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Frequent epistaxis or blood-tinged nasal discharge Nasal obstruction and rhinorrhea Conductive hearing loss from eustachian-tube obstruction Diplopia, which occurs secondary to erosion into the cranial cavity and pressure on the optic chiasm Rarely anosmia, recurrent otitis media, and eye pain
Signs
Visible firm grayish-red mass in the posterior nasal pharynx; nonencapsulated and often lobulated; can be sessile or pedunculated Proptosis, a bulging palate, an intraoral buccal mucosa mass, cheek mass, or a swelling over the zygoma (common with local extension)
Differential diagnosis
Pyogenic granuloma Choanal polyp Angiomatous polyp Nasopharyngeal cyst Chordoma Carcinoma
Lab Studies:
Perform a CBC count for baseline hemoglobin and hematocrit and platelet count. Assess prothrombin time, activated partial thromboplastin time, and International Normalized Ratio to rule out any unforeseen coagulation problems. Patients with JNA are not associated with any higher risk than is found in the general population.
Imaging Studies:
Sinus films
o
Classic presentations show antral bowing (Holman-Miller sign) as the tumor pushes forward on the posterior wall of the maxillary sinus. Widening of the pterygomaxillary and the superior orbital fissures may be evident. Findings may show nasal septal deviation.
CT scan of the head and face with contrast in both axial and coronal planes is confirmatory and demonstrates the extent of the tumor. MRI may aid in better defining the extent of tumor, avoiding radiation exposure to the patient, and providing multiplanar images and superior 3-dimensional reconstruction capability. Bilateral external and internal carotid angiography
This study adds visualization of the feeder vessel, most commonly branches of the internal maxillary artery, and a means of preoperative embolization. Bilateral angiograms should be obtained because other feeder vessels can contribute significantly to larger tumors. The results improve preoperative planning, but the main use of the study is in preoperative embolization. It can be used to differentiate recurrent tumor from scar.
Diagnostic Procedures:
CT scan, MRI, and angiography are sufficient. Nasal mucosal biopsy, although advocated through the mid 1980s, should be avoided because CT scan and MRI allow diagnostic imaging and prevent resultant uncontrolled hemorrhage and the possibility of misleading histology from a superficial biopsy.
Histologic Findings: Findings include mature fibrous tissue containing variable amounts of thin-walled vessels. These vessels are lined with endothelium, but they lack the normal contractile muscular elements in their vessel walls. This may explain their propensity to bleed. Staging: Used for prognosis and therapeutic approaches, it was proposed by multiple surgeons. It is based on CT scan findings.
Johns, 1980 - Not widely accepted Chandler et al, 1984 - Based on staging of nasopharyngeal cancer Sessions et al, 1981 - Revised by Radkowski et al in 1996 Fisch, 1983 - Revised by Andrews et al in 1989
I - Tumor limited to the nasal cavity II - Into pterygopalatine fossa or maxillary, sphenoid, or ethmoid sinuses IIIa - Into orbital or infratemporal fossa without intracranial involvement IIIb - Stage IIIa with extradural intracranial (parasellar) involvement IVa - Intradural without cavernous sinus, pituitary, or optic chiasm involvement
Note: Surgery usually is recommended for stages I-IVa, and radiation, with or without surgery, is recommended for stage IVb JNAs. http://216.109.117.135/search/cache?p=angiofibroma&ei=UTF8&cop=mss&toggle=1&u=www.emedicine.com/med/topic2758.htm&w=angiofibroma&d=557 E5A9733&icp=1&.intl=us
Angiofibroma
Etiology Unknown Increases in size under the influence of testosterone Pathogenesis Unknown, Epidemiology Predilection for teenage boys (peak incidence at age 15) Rarely ever seen in girls, if at all General Gross Description Firm, fibrous masses Well circumscribed General Microscopic Description Numerous vascular spaces embedded in stroma which is collagenized Spaces generally lined by single layer of endothelium Clinical Correlation Present with nasal obstruction and bleeding, as arise from lateral nasopharynx May bleed profusely with surgery, which is treatment of choice May need chemotherapy if surgery cannot remove intracranial extension References Sternberg SS ed. Diagnostic Surgical Pathology, 2d edition, Lippincott-Raven; Philadelphia. 1996, pp. 874-5.
Angiofibroma
"juvenile nasopharyngeal angiofibroma"
benign vascular nasopharyngeal tumor adolescent males supplied by pterygopalatine br. of maxillary artery may extend into skull base, orbit, cranium nasal cavity --> sphenopalatine canal --> pterygopalatine fossa
findings:
DDx:
http://216.109.117.135/search/cache?p=angiofibroma&ei=UTF8&cop=mss&toggle=1&u=chorus.rad.mcw.edu/doc/00021.html&w=angiofibroma&d=83B19C F243&icp=1&.intl=us
Definition
Return to top
Juvenile angiofibroma is a benign growth of the back of the nose or upper throat (nasopharynx) that contains many blood vessels. Causes, incidence, and risk factors Return to top
Juvenile angiofibroma is relatively uncommon. It typically is discovered in adolescent boys and is associated with nasal obstruction a visible nasal mass and nosebleeds (epistaxis). The tumor is locally invasive and capable of eroding bone. Symptoms Return to top
nosebleeds (epistaxis) o frequent o repeated nasal congestion, difficulty breathing through the nose nasal discharge, usually blood tinged hearing loss prolonged bleeding, bruising easily -- episodes are frequent or occur repeatedly (recurrent) Return to top
An X-ray or a CT scan of the head (nose area) confirms the presence of angiofibroma. Nasal mucosal biopsy indicates tumor or polyp.
Treatment Return to top Treatment is required if the angiofibroma is enlarging, obstructing the airway, or causing chronic nosebleeds. In some cases, no treatment is necessary. Surgical treatment includes removal of the tumor. Removal is often difficult because the tumor is unencapsulated and may be deeply invasive. Recurrence of the tumor after surgical resection is common. Clotting of the vessels of the tumor (embolization) may cause the tumor to scar and stop bleeding. Embolization is performed by injecting a substance into the blood vessel to block blood flow through it. Embolization may be adequate by itself to correct the nosebleeds, or it may be followed by surgery to remove the tumor. Expectations (prognosis) Return to top
Although not cancerous, angiofibromas may continue to expand. They may disappear spontaneously. There is a high rate of recurrence after surgical removal. Complications
Return to top
expansion of the angiofibroma to the nose, the sinuses, and other structures anemia from chronic blood loss pressure on the brain from expansion of the angiofibroma (rare)
Calling your health care provider Return to top Call your health care provider if repeated or frequent nosebleeds occur. Prevention Return to top
There is no known way to prevent this condition. Update Date: 10/30/2003 Updated by: Philip L. Graham III, M.D., M.S., F.A.A.P., Department of Pediatrics, Children's Hospital of New York, Columbia University, New York, NY. Review provided by VeriMed Healthcare Network . http://216.109.117.135/search/cache?p=angiofibroma&ei=UTF8&cop=mss&toggle=1&u=www.nlm.nih.gov/medlineplus/ency/article/001572.htm&w=angiofi broma&d=79D53F6CE8&icp=1&.intl=us
Juvenile angiofibroma
Nasal mucosa
The mucosa, or mucous membrane, is a type of tissue that lines the nasal cavity. Mucous membranes are usually moist tissues that are bathed by secretions such as in the nose.
Review Date: 12/6/2001 12:00:00 AM Reviewed By: A.D.A.M. Medical Illustration Team
Definition: Juvenile angiofibroma is a benign growth of the back of the nose or upper throat (nasopharynx) that contains many blood vessels. Alternative Names: Nasal tumor; Angiofibroma - juvenile; Benign nasal tumor Review Date: 10/30/2003 Reviewed By: Philip L. Graham III, M.D., M.S., F.A.A.P., Department of Pediatrics, Children's Hospital of New York, Columbia University, New York, NY. Review provided by VeriMed Healthcare Network. http://216.109.117.135/search/cache?p=angiofibroma&ei=UTF8&cop=mss&toggle=1&xargs=0&pstart=1&b=11&u=www.pennhealth.com/ency/article/0015 72.htm&w=angiofibroma&d=EF257E31DB&icp=1&.intl=us
Juvenile angiofibroma
Alternative names:
nasal tumor; angiofibroma; angiofibroma - juvenile; benign nasal tumor
Definition:
A noncancerous growth of the back of the nose or upper throat (nasopharynx) that contains many blood vessels.
Prevention:
There is no known way to prevent this condition.
Symptoms:
nosebleeds (epistaxis) o frequent, o repeated nasal congestion, difficulty breathing through the nose nasal discharge, usually blood tinged hearing loss prolonged bleeding, bruising easily--episodes are frequent or occur repeatedly (recurrent)
An X-ray or a CT scan of the head (nose area) confirms the presence of angiofibroma. Nasal mucosal biopsy indicates tumor or polyp.
Treatment:
Treatment is required if the angiofibroma is enlarging, obstructing the airway, or causing chronic nosebleeds. In some cases, no treatment is necessary. Surgical treatment includes removal of the tumor. Clotting of the vessels of the tumor (embolization) may cause the tumor to scar and stop bleeding. Embolization is performed by injecting a substance into the blood vessel to block blood flow through it. Embolization may be adequate by itself to correct the nosebleeds, or it may be followed by surgery to remove the tumor.
Expectations (prognosis):
Although not cancerous, angiofibromas may continue to expand. They may disappear spontaneously.
Complications:
expansion of the angiofibroma to the nose, the sinuses, and other structures anemia from chronic blood loss pressure on the brain from expansion of the angiofibroma (rare)
http://216.109.117.135/search/cache?p=angiofibroma&ei=UTF8&cop=mss&toggle=1&xargs=0&pstart=1&b=11&u=www.healthcentral.com/mhc/top/001572 .cfm&w=angiofibroma&d=866D8E804E&icp=1&.intl=us
nosebleeds (epistaxis) o frequent o repeated nasal congestion, difficulty breathing through the nose nasal discharge, usually blood tinged hearing loss prolonged bleeding, bruising easily -- episodes are frequent or occur repeatedly (recurrent)
An X-ray or a CT scan of the head (nose area) confirms the presence of angiofibroma. Nasal mucosal biopsy indicates tumor or polyp.
Treatment: Treatment is required if the angiofibroma is enlarging, obstructing the airway, or causing chronic nosebleeds. In some cases, no treatment is necessary.
Surgical treatment includes removal of the tumor. Removal is often difficult because the tumor is unencapsulated and may be deeply invasive. Recurrence of the tumor after surgical resection is common. Clotting of the vessels of the tumor (embolization) may cause the tumor to scar and stop bleeding. Embolization is performed by injecting a substance into the blood vessel to block blood flow through it. Embolization may be adequate by itself to correct the nosebleeds, or it may be followed by surgery to remove the tumor. Prognosis: Although not cancerous, angiofibromas may continue to expand. They may disappear spontaneously. There is a high rate of recurrence after surgical removal. Complications:
expansion of the angiofibroma to the nose, the sinuses, and other structures anemia from chronic blood loss pressure on the brain from expansion of the angiofibroma (rare)
Call Health Care Provider: Call your health care provider if repeated or frequent nosebleeds occur. Disclaimer:
Review Date: 10/30/2003 Reviewed By: Philip L. Graham III, M.D., M.S., F.A.A.P., Department of Pediatrics, Childrens Hospital of New York, Columbia University, New York, NY. Review provided by VeriMed Healthcare Network
http://216.109.117.135/search/cache?p=angiofibroma&ei=UTF8&cop=mss&toggle=1&xargs=0&pstart=1&b=11&u=www.henryfordhealth.org/12662.cfm& w=angiofibroma&d=1AE8E8B808&icp=1&.intl=us
Figure 2: Angiography showing highly vascular Figure 1: Coronal CT showing large lesion fed mainly by internal maxillary artery extracranial angiofibroma with extension into branches. The tumour is embolized prior to pterygomaxillary and infratemporal fossa. surgical excision.
Figure 3: Left lateral rhinotomy and lip split. Medial maxillary osteotomies performed.
Figure 4: Anterior maxillary segment and nasal turbinates removed to expose underlying tumour.
Figure 5: Anterior maxillotomy is extented laterally for access to lateral portion of tumour. Sphenoethmoidectomy performed and Figure 6: Large "dumbell" shaped tumour posterior wall of maxillary sinus is removed removed. for optimum exposure to safely remove tumour from skull base and pterygomaxillary/infratemporal fossa.
Figure 7: Dacrocystorhinostomy and medial canthopexy are performed. Cavity is packed with bismuth-iodoform-paraffin paste impregnated gauze. The medial maxillary Figure 8: The external and intraoral incisions are segment is returned and fixed with interosseous closed in layers. wires.The upper lateral nasal cartilage is sutured to bone to prevent collapse of the nasal valve. http://216.109.117.135/search/cache?p=angiofibroma&ei=UTF8&cop=mss&toggle=1&b=61&u=www.sickkids.on.ca/otolaryngology/angiofib.asp&w=angiof ibroma&d=95CB347537&icp=1&.intl=us
Pathologic Findings: This fibrovascular tumor appears benign and consists of the characteristic irregularly shaped blood vessels with a single layer of rounded endothelial cells within a uniform connective tissue stroma. The vessel walls are thin and do not have either a muscularis layer or elastic fibers. The stroma consists of elongated mature fibroblasts with plump nucleoli and dispersed collagen bundles. Unique to this case of JNA is a comparative abundance of fibrous stroma creating a histologically hypovascular appearance. Additionally, the use of pre-operative embolization causes vascular thromboses. Foreign particles implanted in the thrombus is observed. Visualization of the embolized tumor show areas of infarction and necrosis. Impression: Known As:
Juvenile nasopharyngeal fibroma Juvenile angiofibroma Myxofibroma Fibroids of the nasopharynx Nasopharyngeal angiofibroma
Discussion: JNA is a histologically benign vascular lesion which grows very slowly but can be locally aggressive. These tumors are found in young males between the ages of 5 to 25, with mean age being 15. This is the most common benign tumor of the nasopharynx although incidence is rare (0.05%). These fibrovascular tumors originate from sphenopalatine foramen in the lateral nasopharyngeal wall and commonly extends into and through foramina and fissures to adjoining cavities. Frank bone invasion does not occur due to the slow growth of the tumor, although osseous erosion and remodeling are often seen. Etiology: The etiology of JNA is unknown but it is postulated that there may be a relation to sex hormones. There is evidence of increased androgen receptors of tumors and successful tumor regression after anti-androgen therapy. Anatomic Spread of Tumor: tumor arises from sphenopalatine foramen on lateral nasopharyngeal wall and produces radiologic and clinical signs.
Direction of tumor spread Anatomic location of tumor extension Radiologic Correlation Medial nasal cavity via sphenopalatine foramen Displacement of nasal septum Lateral pterygopalatine fossa Widening of pterygomaxillary fissure infratemporal fossa via pterygomaxillary fissure Posterior via pterygoid (vidian) canal Distortion of roots of pterygoid plates Anterior maxillary sinus and orbit via inferior Antral bowing sign orbital fissure Cranial sphenoid sinus to sella turcica and cavernous sinus involving middle cranial fossa by extradural spread Signs and Symptoms: Signs and symptoms occur due to growth of tumor into the nose, paranasal sinuses, orbit and skull base. Common early symptoms include:
**This clinical triad is practically diagnostic for JNA in males between 5-25 years old Common late symptoms:
facial deformities ("frog face") proptosis cranial nerve palsies visual disturbance impaired hearing
Radiographic Differential Diagnosis: Differential includes other causes of nasal obstruction-- choanal polyp, angiomatous polyp, chordoma, nasopharyngeal carcinoma, rhabdomyosarcoma, nasopharyngeal cyst, pyogenic granuloma, lymphoma Diagnosis: Diagnosis is made by clinical and imaging findings together with gender and age of patient. Histologic exam of resected specimen is confirmatory. Suspected lesions should NOT be biopsied since they are very vascular and may bleed abundantly requiring nasal packing and transfusion. CT and MRI are used most commonly to show the extent of the lesion.
CT: Most important pre-operative test because it is useful for showing the destruction of bony structures and widening of foramen and fissures at the skull base due to spread of tumor. The following signs for JNA may be observed:
Mass in the pterygopalatine fossa Mass in the posterior nasal cavity Erosion of bone behind sphenopalatine foramen at root of medial pterygoid plate Bony remodeling or reshaping around tumor antral bowing sign: seen on axial CT due to anterior remodeling of the posterior wall of the maxillary sinus.
CT can also help recognize invasion of sphenoid and thus determine the aggressiveness of surgery. MRI: Useful to show presence of intracranial extension of the tumor. MRI also helps discern between sinus invasion, obstruction and retention of secretions. The following signs for JNA may be observed:
Intense tumor enhancement is shown after IV gadolinium administration due to vascularity of mass. Signal voids representing hypertrophic tumor vessels.
Angiography: Most frequently employed for preoperative identification of feeding vessels of tumor for embolization but can be used for radiologic confirmation of JNA if necessary. Demonstrates the prominent vascular supply commonly from the branches of the ipsilateral internal maxillary artery (a branch of the external carotid artery). A characteristic finding of JNA on angiogram is a dense homogeneous blushing or staining mass during the capillary phase. Findings suspicious for intracranial extension includes blood supply of tumor from internal carotid artery. Treatment: Stage tumor to determine surgical approach of tumor removal Clinical Staging Stage Extent of Tumor 1 confined to nasopharynx 2 into nasal cavity and / or sphenoid sinus into one or more of: antrum, ethmoid sinus, pterygomaxillary and 3 infratemporal fossae, orbit, cheek into cranial cavity-----presence or absence of intracranial extension is 4 used to divide patients into therapeutic and prognostic groups
Surgical resection with preoperative embolization is the preferred treatment. Complications include excess blood loss up to 5 L and death due to exsanguination . Pre-operative angiography is required to visualize the feeding vessels of the tumor in order to perform preoperative embolization. The use of embolization has been shown to decrease intraoperative hemorrhage and the rate of tumor recurrence. Radiotherapy is reserved for recurrent tumors. Complications include sarcomatous transformation, danger of inhibiting normal facial growth and risk of hemi-facial atrophy. Spontaneous regression is rare Recurrence:
6-60% recurrence rates can occur as early as 3-4 months after surgery main predictor of recurrence is invasion of sphenoid bone. postoperative radiological surveillance is important due to high rates of recurrence
histologically benign but locally aggressive tumor the most common benign nasopharyngeal tumor although incidence is low (0.05%) occurs in males aged 8-25, mean age 15 most common symptoms include recurrent epistaxis and unilateral nasal obstruction NEVER biopsy this lesion imaging has an integral role in the diagnosis, treatment and follow-up surveillance of this tumor treatment includes surgery with pre-operative embolization
Links to other sites containing radiology case studies of Juvenile Nasopharyngeal Angiofibroma: Submitted by: Hannah S. Choi, MS-III http://216.109.117.135/search/cache?p=angiofibroma&ei=UTF8&cop=mss&toggle=1&b=21&u=cats.med.uvm.edu/cats_teachingmod/radiology/radiology_ht ml/teaching/radio_peds/jna/jna_frameset.htm&w=angiofibroma&d=C688D67144&icp=1&.intl =us
Juvenile angiofibroma
For more information...
Definition:
A benign growth of the back of the nose or upper throat (nasopharynx) that contains many blood vessels.
Alternative Names:
Nasal tumor; Angiofibroma - juvenile; Benign nasal tumor
Symptoms:
nosebleeds (epistaxis) o frequent o repeated nasal congestion, difficulty breathing through the nose nasal discharge, usually blood tinged hearing loss prolonged bleeding, bruising easily -- episodes are frequent or occur repeatedly (recurrent)
An X-ray or a CT scan of the head (nose area) confirms the presence of angiofibroma. Nasal mucosal biopsy indicates tumor or polyp.
Treatment:
Treatment is required if the angiofibroma is enlarging, obstructing the airway, or causing chronic</a><a class='library' href="http://www.meijer.com/pharmacy/Medi_cond_display.asp? urn=urn:content:ency:article:003106&qu=nosebleeds">nosebleeds. In some cases, no treatment is necessary. Surgical treatment includes removal of the tumor. Removal is often difficult because the tumor is unencapsulated and may be deeply invasive. Recurrence of the tumor after surgical resection is common.
Clotting of the vessels of the tumor (embolization) may cause the tumor to scar and stop bleeding. Embolization is performed by injecting a substance into the blood vessel to block blood flow through it. Embolization may be adequate by itself to correct the nosebleeds, or it may be followed by surgery to remove the tumor.
Expectations (prognosis):
Although not cancerous, angiofibromas may continue to expand. They may disappear spontaneously. There is a high rate of recurrence after surgical removal.
Complications:
expansion of the angiofibroma to the nose, the sinuses, and other structures anemia from chronic</a><a class='library' href="http://www.meijer.com/pharmacy/Medi_cond_display.asp? urn=urn:content:ency:article:000045&qu=blood loss">blood loss pressure on the brain from expansion of the angiofibroma (rare)
Prevention:
There is no known way to prevent this condition. http://216.109.117.135/search/cache?p=angiofibroma&ei=UTF8&cop=mss&toggle=1&xargs=0&pstart=1&b=71&u=www.meijer.com/pharmacy/Medi_cond _display.asp%3Furn%3Durn%253Acontent%253Aency%253Aarticle%253A001572%26qu %3Dnasal+tumors&w=angiofibroma&d=B220B39F9C&icp=1&.intl=us