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. First line therapy is usually surgery, with the goal of removing as much of the tumor as possible. Staging systems for juvenile nasopharyngeal angiofibroma. Juvenile nasopharyngeal angiofibroma jna is a rare and benign but locally aggressive fibrovascular tumor arising from the posterolateral wall of the. Although the exact pathogenesis of the tumour is not yet known, natural history and growth patterns can be predicted. Pdf juvenile nasopharyngeal angiofibroma researchgate. 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 nasopharyngeal angiofibroma jna springerlink. A juvenile nasopharyngeal angiofibroma jna is a noncancerous tumor made up of blood vessels that grow at the posterior aspect of the nose.
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 evaluation and workup return to. According to this classification, the angiofibroma is classified as type i when the tumor is restricted to the nasal cavity and the nasopharynx without bone. In 1906 chareau revived the interest in the study of juvenile nasopharyngeal angiofibroma. However, since a vast majority of the cases do occur between 14 and 25 years of age, this term is retained. Juvenile nasopharyngeal angiofibroma is a benign but locally invasive tumor accounting for about 0. Juvenile nasopharyngeal angiofibromas occur almost exclusively in males and usually in adolescence. Nasal cavity, paranasal sinuses, nasopharynx nasopharyngeal angiofibroma. Given its vascularity, prominent flow voids are seen on mri leading to a salt and pepper appearance.
Juvenile nasopharyngeal angiofibroma jna is a rare and benign but locally aggressive fibrovascular tumor arising from the. Juvenile nasopharyngeal angiofibroma has been documented since the time of hippocrates 4 bc. Nasopharyngeal angiofibroma also called juvenile nasopharyngeal angiofibroma is a histologically benign but locally aggressive vascular tumor that grows in the back of the nasal cavity. Juvenile nasopharyngeal angiofibroma jna is a benign vascular tumor that appears in the nasal cavity.
Juvenile nasopharyngeal angiofibroma iowa head and neck. Angiofibroma nasofaring juvenile pdf nasopharyngeal angiofibroma is a histologically benign but locally aggressive vascular tumor that grows in. Juvenile nasopharyngeal angiofibroma jna is a rare, benign, but locally aggressive tumor occurring almost exclusively in adolescent males. Juvenile nasopharyngeal angiofibroma jna onset most commonly is in the second decade. Originating in the margin of the sphenopalatine foramen, it extends itself to the pterygopalatine fosse, paranasal sinus and nasal cavity, leading to symptoms. Nasopharyngeal angiofibroma symptoms aurora health care.
Juvenile nasopharyngeal angiofibroma jna is a locally aggressive benign vascular tumour exclusively amongst adolescence males. Juvenile nasopharyngeal angiofibroma is a rare benign tumour of vascular origin found in adolescent males, originating around the sphenopalatine foramen. Historical treatment of jna has included embolization, surgical resection, and radiation. 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. Nasopharyngeal angiofibroma an overview sciencedirect. Patients may present with lifethreatening epistaxis. 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. A sevenyear experience with patients with juvenile nasopharyngeal. The typical patient is an adolescent male with a clinical history of recurrent epistaxis and nasal obstruction. 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. Review article nasopharyngeal angiofibroma ashutosh hota. Infact hippocrates goes on to describe a polyp in the nose which weeped blood.
Juvenile nasopharyngeal angiofibroma jna is a rare benign tumor arising predominantly in the. 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 angiofibroma rads see also with virtual slide. Introduction uncommon, benign and extremely vascular tumour up to 0. Surgical management and outcome of juvenile nasopharyngeal. Pdf imaging in the diagnosis of juvenile nasopharyngeal. Juvenile nasopharyngeal angiofibroma jna is a pathologically benign yet locally aggressive and destructive tumor that develops in the choana and nasopharynx. Juvenile nasopharyngeal angiofibroma is a rare, highly luiz a cr, romualdo s lt, fava a s. As the tumor is largely spaceoccupying and highly vascular, patients typically. Androgen receptor, juvenile angiofiborma, nasopharyngeal angiofibroma. Surgical management of juvenile nasopharyngeal angiofibroma. There are many classification described although none is.
The juvenile nasopharyngeal angiofibroma is a benign neoplasm of the posterior. Examination under anesthesia and tomography and angiography were very rewarding in. 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. Angiofibroma juvenile nasopharyngeal angiofibroma upmc. Pdf juvenile angiofibroma jna is a relatively uncommon, highly vascular and benign tumor that presents most commonly in adolescent males. The pictures used in this presentation have been obtained from a number of sources. Although rare cases have been reported in females, the tumor has such a strong male. Juvenile angiofibroma is a rare benign lesion originating from the pterygopalatine fossa with distinctive epidemiologic features and growth patterns. Juvenile nasopharyngeal angiofibroma is an uncommon tumor found in the nasopharynx of the adolescent male.
Juvenile nasopharyngeal angiofibroma for parents nemours. These tumors have always been a challenge for skullbase surgeons globally. Surgical resection of juvenile angiofibroma vula university of. It is a highly vascularized slow growing tumor, but locally invasive and destructive. Objective to develop a new staging system for juvenile nasopharyngeal angiofibroma that reflects changes in surgical approaches endonasal, route of intracranial extension, and the extent of vascular supply from the internal carotid artery. The juvenile nasopharyngeal angiofibroma jna is a highly nasoangiofibroma youth is a highly vascularized tumor almost exclusively male. Intracranial involvement has been reported to occur. Juvenile nasopharyngeal angiofibroma jama otolaryngology. Juvenile angiofibroma ja is a benign, highly vascular tumor which is. A juvenile nasopharyngeal angiofibroma jna is a growth in the area behind the nose. General characteristics, management and outcomes of 11 cases of jna presented to our centre between 2000 till 2015 were studied and evaluated. Juvenile nasopharyngeal angiofibroma jna is a rare benign tumor arising predominantly 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.
The method of treatments and the end results are discussed. Treatments for 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. These growths are benign not cancer, but can damage nerves and bones and block ear and sinus drainage.
Juvenile nasopharyngeal angiofibroma jna is a rare benign nasopharyngeal tumor that accounts for 0. Fisch classification, however, is currently accepted. They often grow quickly during puberty, then slow down or stop growing entirely after adolescence. Juvenile nasopharyngeal angiofibroma jna jnas are wellvascularized benign lesions typically occurring in adolescent males. Juvenile nasopharyngeal angiofibroma is described as a rare and benign disease, which is mainly diagnosed in male adolescents.
Sirolimus for the treatment of juvenile nasopharyngeal. Treatment for nasopharyngeal angiofibroma jna is primarily surgical. In some situations, depending on where the tumor has spread, complete removal may not be possible. The age range for this disease is 7 to 19 years old, with most people being diagnosed between 10 and 19 years old. Juvenile nasopharyngeal angiofibroma radiology reference. Ppt journal juvenile nasopharynx angiofibroma free download as powerpoint presentation. 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. Juvenile nasopharyngeal angiofibroma electronic journal of. Patients usually present with nasal obstruction and epistaxis. Jna is classified as type i when the tumor is restricted to the nasal cavity and the nasopharynx without bone. Shaheen in 1930 reported the first female patient with juvenile nasopharyngeal.
Angiofibroma is a benign noncancerous nasal cavity tumor that almost exclusively affects adolescent boys. Juvenile nasopharyngeal angiofibroma jna is a benign, locally aggressive tumor found commonly in young boys. Juvenile nasopharyngeal angiofibroma evaluation and workup. Juvenile nasopharyngeal angiofibroma boston childrens. This book, the first of its kind devoted to the management of this tumor, is based on dr n. Juvenile nasopharyngeal angiofibroma jna is a rare, benign, highly vascular, and locally aggressive tumor that predominantly occurs in adolescent males. It is a highly vascular, locally invasive, nonencapsulated lesion which begins in the pubescent period and causes varying symptoms during the adolescent years. Juvenile nasopharyngeal angiofibroma jna is a rare, histologically benign skull base tumor that develops in adolescents and young.
It is an aggressive neoplasm and shows a propensity for destructive local spread often extending to the base of the skull and into the cranium. It also may be referred to as juvenile nasopharyngeal angiofibroma jna. Juvenile nasopharyngeal angiofibroma is a pathologically benign yet locally aggressive and destructive vascular lesion of head and neck. Janakirams paradigm shifting management technique for these highly vascular tumors. Although it is nonmalignant not cancerous, it can expand quickly and extensively. What are the treatment options for juvenile nasopharyngeal angiofibroma. Although the use of nonsurgical therapies is described in the literature, surgery is currently considered the ideal treatment for. Juvenile nasopharyngeal angiofibromas are benign yet aggressive anterior skull base tumors that. Seven cases of biopsyproved juvenile nasopharyngeal angiofibroma are presented. Review article nasopharyngeal angiofibroma ashutosh hota s. Their use is purely for academic and teaching purposes. Juvenile nasopharyngeal angiofibroma is a rare tumor in adolescent males.
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