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