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