Juvenile nasopharyngeal angiofibroma also known as nasopharyngeal fibroma is a distinctive tumor that is associated with nasal obstruction, drainage, and epistaxis in adolescent males. A 52yearold man underwent surgery for nasopharyngeal angiofibroma after adjuvant radiofrequencyinduced thermotherapy. Staging of juvenile nasopharyngeal angiofibromas is performed with crosssectional imaging and relies on the identification of local tumor extent, and invasion of adjacent spaces. Magnetic resonance imaging findings ayse gul alimli, murat ucar, cigdem oztunali, koray akkan, oznur boyunaga, cagr. It is the most common benign tumor of the nasopharnyx but only accounts for less than 0. Common practice is to excise the tumor with open or endoscopic surgery. Juvenile nasopharyngeal angiofibroma jna is a rare tumor that predominantly occurs in adolescent males, the average age of occurence being 15. Inspection shows a red, tumorlike lesion along the posterior wall of the nasopharynx. Differential diagnosis cellular angiofibroma surgical. In addition to its usefulness in the diagnosis of this entity, arteriography also has therapeutic implications.
The focus of this chapter will be on nasopharyngeal carcinoma, with a brief overview of a few other lesions. Seen by local otolaryngologist who identified a nasal mass consistent with a jna in the right nasal cavity. 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. All patients had ct scans done and magnetic resonance imaging was required for two patients. 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 infratemporal fossa. Comprehensive preoperative staging system for endoscopic. Even so, these benign tumors can grow extremely rapidly and sometimes spread from the nasal. We aimed to report the characteristic magnetic resonance imaging mri findings and the spread patterns of jna. 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. Here, we describe three patients who received therapy with the mtor inhibitor sirolimus with improvement in clinical symptoms, imaging, and. This type of tumor almost always develops in adolescent boys and might be called a juvenile nasopharyngeal angiofibroma.
Because the location of these tumors makes conventional surgery difficult, interest in endoscopic resection is increasing, particularly for the treatment of lesions that do not extend laterally into the infratemporal fossa. Fourdimensional ct angiography 4dcta in the evaluation. Jul 15, 2015 juvenile nasopharyngeal angiofibroma jna is a rare benign tumor arising predominantly in the nasopharynx of adolescent males. Nasopharyngeal angiofibroma is usually encapsulated and composed of vascular tissue and fibrous stroma with coarse or fine collagen fibers. May 16, 2018 nasopharyngeal angiofibroma is usually encapsulated and composed of vascular tissue and fibrous stroma with coarse or fine collagen fibers. Nasopharyngeal angiofibroma an overview sciencedirect topics. Biopsy should be avoided as to avoid extensive bleeding since the tumor is composed of blood vessels without a muscular coat. 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. Nasal cavity, paranasal sinuses, nasopharynx nasopharyngeal angiofibroma. Surgical management of juvenile nasopharyngeal angiofibroma.
Juvenile nasopharyngeal angiofibroma jna is an uncommon, benign tumor of the nose that is generally seen in boys. 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. We report the case of a 17yearold male who presented in 1995 with a mass filling the left posterior nasal cavity. Nasopharyngeal angiofibroma is a benign, vascular, polypoid fibrous mass in located in the the posterolateral wall of the nasal cavity. The recent imaging modalities like ct scan and mri has further simplified non invasive ways of examining the nasopharynx. Clusters of rounded, epithelioid cells, often around vessels. May 15, 2012 nasopharyngeal angiofibroma na is a rare, vascular tumor affecting adolescent males. Angiofibromas are benign, vascular tumors that most often occur in young, adolescent males on the head and neck. Usually, the presenting symptom is a painless nasal obstruction or epistaxis.
The mr imaging signals of 32 patients with histopathologically. Presenting symptoms were epistaxis and nasal blockage while two patients also had dacryocystitis and secretory otitis media. Most tumors were completely excised using the expanded endonasal. Juvenile nasopharyngeal angiofibroma jna is a rare tumor that exhibits a predictable spreading pattern. Neuroradiologists play a crucial role in the multidisciplinary evaluation and treatment of patients with juvenile nasopharyngeal angiofibroma. The first soft tissue tumour with a relatively pelvic sitespecificity was a benign stromal polyp described in the early 1960. Endoscopic removal of juvenile nasopharyngeal angiofibromas e3. Abstract juvenile nasopharyngeal angiofibroma jna is a benign, highly vascular, and locally invasive tumor. Juvenile nasopharyngeal angiofibroma jna is a rare, benign neoplasm that accounts for less than 0. Nasopharyngeal angiofibroma, an angiographic evaluation. Radiofrequencyinduced thermotherapy of nasopharyngeal. Nasopharynx is located at the confluence of nasal, aural and pharyngeal air passages. Some authors advocate the use of intranasal endoscopic surgery for lesions with limited extension to the infratemporal fossa.
The purpose of this study was to determine the mr imaging features of nasal lobular capillary hemangioma and contrast its imaging characteristics to inverting papilloma. Sep 01, 2008 juvenile nasopharyngeal angiofibroma is a rare, benign tumor that occurs most often in adolescent males. Juvenile nasopharyngeal angiofibroma jna is rare otolaryngology. Tumor and tumorlike lesions of the sinonasal tract may be classified 1 as benign or malignant, 2 as carcinoma, sarcoma, adenocarcinoma, or lymphoma, 3 according to the tissue of origin eg, epithelial, bone, lymphoid, or mesenchymal, or 4 as a combination of above. Although the clinical presentation of these tumors differs vastly from the classically described juvenile nasopharyngeal angiofibroma jna, the histopathology is similar. The lateral attachments of the nasal angiofibroma were incised using the cottle elevator and weilblakesley forceps v. Mar 22, 20 juvenile nasopharyngeal angiofibroma jna is a rare, benign, highly vascular, and locally aggressive tumor that predominantly occurs in adolescent males. The first description of this tumour dates from nucci et al. Stage i tumors limited to nasal cavity, nasopharynx with no bony destruction stage ii tumors invading pterygomaxillary fossa, paranasal sinuses with bony destruction stage iii tumors invading infratemporal fossa, orbit andor parasellar region remaining lateral to cavernous sinus stage iv tumors invading cavernous sinus.
The tumor contains many blood vessels and spreads within the area in which it. Juvenile nasopharyngeal angiofibromas occur almost exclusively in males and usually in adolescence 15 years. Over time, jnas will eventually erode bone and invade the infratemporal fossa. Axial ct, soft tissue and bone windows, at the level of the maxillary sinus show characteristic features of juvenile nasopharyngeal angiofibroma. Cellular angiofibroma ca belongs to soft tissue tumours that predominantly occur in the distal genital tract of both genders. Imaging in the diagnosis of juvenile nasopharyngeal. Juvenile nasopharyngeal angiofibroma jna is a rare, benign, highly vascular, and locally aggressive tumor that predominantly occurs in adolescent males. Radiologists prior knowledge on the tumors characteristics aids in establishing a diagnosis. Sirolimus for the treatment of juvenile nasopharyngeal angiofibroma. The tumors are fleshcolored to reddish and approximately 1 to 5 millimeters in diameter. Endoscopic excision of nasopharyngeal angiofibromas. Pdf juvenile nasopharyngeal angiofibroma in a woman. Although the clinical presentation of these tumors differs vastly from the classically described juvenile nasopharyngeal angiofibroma jna, the histopathology is. Routine and dynamic mr imaging study of lobular capillary.
Nasopharyngeal angiofibroma definition of nasopharyngeal. 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. If nasopharyngeal angiofibroma is suspected based on physical examination a smooth vascular submucosal mass in the posterior nasal cavity of an adolescent male, imaging studies such as ct or mri should be performed. Angiofibromas have been sporadically described in extanasopharyngeal locations. Juvenile nasal angiofibroma jna is a rare sinonasal tumor that is found almost exclusively in adolescent and young adult males. Transnasal endoscopic resection of juvenile nasopharyngeal.
Contrastenhanced ct scanning and mr imaging are valuable imaging modalities in the workup of juvenile nasopharyngeal angiofibroma jna 1. Coffin md, rita alaggio md, in practical soft tissue pathology. Note a disseccao do segundo segmento do nervo trigemeo v2. Review article nasopharyngeal angiofibroma ashutosh hota s. Generally, an angiofibroma presents as 1 to 5 mm skincolored to erythematous domeshape papule on the face. Extranasopharyngeal angiofibromas ena arising exclusively from the nasal septum, nasal septal angiofibromas nsa, are extremely rare with only cases to date. These lesions include fibrous papule, facial angiofibroma, pearly penile papule, adenoma sebaceum, periungual fibroma, and koenens tumor. Though it is a benign tumor, it is locally invasive and can invade the nose, cheek, orbit.
Most authorities agree that surgery is the preferred modality of treatment, at least for primary tumors. Imageguided, endoscopic, laserassisted removal has also recently been used. Oct 18, 2018 juvenile nasopharyngeal angiofibroma jna is an uncommon, benign tumor of the nose that is generally seen in boys. The patient in this photo has several facial angiofibromas. The mainstay of treatment for nasopharyngeal angiofibroma is surgical resection. It was first described by hippocrates, a greek physician, in the 5th century bc. Given its vascularity, prominent flow voids are seen on mri leading to a salt and pepper appearance. Ct scan and mri demonstrate the extent of the tumor. For a discussion of this entity please refer to the parent article.
Introduction uncommon, benign and extremely vascular tumour up to 0. Pdf imaging in the diagnosis of juvenile nasopharyngeal. Juvenile nasopharyngeal angiofibroma jna is a rare benign tumor arising predominantly in the nasopharynx of adolescent males. Typical signs are nasal and eustachian tube obstruction, adenoidal speech, and dysphagia. Nasopharyngeal angiofibroma presents with symptoms of nasal obstruction and epistaxis.
Generally, an angiofibroma presents as 1 to 5 mm skincolored to erythematous. Learn more about juvenile nasopharyngeal angiofibroma treatments from. Magnetic resonance imaging versus computed tomography and. Treatments for juvenile nasopharyngeal angiofibroma boston. These tumors are not cancerous and only rarely become cancerous.
Due to aggressive local growth, skull base location and risk of profound hemorrhage, na is a challenge for surgeons. Juvenile nasopharyngeal angiofibroma jna is a rare, benign, highly vascular, and locally aggressive tumor that predominantly occurs in. Comprehensive preoperative staging system for endoscopic single and multicorridor approaches to juvenile nasal angiofibromas. Juvenile nasopharyngeal angiofibromas staging radiology. The nasopharynx is located at the crossroads between the skull base superiorly, the nasal cavity anteriorly, and the oropharynx inferiorly. Juvenile nasopharyngeal angiofibroma linkedin slideshare. The nose and nasopharynx were involved in four cases and the nose and right maxillary sinus in one. Juvenile nasopharyngeal angiofibromas jna are a rare benign, but locally aggressive, vascular tumor. It most commonly affects adolescent males because it is a hormonesensitive tumor.
Imaging in the diagnosis of juvenile nasopharyngeal angiofibroma. Angiofibroma is a descriptive term for a group of lesions with different clinical presentations but with the same histopathology. Computerized tomography scan also called ct or cat scan, this imaging technique uses a combination of xrays and computer technology to produce cross. Angiofibromas are small, reddish brown or even fleshcolored, smooth, shiny, 0. From a series of 5 cases studied arteriographically, 2 will be reported in some detail to illustrate these points. Nasopharyngeal angiofibroma na is a rare, vascular tumor affecting adolescent males. The tumor usually arises in puberty and is more common in boys than in girls.
The two cardinal symptoms of angiofibroma are nasal obstruction and intermittent unprovoked. Jna frequently involves multiple anatomical compartments which are. Disease processes can spread to and from the nasopharynx through welldefined anatomic pathways. Juvenile nasopharyngeal angiofibroma radiology reference. Video 2 shows nasal endoscopy for the previous patient 24 hours after embolization which was of poor result as only evident in the right periphery of the tumor. Juvenile nasopharyngeal angiofibroma jna is an uncommon benign and highly vascularized tumour with a propensity to local invasiveness and an adolescent males predilection, comprising 0. Review article nasopharyngeal angiofibroma ashutosh hota. Juvenile nasopharyngeal angiofibroma jna onset most commonly is in the second decade. The excision of angiofibroma by pernasal route using endoscope is not new, but it requires preoperative embolization. Nasopharyngeal angiofibroma is a relatively uncommon tumor of adolescence which is usually manifested by massive nosebleeds and nasal obstruction.
Video 1 shows imaging of nasopharyngeal angiofibroma for an adolescent male patient presenting with nasal obstruction, epistaxis for few months. Intricate mixture of stellate and staghorn blood vessels with variable vessel wall thickness ranging from single layer of endothelium to variable smooth muscle coat. Transnasal endoscopic excision of nasopharyngeal angiofibroma. Sharma, ekkehard kasper, onkar deshmukh, iype cherian. Jul 19, 2015 cellular angiofibroma ca represents a quite recently described mesenchymal tumour that occurs in both genders, in particular in the vulvovaginal region in women and in the inguinoscrotal area in men. An angiofibroma is a benign tumor that grows in the nasal cavity. Dec 21, 2015 axial ct, soft tissue and bone windows, at the level of the maxillary sinus show characteristic features of juvenile nasopharyngeal angiofibroma. Nasopharyngeal angiofibroma an overview sciencedirect. Juvenile nasopharyngeal angiofibroma is a rare, benign tumor that occurs most often in adolescent males. Lobular capillary hemangioma is an uncommon lesion, and its mr imaging appearance has not been fully characterized.
Juvenile nasopharyngeal angiofibromas occur almost exclusively in males and usually in. A concise classification system and appropriate treatment options. The tumor is locally aggressive and can invade into surrounding structures and cause significant complications. 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 angiofibroma naf, occurring mostly in young men, is a histologically benign tumor with aggressive clinical behavior that includes repeated epistaxis and intractable nasal obstruction. Juvenile nasal angiofibromas jna is a benign neoplastic lesion encountered in the anterior skull base, and is characterized by significant vascularity and a propensity of bone erosion with intracranial extension. Imaging and management of angiofibroma springerlink. May 16, 2018 patterns of vascularization and surgical morbidity in juvenile nasopharyngeal angiofibroma. Juvenile angiofibroma is a noncancerous growth that causes bleeding in the nose and sinuses. Nasal septal angiofibroma, a subclass of extranasopharyngeal. Typically, the tumor has its onset in childhood, the ages of the patients ranging from seven to nineteen years, although cases in patients up to fifty years of. Early postoperative ct scanning for juvenile nasopharyngeal. The treatment of choice is embolization followed by surgery. To the best of the authors knowledge, this is the first case of angiofibroma with clinical followup after.
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