Juvenile nasopharyngeal angiofibroma jna jnas are wellvascularized benign lesions typically occurring in adolescent males. It most commonly affects adolescent males and may grow into fissures of the. View the article pdf and any associated supplements and figures for a period of 48 hours. Seven cases of juvenile nasopharyngeal angiofibroma were seen in the division of radiation oncology of the department of human oncology, university of wisconsin hospitals from 1961 to 1977. A juvenile nasopharyngeal angiofibroma jna is a noncancerous tumor made up of blood vessels that grow at the posterior aspect of the nose. They concluded that juvenile angiofibroma jna growth and vascularization are driven by factors released from stromal fibroblasts. 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. Jnas are also reported to arise from the posterior aspect of the middle turbinate and rarely from other sites of the nasal cavity and in paranasal sinuses. Juvenile angiofibroma is a rare benign lesion originating from the pterygopalatine fossa with distinctive epidemiologic features and growth patterns.
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 jna is a benign neoplasm of the nasopharynx. The age range for this disease is 7 to 19 years old, with most people being diagnosed between 10 and 19 years old. These red erythematous elevated skin lesions papules are tumors madeup of fibrous tissue angiofibroma and occur with tuberous sclerosis. Juvenile nasopharyngeal angiofibroma jna is a histologically benign vascular tumor that arises in boys between the ages of 12 and 15 years, with only rare cases reported in patients younger than 10 or older than 25. Juvenile nasopharyngeal angiofibroma jna is a rare and benign but locally aggressive fibrovascular tumor arising from the posterolateral wall of the. Sclerosing naevus these usually do not exhibit vascular ectasia. The tumour is dissected free until all that remains is the pedicle. Juvenile nasopharyngeal angiofibroma has been documented since the time of hippocrates 4 bc. The patient underwent preoperative embolization and an uneventful resection. Juvenile nasopharyngeal angiofibroma electronic journal of.
Juvenile nasopharyngeal angiofibroma iowa head and neck. Review article nasopharyngeal angiofibroma ashutosh hota s. Juvenile nasopharyngeal angiofibroma jna is a benign vascular tumor that appears in the nasal cavity. In 1906 chareau revived the interest in the study of juvenile nasopharyngeal angiofibroma. After examining you or your child and asking about your symptoms, your doctor will try to take a look at the tumor. Juvenile nasopharyngeal angiofibroma is a rare, benign tumor that occurs most often in adolescent males.
The pictures used in this presentation have been obtained from a number of sources. Juvenile nasopharyngeal angiofibroma is a benign but locally invasive tumor accounting for about 0. Surgical resection of juvenile angiofibroma vula university of. However, the extensions of the tumor seem to be independent, each one with distinct behavior. Sep 01, 2008 juvenile nasopharyngeal angiofibroma is a rare, benign tumor that occurs most often in adolescent males. The opinions or assertions contained herein are the private ones of the author and are not to be construed as official or necessarily as reflecting the views of the medical department of the navy or of the naval service at large. Although it is a rare neoplasm, juvenile nasopharyngeal angiofibroma jna is associated. Recurrence of juvenile angiofibroma after surgical removal is commonly ranging from 20% to 30%. Only a doctor can diagnose nasopharyngeal angiofibroma. The tumor cells consist of granular cytoplasm, which may be confused with a granular cell tumor. Pictures of skin diseases and problems angiofibroma. Well circumscribed but unencapsulated polypoid fibrous mass, bleeds severely on manipulation and biopsy, may occlude nares. Nasoangiofibroma youth is a highly vascularized tumor almost exclusively male adolescents presentation. May 16, 2018 juvenile nasopharyngeal angiofibroma jna occurs exclusively in males.
Mar 07, 2020 it most commonly affects adolescent males and may grow into fissures of the. This is a concise presentation for medical students especially from india. Juvenile nasopharyngeal angiofibroma jna is the most common type of benign nasopharyngeal tumor, accounting for 0. Juvenile nasopharyngeal angiofibroma for medical students. 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. Juvenile nasopharyngeal angiofibroma jna is an uncommon, slowgrowing, benign but locally invasive vascular tumor arising from tissues in the sphenopalatine foramen inside the nasal cavity. Juvenile angiofibroma information mount sinai new york. What are the treatment options for juvenile nasopharyngeal angiofibroma. Juvenile nasopharyngeal angiofibromas jna are a rare benign, but locally aggressive, vascular tumor.
Staging systems for juvenile nasopharyngeal angiofibroma. Angiofibroma nasofaring belia pdf juvenile nasopharyngeal angiofibroma is a pathologically benign yet locally aggressive and destructive vascular lesion of head and neck. Juvenile nasopharyngeal angiofibroma jna is a locally aggressive benign vascular tumour exclusively amongst adolescence males. Juvenile nasopharyngeal angiofibroma is a rare, highly luiz a cr, romualdo s lt, fava a s. Juvenile nasopharyngeal angiofibroma radiology reference. How is juvenile nasopharyngeal angiofibroma jna diagnosed. Despite tumor reduction with hormones, this approach is not routinely used. Unlimited viewing of the article pdf and any associated supplements and figures. Juvenile angiofibromas msd manual consumer version. The diseasefree period ranged from 10 to 36 months postoperatively. When multiple angiofibromas are seen in an individual, particularly clustered over the central face, the differential diagnosis should include the tuberous sclerosis complex, and appropriate.
Examination under anesthesia and tomography and angiography were very rewarding in determining the full extent of tumor. Juvenile angiofibroma definition of juvenile angiofibroma. Juvenile nasopharyngeal angiofibromas occur almost exclusively in males and usually in adolescence 15 years. Although the use of nonsurgical therapies is described in the literature, surgery is currently considered the ideal treatment for. 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. The typical patient is an adolescent male with a clinical history of recurrent epistaxis and nasal obstruction. Juvenile nasopharyngeal angiofibroma jna is a rare benign tumor arising predominantly in the nasopharynx of adolescent males. Imaging findings are most compatible with a juvenile nasopharyngeal angiofibroma. Intricate mixture of stellate and staghorn blood vessels with variable vessel wall thickness ranging from single layer of endothelium to variable smooth muscle coat.
Axial ct, soft tissue and bone windows, at the level of the maxillary sinus show characteristic features of juvenile nasopharyngeal angiofibroma. Juvenile nasopharyngeal angiofibroma jna is a rare and benign but locally aggressive fibrovascular tumor arising from the. Angiofibroma is a benign noncancerous nasal cavity tumor that almost exclusively affects adolescent boys. Juvenile angiofibroma is a rare benign lesion originating from the. Features are characteristic of a juvenile angiofibroma. First line therapy is usually surgery, with the goal of removing as much of the tumor as possible.
Although rare cases have been reported in females, the tumor has such a strong male. Angiofibroma nasofaring juvenile free download as pdf file. Ppt juvenile nasopharyngeal angiofibroma powerpoint. Juvenile nasopharyngeal angiofibroma jna diagnosis and. Chief, eye, ear, nose and throat service, united states naval hospital, oakland, calif. S100 stain will highlight the melanocytes, and will be negative in fibrous papules. Its tumorlike behavior causing obstruction of the airway and its fibrovascular structure giving rise to severe hemorrhage have long. Juvenile angiofibroma rads see also with virtual slide. The juvenile nasopharyngeal angiofibroma has a characteristic growth in all directions from its origin. Schuon et al reported on the immunohistochemical analysis of growth mechanisms in juvenile nasopharyngeal angiofibroma. 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. 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. It is a rare, benign, vascular neoplasm that accounts for less than 0. 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.
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. As a solitary lesion, there are no associated systemic implications. Jul 15, 2015 juvenile nasopharyngeal angiofibroma jna is a benign neoplasm of the nasopharynx. Treatments for juvenile nasopharyngeal angiofibroma. The lessons learned from the personal care of 44 male patients suffering from juvenile angiofibroma are discussed with reference to natural history, pathogene. Imaging in the diagnosis of juvenile nasopharyngeal angiofibroma article pdf available in journal of clinical imaging science 3suppl 1. Angiofibromas are benign, vascular tumors that most often occur in young, adolescent males on the head and neck. Adolescents and young adults between 14 and 25 years are affected, and there is a distinct male predominance. Juvenile nasopharyngeal angiofibroma evaluation and workup return to.
However, a wide age range of individuals may be affected. Their use is purely for academic and teaching purposes. Although the use of nonsurgical therapies is described in the literature, surgery is currently considered the ideal. They often grow quickly during puberty, then slow down or. Angiofibroma juvenile nasopharyngeal angiofibroma upmc. The method of treatments and the end results are discussed. Complications in the endoscopic and endoscopicassisted treatment. Pdf imaging in the diagnosis of juvenile nasopharyngeal. Pdf juvenile angiofibroma is a rare benign lesion originating from the pterygopalatine fossa. Two patients had undergone a previous operation at another hospital. Nasopharyngeal angiofibroma symptoms aurora health care. Juvenile nasopharyngeal angiofibroma jna occurs exclusively in males. Juvenile angiofibroma free download as powerpoint presentation. Pdf juvenile nasopharyngeal angiofibroma researchgate.
Females with juvenile nasopharyngeal angiofibroma jna should undergo genetic testing. The juvenile nasopharyngeal angiofibroma is a benign neoplasm of the posterior. In some situations, depending on where the tumor has spread, complete removal may not be possible. Juvenile angiofibroma human head and neck clinical medicine. Juvenile nasopharyngeal angiofibroma pubmed central pmc. Introduction uncommon, benign and extremely vascular tumour up to 0. Genetic evidence that juvenile nasopharyngeal angiofibroma is. Given its vascularity, prominent flow voids are seen on mri leading to a salt and pepper appearance. It most commonly affects adolescent males because it is a hormonesensitive tumor.
General characteristics, management and outcomes of 11 cases of jna presented to our centre between 2000 till. Juvenile nasopharyngeal angiofibroma is a rare benign tumour of vascular origin found in adolescent males, originating around the sphenopalatine foramen. Age and sex distribution angiofibroma of skin is a relatively common tumor that is observed typically in middleaged adults. May 16, 2018 despite tumor reduction with hormones, this approach is not routinely used. Infact hippocrates goes on to describe a polyp in the nose which weeped blood. Common practice is to excise the tumor with open or endoscopic surgery.
Examination under anesthesia and tomography and angiography were very rewarding in determin. Juvenile nasopharyngeal angiofibroma knowledge for. The patient in this photo has several facial angiofibromas. Juvenile nasopharyngeal angiofibroma boston childrens. Starting with a brief introduction to the history, etiology and relevant anatomy, the book goes on to discuss. Nasopharyngeal angiofibroma an overview sciencedirect. Dermal dendritic cells usually stain for factor xiiia. Juvenile angiofibroma is a noncancerous growth that causes bleeding in the nose and sinuses. Men and boys who have frequent bloody noses and trouble breathing through their nose should visit the doctor.
We report the case of a 17yearold male who presented in 1995 with a mass filling the left posterior nasal cavity. The tumors are fleshcolored to reddish and approximately 1 to 5 millimeters in diameter. Nineteen patients with juvenile nasopharyngeal angiofibroma jna were surgically treated with different techniques from january 1968 through december 1985. Juvenile angiofibroma is a rare noncancerous benign tumor that grows in the back of the nose, in the. Shaheen in 1930 reported the first female patient with juvenile nasopharyngeal. Genetic evidence that juvenile nasopharyngeal angiofibroma. Juvenile nasopharyngeal angiofibroma jna onset most commonly is in the second decade. Patients usually present with nasal obstruction and epistaxis. 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. The tumor contains many blood vessels and spreads within the area in which it started locally invasive. The natural history, pathogenesis, and treatment of juvenile. This uncommon lesion of the upper air passages has been a wellestablished entity for at least 100 years but its precise nature and origin are still subject to debate. It is not as invasive as to surgery that is almost free of acute complications in. Although it is nonmalignant not cancerous, it can expand quickly and extensively.
Surgical management and outcome of juvenile nasopharyngeal. Diagnosis, staging, and treatment of juvenile nasopharyngeal. The tumour is probably a form of vascular malformation and consists of irregular vascular endotheliallined spaces embedded in a fibrous stroma. Juvenile angiofibroma ja is a benign vascular neoplasm which affects young males between 9 and 19 years of age and accounts for 0. Juvenile nasopharyngeal angiofibromas are a rare benign, but locally aggressive, vascular tumours that occur almost exclusively in young men on imaging, they present as a vividly enhancing softtissue masses centred on the sphenopalatine foramen. Juvenile nasopharyngeal angiofibroma jna is rare in patients older than 25 years. 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.
Table 2 radkowski classification of juvenile nasopharyngeal angiofibroma. In this video, we are going to see about juvenile nasopharyngeal angiofibroma. Juvenile nasopharyngeal angiofibroma knowledge for medical. There are many classification described although none is. Juvenile nasopharyngeal angiofibroma a free powerpoint ppt presentation displayed as a flash slide show on id. Jnas occur almost exclusively in the nasopharynx of adolescent. It also may be referred to as juvenile nasopharyngeal angiofibroma jna. 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.
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