Seen almost exclusively in adolescent males of 1020 years it is encapsulated, slowgrowing,vascular tumor although benign it is locally aggressive and has a high recurrence rate 3. Its etiology is typically from the anterior nares, although we present a case of epistaxis from an uncommon source juvenile nasopharyngeal angiofibroma. Nasopharyngeal angiofibroma na is a rare, nonencapsulated and highlyvascularized benign tumor that primarily affects adolescent males 1,2. Review article nasopharyngeal angiofibroma ashutosh hota s. Juvenile nasopharyngeal angiofibroma and familial adenomatous. 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 jna is a rare and benign but locally aggressive fibrovascular tumor arising from the posterolateral wall of the. This location and pattern of spread in a young adult or teenager is typical of juvenile angiofibroma. In this report, there is given an account of a fibroma of this type that occurred in the mouth of a boy aged 10 years in a close relation to the dental structures. As a result, unilateral nasal obstruction and recurrent epistaxis are. Even for an experienced pathologist accurate diagnosis of an angiofibroma is difficult when its location is an extremely rare one. Juvenile nasopharyngeal angiofibroma knowledge for medical. Mean age at diagnosis is 14 years may regress in late teens but may persist into adulthood rare in patients older than 25 years.
We present the case of a 14yearold male harboring a jna, presenting with an active severe and persistent epistaxis. 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. Vessels are thinwalled, lack elastic fibers, have absent or incomplete smooth muscle, and vary in appearance from stellate or staghorn to barely conspicuous because of stromal compression. Mccluggage wg, ganesan r, hirschowitz l, rollason tp. May 16, 2018 nasopharyngeal angiofibroma is usually encapsulated and composed of vascular tissue and fibrous stroma with coarse or fine collagen fibers. Juvenile nasopharyngeal angiofibroma jna is a benign tumor that tends to bleed and occurs in the nasopharynx of prepubertal and adolescent males.
Laboratory findings blood count and chemistry with liver enzymes were normal at the beginning and also after one and 4 weeks of treatment. Facial angiofibromas may be present at any age but they usually develop in late infancy and progressively increase in number and size afterwards. Juvenile nasopharyngeal angiofibroma is an enigma to physicians and scientists. Facial angiofibroma skin cream the safety and scientific validity of this study is the responsibility of the study sponsor and investigators. Management of nasopharyngeal angiofibroma in a 72yearold. Ppt juvenile nasopharyngeal angiofibroma powerpoint. Evolution of management article pdf available in international journal of pediatrics 201222. Juvenile nasopharyngeal angiofibroma is a benign but locally invasive tumor accounting for about 0. The vascular component of the lesion is prone to bleeding and responsible for its clinical malignancy. Nasopharyngeal angiofibroma is usually encapsulated and composed of vascular tissue and fibrous stroma with coarse or fine collagen fibers. It shows very aggressive behavior due to local invasiveness and is associated with various symptoms.
Comment in autosomal dominant tumor syndromes, such as men1 and ts, affected individuals are presumably born with one normal copy and one abnormal mutated copy of the responsible tumor suppressor gene. A sevenyear experience with patients with juvenile. To better understand jna, the physician scientists in our programs are currently conducting a study involving whole. All the relevant articles indexed in pubmed and lilacs, besides reference book chapters, published between 1959.
Management 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. Longterm results of radiation therapy for juvenile nasopharyngeal angiofibroma. Juvenile angiofibroma human head and neck clinical medicine. The evaluation and management of epistaxis in children are discussed separately. The patient underwent preoperative embolization and an uneventful resection.
Feb 10, 2017 this location and pattern of spread in a young adult or teenager is typical of juvenile angiofibroma. In juvenile nasopharyngeal angiofibromas, one of the most important. Recurrence of juvenile angiofibroma and its prevention. Reddy ka, mendenhall wm, amdur rj, stringer sp, cassisi nj.
Juvenile nasopharyngeal angiofibroma jna onset most commonly is in the second decade. Juvenile angiofibroma ja is a benign, highly vascular tumor which is diagnosed. Extranasopharyngeal angiofibroma arising from the infratemporal. Juvenile nasopharyngeal angiofibroma jna is a rare, benign, but locally aggressive tumor occurring almost exclusively in adolescent males. The diagnosis can easily be made by the symptoms, the typical angiographic pattern and the endoscopic picture. Sep 01, 2008 juvenile nasopharyngeal angiofibroma is a rare, benign tumor that occurs most often in adolescent males. Hippocrates described the tumor in the 5th century bc, but friedberg first used the term angiofibroma in 1940. 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. These are markedly vascular lesions and can present with lifethreatening epistaxis. Due to aggressive local growth, skull base location and risk of profound hemorrhage, na is a challenge for surgeons. Adolescents and young adults between 14 and 25 years are affected, and there is a distinct male predominance. Ebm question is morbidity less with endoscopic resection blood loss, cosmesis, cn injury. Nobody yet understands why this disease appears almost always in adolescent boys or why it disappears when those patients reach their 20s and 30s.
Staging of juvenile nasopharyngeal angiofibromas is performed with crosssectional imaging and relies on the identification of local tumor extent, and invasion of adjacent spaces. T1 and t2weighted images in the axial plane as well as t1weighted images in the sagittal plane were acquired prior to. Pediatric dermatology of north texas specializes in challenging skin conditions in infants, children, adolescents, and teens. Juvenile nasopharyngeal angiofibroma radiation therapy. Juvenile angiofibroma definition juvenile angiofibroma is a noncancerous growth that causes bleeding in the nose and sinuses. May 16, 2018 juvenile nasopharyngeal angiofibroma jna occurs exclusively in males. The diagnosis of angiofibroma is dependent more on histological confirmation due to its close resemblance with other lesions, such as angiomyoma, haemangioma, lymphangioma and haemangiopericytoma. Juvenile nasopharyngeal angiofibroma jna is the most common benign neoplasm of the nasopharynx, but it accounts for only 0. Though it is a benign tumor, it is locally invasive and can invade the. Juvenile nasopharyngeal angiofibromas staging radiology.
The tumor contains many blood vessels and spreads within the area in which it started locally invasive. Listing a study does not mean it has been evaluated by the u. Staging of tumors is very important in treatment and surgical decision making, as well as in. Angiofibroma is a histologically benign but locally invasive tumour of the blood. Juvenile nasopharyngeal angiofibroma, surgical approach, recurrence, residual disease introduction juvenile nasopharyngeal angiofibroma jna is a relatively rare tumor occurring mainly in adolescent boys. We report the case of a 17yearold male who presented in 1995 with a mass filling the left posterior nasal cavity. Juvenile nasopharyngeal angiofibroma pediatric research and. In the general population, jna occurs at an incidence of roughly 1. Epistaxis is a common complaint seen in the younger population.
The content on this site is presented in a summary fashion, and is intended to be used for educational and entertainment purposes only. An angiofibroma consists of vascular and interstitial cells that express angiogenic factors such as vascular endothelial growth factor vegf. Nasopharyngeal a n g i o f i b ro m a angela blount, mda, kristen o. Juvenile angiofibroma free download as powerpoint presentation. However, the extensions of the tumor seem to be independent, each one with distinct behavior. Pdf juvenile nasopharyngeal angiofibroma researchgate. Cellular angiofibroma and related fibromatous lesions of the vulva. Juvenile nasopharyngeal angiofibromas staging dr jeremy jones and assoc prof frank gaillard et al.
Juvenile nasopharyngeal angiofibroma most common benign tumor of nasopharynx. Keywords juvenile nasopharyngeal angiofibroma endoscopic surgery embolization sinus surgery epistaxis sinonasal tumor skull base tumor nasopharyngeal tumor. Juvenile nasopharyngeal angiofibroma jna is a benign neoplasm of the nasopharynx. Jan 10, 2017 at present, the treatment of choice for juvenile angiofibroma ja is surgery, which has evolved to endoscopic approaches, performed at referral centers. This fact translates into the need to combine gentle movements of tumor. 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. See evaluation of epistaxis in children and management of epistaxis in children. An unusual case of epistaxis juvenile nasopharyngeal. Pdf the management of juvenile nasopharyngeal angiofibroma jna has changed during the last. May 15, 2012 nasopharyngeal angiofibroma na is a rare, vascular tumor affecting adolescent males.
These tumors are benign, however they are locally invasive. Juvenile nasopharyngeal angiofibroma is a rare, benign tumor that occurs most often in adolescent males. Juvenile nasopharyngeal angiofibroma is a rare, highly vascular, and histologically benign tumor, generally observed in male adolescents. Juvenile nasopharyngeal angiofibroma publications pubfacts. Nasopharyngeal angiofibroma na is a rare, vascular tumor affecting adolescent males. The epicenter of ja is located in the sphenopalatine foramen, and its patterns of extension are medial and lateral, complicated due to the deep location of the structures involved and close. Facial angiofibromas treated with topical rapamycin. In 1906 chareau revived the interest in the study of juvenile nasopharyngeal angiofibroma. It is not intended to be and should not be interpreted as medical advice or a diagnosis of any health or fitness problem, condition or disease. At present, the treatment of choice for juvenile angiofibroma ja is surgery, which has evolved to endoscopic approaches, performed at referral centers. Juvenile nasopharyngeal angiofibroma radiology reference. Extremely few studies have described na in elderly individuals.
Download fulltext pdf download fulltext pdf juvenile angiofibroma. Juvenile nasopharyngeal angiofibromas are a rare benign, but locally aggressive, vascular tumors that occur almost exclusively in young men. Patients usually present with nasal obstruction and epistaxis. Juvenile nasopharyngeal angiofibroma is a vascular tumor that typically occurs in men. 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 electronic journal of. The unusual tumour, juvenile angiofibroma, is normally found in the nasopharyngeal region of pubertal males.
The juvenile nasopharyngeal angiofibroma has a characteristic growth in all directions from its origin. Juvenile nasopharyngeal angiofibroma surgical approaches. There is limited evidence regarding the prevalence of nosebleeds in children. Juvenile nasopharyngeal angiofibroma has been documented since the time of hippocrates 4 bc. Juvenile nasopharyngeal angiofibroma is a rare benign vascular tumor of the nasopharynx. Seen almost exclusively in adolescent males of 1020 years it is encapsulated, slowgrowing,vascular tumor although benign it is locally aggressive and has a. For a discussion of this entity please refer to the parent article. Shaheen in 1930 reported the first female patient with juvenile nasopharyngeal.
Angiofibromas are small, reddish brown or even fleshcolored, smooth, shiny, 0. Juvenile nasopharyngeal angiofibroma knowledge for. Common practice is to excise the tumor with open or endoscopic surgery. After informed consent, we decided to treat the lesions with a novel and promising therapy. The juvenile nasopharyngeal angiofibroma is a rare and histologically benign tumour of the adolescent male. The juvenile nasopharyngeal angiofibroma is a rare benign vascular tumor representing about 0.
T1 an unusual case of epistaxis juvenile nasopharyngeal angiofibroma. Angiofibromas have been sporadically described in extanasopharyngeal locations. Surgical management of juvenile nasopharyngeal angiofibroma. Comparison between endoscopic and open surgery in 37 patients. Infact hippocrates goes on to describe a polyp in the nose which weeped blood. It is advantageous to combine midfacial degloving with endoscopy when. Juvenile nasopharyngeal angiofibroma jna is a relatively rare tumor of the nasopharynx, comprising only 0. Endoscopic surgery for juvenile nasopharyngeal angiofibroma where are. Angiofibroma is a relatively rare tumor occurring almost exclusively in adolescent boys. Juvenile nasopharyngeal angiofibroma pediatric research. Juvenile nasopharyngeal angiofibromas occur almost exclusively in males and usually in adolescence 15 years. Nasopharyngeal angiofibroma is a histologically benign tumor composed of stroma and vessels. Introduction nasopharyngeal angiofibroma is a histologically and biologically benign tumor with aggressive behavior due to its location and associated symptoms including significant epistaxis and nasal obstruction 15. For this purpose the following formulation was prescribed.
Primary radiation therapy for juvenile nasopharyngeal angiofibroma. An angiofibroma from a patient with ts did not demonstrate allelic deletion of the men1 gene. It most commonly affects adolescent males and may grow into fissures of the. Juvenile angiofibroma human head and neck clinical. Juvenile nasopharyngeal angiofibroma is a pathologically benign yet locally aggressive and destructive vascular lesion of head and neck region typically affecting adolescent boys. Pediatric dermatology of north texas works with specific compounding pharmacies in the dfw area to offer a topically prepared compound rapamycin that is used offlabel for the treatment of facial angiofibromas in tuberous sclerosis patients. Difficulty breathing through the nose easy bruising. The vascular component of the lesion is prone to bleeding and. Females with juvenile nasopharyngeal angiofibroma jna should undergo genetic testing.
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