Nasopharyngeal angiofibroma naf is a highly vascular, histologically benign. At age 19, routine sigtnoidoscopic surveil lance led to the diagnosis of fap, and he underwent subto tal colectomy. 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. Juvenile nasopharyngeal angiofibroma jna is a rare and benign but locally in the nasopharynx and nasal cavity, leading to manifestations such as nasal. A juvenile nasopharyngeal angiofibroma jna is a growth in the area behind the nose. Juvenile nasopharyngeal angiofibroma, histopathology embolization, treatment, radiosurgery. 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.
Classically adolescent males with recurrent nose bleeds. The juvenile nasopharyngeal angiofibroma jna is a histological benign tumor, almost exclusive of male adolescents, with an incidence of 1. 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. Nasopharyngeal angiofibroma nasopharynx, head and neck, histopathology. Juvenile nasopharyngeal angiofibroma etiology, pathology. Intracranial involvement has been reported to occur. Nasopharyngeal angiofibroma an overview sciencedirect topics. Angiofibroma is a fibrous and vascular tumour that commonly occurs in adolescent males and involves the nasopharynx. Histologically, angiofibromas are characterized by fibrosis of the dermis and blood vessels. In places endothelial cells were only separated from. The clinical and imaging materials of 51 cases of jna diagnosed at our hospital between 1981 and 2011 were collected and. Nasopharyngeal angiofibroma is an uncommon, benign, fibrovascular tumor which exhibits a distinct predilection for adolescent males and accounts for less than 1% of all head and neck neoplasms 1, 2. Pdf on may 1, 2010, s marc stokes and others published nasopharyngeal angiofibroma of the nasal. Angiofibroma juvenile nasopharyngeal angiofibroma upmc.
Nasopharyngeal angiofibroma evaluation and management 44 p j m h s vol. Pdf histopathological classification of nasopharyngeal. Review article nasopharyngeal angiofibroma ashutosh hota s. Nasopharyngeal angiofibroma an overview sciencedirect. Journal of oral and maxillofacial pathology jomfp is the official publication of the indian association of oral and maxillofacial pathology.
Given the strict epidemiology, hormonal influences seem likely as investigators have revealed the presence of androgen, testosterone, and dihydrotestosterone receptors 3, 4 and lack. They often grow quickly during puberty, then slow down or stop growing entirely after adolescence. Juvenile nasopharyngeal angiofibroma a histopathological. Juvenile nasopharyngeal angiofibroma 420 introduction juvenile nasopharyngeal angiofibroma jna represents 0. A clinical, histopathological and immunohistochemical study of 42 cases with emphasis on stromal features. Angiofibroma is a pink, firm, 1 to 2mm domeshaped papule most commonly located over the nasal bridge and cheeks fig. Forces institute of pathology no female patients were. These tumors differed from a n ordinary fibroma in the respect that the vascular elements represented at least one important component of the tumor, and the term juvenile nasopharyngeal angiofibroma as used by martin et al. A juvenile nasopharyngeal angiofibroma jna is a noncancerous tumor made up of blood vessels that grow at the posterior aspect of the nose. Since it occurs in the second decade of life, it is proposed that nasopharyngeal angiofibroma is a testosterone dependent tumour a nidus of hamartomatous vascular tissue in. Nasopharyngeal angiofibroma johns hopkins surgical. Wed like to send you periodic updates regarding pathology educational materials released by our department. Thompson, in head and neck pathology second edition, 20. Nasopharyngeal angiofibroma lester dr thompson, md.
A dose of 3,000 rads in three weeks leads to thrombosis, fibrosis, and tumor regression, although a latent period up. Common symptoms include epistaxis and nasal obstruction. These growths are benign not cancer, but can damage nerves and bones and block ear and sinus drainage. Juvenile nasopharyngeal angiofibroma iowa head and neck. Epidemiology juvenile nasopharyngeal angiofibromas. The juvenile nasopharyngeal angiofibroma is a benign neoplasm of the posterior nasopharynx it occurs almost exclusively in adolescent males, with an average age at diagnosis of 15 years old. The age range for this disease is 7 to 19 years old, with most people being diagnosed between 10 and 19 years old. Fibroangioma, histopathology, juvenile nasopharyngeal angiofibroma, nasopharyngeal neoplasms, surgical approval.
A risk factor is anything that raises your chance of getting a disease such as cancer. Angiofibroma nasofaring belia pdf juvenile nasopharyngeal angiofibroma is a pathologically benign yet locally aggressive and destructive vascular lesion of. Surgical experience with nasopharyngeal angiofibroma. Learn about the risk factors for nasopharyngeal cancer and what you might be able to do to help lower your risk. Juvenile primary extranasopharyngeal angiofibroma, presenting as.
Juvenile nasopharyngeal angiofibroma is the most common benign tumour of the nasopharynx it is usually seen in males in the second decade of life and usually presents with recurrent profuse epistaxis etiology. Pdf imaging in the diagnosis of juvenile nasopharyngeal. Correlating histology, surgical approach and blood loss. Nasopharyngeal angiofibroma symptoms aurora health care. It is described as a rare nasopharyngeal angiofibroma. Between 1948 and 1975, we have seen 48 patients with 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. It also may be referred to as juvenile nasopharyngeal angiofibroma jna. It usually presents with a triad of unilateral nasal obstruction, episodes of spontaneous nasal bleeding and nasalnasopharyngeal mass. This is an uncommon tumour of the posterior nasal cavity and lateral nasopharynx.
Angiofibroma is a benign noncancerous nasal cavity tumor that almost exclusively affects adolescent boys. The nasopharyngeal angiofibroma jama otolaryngologyhead. The nasopharyngeal angiofibroma is an aggressive, vascular tumor that may represent a challenge for the anesthesiologist by the risk of profuse bleeding. Hence the name juvenile nasopharyngeal angiofibroma. Sclerosing naevus these usually do not exhibit vascular ectasia. Most of the tumour vessels, which lacked elastic laminae, were characterized by vascular walls of irregular thickness and variable muscle content.
Juvenile nasopharyngeal angiofibroma electronic journal of. Nasopharyngeal angiofibroma evaluation and management. Juvenile nasopharyngeal angiofibroma has been documented since the time of hippocrates 4 bc. Surgery is the treatment of choice for angiofibroma. To describe a clear and simplified classification system for juvenile nasopharyngeal angiofibroma jna, and to describe suitable management options. Angiofibroma nasofaring belia pdf juvenile nasopharyngeal angiofibroma is a pathologically benign yet locally aggressive and destructive vascular lesion of head and neck. We report the case of a years old, 52 kg, asa1, who was scheduled for the resection of an angiofibroma invading facial sinuses and internal carotid artery, and submitted to external carotid and internal maxillary arteries embolization one. Goldstandard treatment consists in surgical removal, which. Desmoplastic response of the nasopharyngeal periosteum or embryonic fibrocartilage between the basiocciput and the basisphenoid.
Most of the patients presented at an advanced stage. 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. Nasopharyngeal cancer causes, risk factors, and prevention. S100 stain will highlight the melanocytes, and will be negative in fibrous papules. Dermal dendritic cells usually stain for factor xiiia. Youll hear about new websites, ipad apps, pathcasts, and other educational materials.
Thirtytwo cases of nasopharyngeal angiofibroma, including 2 recurrences, all of which had been excised from males between 7 and 25 years, were subjected to systematic immunohistochemical study. The incidence of juvenile angiofibroma is less than 0. 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. Nasopharyngeal angiofibroma naf is a highly vascular, histologically benign, but sometimes locally aggressive tumor of the nasopharynx that occurs almost exclusively in males, usually between 10 and 20 years of age. Juvenile nasopharyngeal angiofibroma jna is a rare, histologically benign skull base tumor that develops in. Carcinoma, endoscopic approach to juvenile nasopharyngeal angiofibroma, nasopharynx.
Juvenile nasopharyngeal angiofibroma makhasana ja, kulkarni ma. The juvenile nasopharyngeal angiofibroma is a benign neoplasm of the posterior. Juvenile angiofibroma rads see also with virtual slide. Angiofibroma juvenil pdf juvenile nasopharyngeal angiofibromas jna are a rare benign but locally aggressive vascular tumor. Nasopharyngeal angiofibroma histopathology youtube. Juvenile nasopharyngeal angiofibroma jna is a benign, locally aggressive tumor found commonly in young boys. Radiotherapy is the treatment of choice for most patients. Given the strict epidemiology, hormonal influences seem likely as investigators have revealed the presence of androgen, testosterone, and dihydrotestosterone receptors 3, 4 and. Nasopharyngeal angiofibroma in patients with familial. Juvenile nasopharyngeal angiofibroma jna springerlink. Despite continuous advance of chinese and international npc histopathological classification research, it was difficult to unify previous with current china classifications, and the china with world health organization who classifications. Evaluation showed a nasopharyngeal mass, and histopathology of the surgical resection specimen revealed angiofibroma.
Pdf nasopharyngeal angiofibroma of the nasal cavity. A number of childhood conditions are characterized by angiofibromas, but the most common is tuberous sclerosis. Nasopharyngeal angiofibroma is a benign but aggressive tumor of unknown etiology, typically occurring in adolescent males. Imaging in the diagnosis of juvenile nasopharyngeal angiofibroma article pdf available in journal of clinical imaging science 3suppl 1. In 1906 chareau revived the interest in the study of juvenile nasopharyngeal angiofibroma. Malignancy mimicking juvenile nasopharyngeal angiofibroma.
Anesthetic management of 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 benign tumor arising predominantly in the nasopharynx of adolescent males. We wish to highlight the importance of complete clinical examination and preoperative imaging in differentiating an ideal management of nasopharyngeal masses. Juvenile nasopharyngeal angiofibroma jna is a rare, benign neoplasm that accounts for less than 0. Juvenile nasopharyngeal angiofibroma radiology reference. There are, however, potentially serious short and longterm complications associated with the use of radiotherapy in the.
Nasopharyngeal angiofibroma is usually encapsulated and composed of vascular tissue and fibrous stroma with coarse or fine collagen fibers. Review article nasopharyngeal angiofibroma ashutosh hota. Juvenile nasopharyngeal angiofibroma evaluation and workup return to. Despite its benign character, it is locally aggressive and vascular.
Infact hippocrates goes on to describe a polyp in the nose which weeped blood. The jna juvenile nasopharyngeal angiofibroma is a rare neoplasm, of benign histology, that affects almost exclusively male sex adolescent. Recent reports have suggested that radiotherapy may be preferable to other forms of treatment of juvenile nasopharyngeal angiofibroma. Nasal cavity, paranasal sinuses, nasopharynx nasopharyngeal angiofibroma. Currently, at age 34, he is free of recurrent angiofibroma. Juvenile nasopharyngeal angiofibroma jna is a rare benign tumor.
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