak angiofibroma nasofaring adalah tumor jinak nasofaring yang secara histopatologis merupakan tumor jinak, tetapi secara klinis bersifat destruktif. It is almost exclusicely encountered in adolescent males 1. Juvenile nasopharyngeal angiofibroma, surgical approach, recurrence, residual disease introduction juvenile nasopharyngeal angiofibroma jna is a relatively rare tumor occurring mainly in adolescent boys.
Early postoperative ct is reliable in detecting or excluding residual disease in patients with jna 2,24. 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. However, the extensions of the tumor seem to be independent, each one with distinct behavior. Juvenile angiofibroma is a rare benign lesion originating from the pterygopalatine fossa with distinctive epidemiologic features and growth patterns. In the general population, jna occurs at an incidence of roughly 1. Juvenile angiofibroma is a noncancerous growth that causes bleeding in the nose and sinuses. Exclusively endoscopic surgery for juvenile nasopharyngeal angiofibroma. Juvenile nasopharyngeal angiofibroma jna is a rare, benign, but locally aggressive tumor occurring almost exclusively in adolescent males.
Juvenile nasopharyngeal angiofibroma boston childrens. Juvenile nasopharyngeal angiofibroma knowledge for medical. Pdf juvenile nasopharyngeal angiofibroma researchgate. Juvenile nasopharyngeal angiofibroma originates in the sphenopalatine forame, causing epistaxes and. Endoscopic endonasal resection of juvenile nasopharyngeal. A juvenile nasopharyngeal angiofibroma jna is a noncancerous tumor made up of blood vessels that grow at the posterior aspect of the nose. Juvenile nasopharyngeal angiofibromas are a rare benign, but locally aggressive, vascular tumors that occur almost exclusively in young men. It is the most common benign tumor of the nasopharnyx but only accounts for less than 0. They often grow quickly during puberty, then slow down or. Juvenile nasopharyngeal angiofibroma evaluation and workup return to.
Juvenile nasopharyngeal angiofibroma knowledge for. Reddy ka, mendenhall wm, amdur rj, stringer sp, cassisi nj. Bedah sinonasal endoskopik angiofibroma nasofaring belia. Juvenile nasopharyngeal angiofibroma jna is a benign tumor that tends to bleed and occurs in the nasopharynx of prepubertal and adolescent males.
Angiofibroma nasofaring pada pasien usia lanjut sony yudianto a, tjekeg m, ardika nuaba g. We describe a case of a 24yearold male presenting urgently with a juvenile nasopharyngeal angiofibroma jna with difficulty breathing, inability to swallow, and respiratory distress following throat swelling. Juvenile nasopharyngeal angiofibroma for medical students. Nasopharyngeal angiofibroma symptoms aurora health care. Juvenile nasopharyngeal angiofibroma jna is an uncommon, benign tumor of the nose that is generally seen in boys. The tumor is locally aggressive and can invade into surrounding structures and cause significant complications. The typical patient is an adolescent male with a clinical history of recurrent epistaxis and nasal obstruction. Juvenile nasopharyngeal angiofibromas jna are a rare benign, but locally aggressive, vascular tumor. Treatments for juvenile nasopharyngeal angiofibroma. Juvenile nasopharyngeal angiofibroma jna is rare in patients older than 25 years. Although the use of nonsurgical therapies is described in the literature, surgery is currently considered the ideal treatment for. Juvenile nasopharyngeal angiofibroma linkedin slideshare. Juvenile nasopharyngeal angiofibroma presenting with acute. It most commonly affects adolescent males and may grow into fissures of the.
It shows very aggressive behavior due to local invasiveness and is associated with various symptoms. In some situations, depending on where the tumor has spread, complete removal may not be possible. The tumour is sessile or polypoid and is histologically benign, but has a tendency to recur and is locally destructive, causing pressure necrosis of adjacent soft tissue and bone. Tumor ini sering terjadi pada lakilaki prepubertas dan remaja, jarang ditemukan pada pasien. Juvenile nasopharyngeal angiofibroma jna is a benign neoplasm of the nasopharynx. 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. Infact hippocrates goes on to describe a polyp in the nose which weeped blood. Genetic evidence that juvenile nasopharyngeal angiofibroma.
Complications in the endoscopic and endoscopicassisted treatment. Juvenile nasopharyngeal angiofibroma of jna 1,5,11 figure 2. Juvenile nasopharyngeal angiofibroma otorrinos2dos blog. Treatments for juvenile nasopharyngeal angiofibroma boston. Juvenile nasopharyngeal angiofibroma is described as a rare and benign disease, which is mainly diagnosed in male adolescents. Juvenile nasopharyngeal angiofibromas occur almost exclusively in males and usually in adolescence 15 years. Madana dkk 10 melaporkan 1 kasus angiofibroma ektra nasofaring pada wanita usia 37 tahun. The juvenile nasopharyngeal angiofibroma jna is a highly vascularized tumor almost.
Juvenile nasopharyngeal angiofibroma sciencedirect. Juvenile nasopharyngeal angiofibroma boston childrens hospital. Originating in the margin of the sphenopalatine foramen, it extends itself to the pterygopalatine fosse, paranasal sinus and nasal cavity. T jlh he socalled juvenile nasopharyngeal angiofibroma is a relatively uncommon benign neoplasm that primarily affects adolescent males. Management of juvenile nasopharyngeal angiofibroma. Though it is a benign tumor, it is locally invasive and can invade the. Park dkk9 juga melaporkan 1 kasus angiofibroma nasofaring pada lakilaki usia 48 tahun yang telah meluas ke intraorbital. Sessions et al 1, fisch 2, chandler et al 3, radkowski et al 4, and onerci et al 5 have proposed staging or classification systems for jna.
Ppt journal juvenile nasopharynx angiofibroma free download as powerpoint presentation. Introduction uncommon, benign and extremely vascular tumour up to 0. 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. This is a concise presentation for medical students especially from india. Endoscopic removal of juvenile nasopharyngeal angiofibromas. The records of 37 patients with tumors extending laterally from the nasopharynx were included in the study. The most common presenting symptom is painless nasal obstruction or epistaxis. Previously, we used fischs system to study the diagnosis and management of jnas.
This is a histologically benign lesion with very aggressive local behavior. Juvenile nasopharyngeal angiofibroma jna onset most commonly is in the second decade. Abstract juvenile nasopharyngeal angiofibroma jna is a pathologically benign yet locally aggressive and destructive tumor that develops in. 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 angiofibroma iowa head and neck. Sinonasnasopharyngeal angiofibroma juvenile with subsequent. Review article nasopharyngeal angiofibroma ashutosh hota.
In 1906 chareau revived the interest in the study of juvenile nasopharyngeal angiofibroma. Females with juvenile nasopharyngeal angiofibroma jna should undergo genetic testing. Two types of lateral extension in juvenile nasopharyngeal. First line therapy is usually surgery, with the goal of removing as much of the tumor as possible. May 16, 2018 juvenile nasopharyngeal angiofibroma jna occurs exclusively in males. Hippocrates described the tumor in the 5th century bc, but friedberg first used the term angiofibroma in 1940. A concise classification system and appropriate treatment options.
Well circumscribed but unencapsulated polypoid fibrous mass, bleeds severely on manipulation and biopsy, may occlude nares. Although histologie findings indicate benignancy, it is a clinically aggressive tumor of naso pharyngeal origin that tends also to involve adjacent maxillary, palatal, and cranial structures by extension. Adolescents and young adults between 14 and 25 years are affected, and there is a distinct male predominance. Dec 21, 2015 juvenile angiofibroma is the most common benign nasopharyngeal tumor. Sirolimus for the treatment of juvenile nasopharyngeal angiofibroma. The juvenile nasopharyngeal angiofibroma has a characteristic growth in all directions from its origin. The present article is a retrospective study of surgically treated patients of juvenile nasopharyngeal angiofibroma over a period of 5 years. Typical signs are nasal and eustachian tube obstruction, adenoidal speech, and dysphagia. Juvenile nasopharyngeal angiofibroma radiology reference. Frequently, the term juvenile is not included in the name of this tumor because this lesion may occur in adults occasionally, as well as in adolescents. It is a highly vascularized slow growing tumor, but locally invasive and destructive. The tumor usually arises in puberty and is more common in boys than in girls. These tumors are benign, however they are locally invasive.
Juvenile nasopharyngeal angiofibroma arises in the confluence of the posterolateral nasal wall and the lateral nasopharynx and occurs almost exclusively in males during adolescence. Jul 15, 2015 juvenile nasopharyngeal angiofibroma jna is a benign neoplasm of the nasopharynx. Jnas originate from the posterior choanal tissues and rapidly extend into the surrounding regions, including the nasopharynx, the orbits, and even the intracranial cavity. Patterns of vascularization and surgical morbidity in juvenile nasopharyngeal angiofibroma.
Mri provides additional assessment of the tumors interface with adjacent soft tissue and is particularly valuable in evaluating intracranial and cavernous sinus extension. Aug 24, 2015 endoscopic view of endoscopic resection of jna. The pictures used in this presentation have been obtained from a number of sources. Juvenile nasopharyngeal angiofibroma jna occurs primarily in adolescent males and accounts for 0. Although the use of nonsurgical therapies is described in the literature, surgery is currently considered the ideal. Although it is nonmalignant not cancerous, it can expand quickly and extensively.
Review article nasopharyngeal angiofibroma ashutosh hota s. Juvenile nasopharyngeal angiofibroma jna is a rare histologically benign tumor, highly vascularized, with usually aggressive behavior, and can extend from the nasal cavity to neighboring structures. Recurrent juvenile nasopharyngeal angiofibroma treated. Juvenile nasopharyngeal angiofibroma is a slowly growing tumor that is characterized by submucosal spreading, nonencapsulated, hypervascular, and locally destructive mass 6. Axial ct, soft tissue and bone windows, at the level of the maxillary sinus show characteristic features of juvenile nasopharyngeal angiofibroma. Central stalk joining the 2 components occupy the spf at the upper end of the vertical plate of palatine bone.
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