Angiofibroma is a descriptive term for a group of lesions with different clinical presentations but with the same histopathology. May be seen in the context of tuberous sclerosis especially butterfly area of the face. Angiofibroma juvenile nasopharyngeal angiofibroma upmc. Aijcr original article histopathology of nasal masses.
Most of the tumour vessels, which lacked elastic laminae, were characterized by vascular walls of irregular thickness and variable muscle content. Nasopharyngeal angiofibroma, sinonasal tumor, vascular tumor. Tumors limited to nasal cavity, nasopharynx with no bony destruction stage ii tumors invading pterygomaxillary. The histological exam report read hyperplasia of the fig. Cellular angiofibroma and related fibromatous lesions of the vulva. Tumour extension produces rhinosinusal symptoms and facial swelling. Nasopharyngeal angiofibroma of the nasal cavity ncbi. Pathology of cellular angiofibroma dr sampurna roy md. Intricate mixture of stellate and staghorn blood vessels with variable vessel wall thickness ranging from single layer of endothelium to variable smooth muscle coat. May 16, 2018 nasopharyngeal angiofibroma is usually encapsulated and composed of vascular tissue and fibrous stroma with coarse or fine collagen fibers. We documented presenting signs, signalment, radiography, cytology, histopathology, treatment and outcome in all cases.
Case report a 28yearold saudi male presented with 4 years history of recurrent epistaxis and bilateral nasal obstruction. 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. Desmoplastic response of the nasopharyngeal periosteum or embryonic fibrocartilage between the basiocciput and the basisphenoid. Nasopharyngeal angiofibroma an overview sciencedirect. 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. In previous years, when trans palatal palpation for an adenoidal or nasopharyngeal mass was advocated, it was noted as being firm in consistency but current practitioners have had no occasion to put this to test. 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. Cellular angiofibroma is a recently described histologically distinctive benign mesenchymal neoplasm composed of two main components, the cellular spindle cell component and prominent stromal blood vessels local excision with negative margins appears to be adequate treatment for these lesions.
Juvenile nasopharyngeal angiofibroma radiology reference. Jun 24, 2019 endoscopic surgery of nasopharyngeal angiofibroma. Stanford medicine school of medicine departments surgical pathology criteria cellular angiofibroma navigation for this section. Juvenile angiofibroma is a rare benign lesion originating from the pterygopalatine. Adolescents and young adults between 14 and 25 years are affected, and there is a distinct male predominance. Juvenile nasopharyngeal angiofibroma, histopathology embolization, treatment, radiosurgery. 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. A 52 year old woman was seen in the outpatient department for a cyst on her vulva. Juvenile nasopharyngeal angiofibroma iowa head and neck. It most commonly affects adolescent males because it is a hormonesensitive tumor. When confronted with a unilateral nasal mass in a pediatric patient, aside from the usual considerations, embryologic anomalies particularly those of the intracranial protrusion.
Juvenile nasopharyngeal angiofibroma jna is a benign neoplasm of the nasopharynx. Given the strict epidemiology, hormonal influences seem likely as investigators have revealed the presence of androgen, testosterone, and dihydrotestosterone receptors 3, 4 and lack. S100 stain will highlight the melanocytes, and will be negative in fibrous papules. Classically adolescent males with recurrent nose bleeds. Age and sex distribution angiofibroma of skin is a relatively common tumor that is observed typically in middleaged adults. Given the strict epidemiology, hormonal influences seem likely as investigators have revealed the presence of androgen, testosterone, and. Angiofibromas may be approached directly using the endoscopic endonasal approach eea. Polypoidal lesions nasal cavity, histopathology rhinosporidiosis, rhinoscleroma, inverted papilloma, angiofibroma, neurilemmoma, squamous cell carcinoma.
If you have any concerns with your skin or its treatment, see a dermatologist for advice. Soepronos textbook is available on and includes detailed information on over 600 entities and includes four dvd diskettes with highresolution images that provide a virtual dermatopathology reference and guide. However, a wide age range of individuals may be affected. It is impossible to determine clinically what pathology lies underneath. 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. Ultrastructure of nasopharyngeal angiofibromas wiley online library. Angiofibroma is a pink, firm, 1 to 2mm domeshaped papule most commonly located over the nasal bridge and cheeks fig. A well circumscribed lesion but is usually not encapsulated. The jna juvenile nasopharyngeal angiofibroma is a rare neoplasm, of benign histology, that affects almost exclusively male sex adolescent. Nasopharyngeal angiofibroma evaluation and management 44 p j m h s vol. Nasopharyngeal angiofibroma lester dr thompson, md.
These lesions include fibrous papule, facial angiofibroma, pearly penile papule, adenoma sebaceum, periungual fibroma, and koenens tumor. Forces institute of pathology no female patients were. This is a cellular tumour and is composed of small spindle cells arranged in short fascicles together with wispy collagen bundles and relatively abundant small or mediumsized rounded vessels. Nasopharyngeal angiofibroma ulcerated and demonstrate superficial slough in situations with epistaxis and nasal packing. The tumor cells consist of granular cytoplasm, which may be confused with a granular cell tumor. The tumor contains many blood vessels, spreads within the area in which it started locally invasive, and can cause bone damage. Nasopharyngeal angiofibroma an overview sciencedirect topics. A yearold boy presented with frequent epistaxis and nasal obstruction persisting. Transpalatal excision and final histopathology yielded nasal glial heterotopia.
Nasal cavity, paranasal sinuses, nasopharynx nasopharyngeal angiofibroma. Here you can read posts from all over the web from people who wrote about angiofibroma and nasal polyps, and check the relations between angiofibroma and nasal polyps. Introduction nasal polyps are defined as prolapsed lining of the nasal sinuses. The most common treatment for angiofibroma is surgery. The incidence of juvenile angiofibroma is less than 0. Nasopharyngeal angiofibroma na area rare benign but clinically. Nasopharyngeal angiofibroma is usually encapsulated and composed of vascular tissue and fibrous stroma with coarse or fine collagen fibers. Juvenile nasopharyngeal angiofibroma evaluation and workup return to. Angiofibroma of the nasal cavity in dogs burgess 2011. Jul 15, 2015 juvenile nasopharyngeal angiofibroma jna is a benign neoplasm of the nasopharynx.
A number of childhood conditions are characterized by angiofibromas, but the most common is tuberous sclerosis. A yearold boy presented with frequent epistaxis and. Hence the name juvenile nasopharyngeal angiofibroma. Nasal polyps histopathologic spectrum springerlink. If nasopharyngeal angiofibroma is suspected based on physical examination a smooth vascular submucosal mass in the posterior nasal cavity of an adolescent maleimaging studies such as ct or mri should be performed.
The first soft tissue tumour with a relatively pelvic sitespecificity was a benign stromal polyp described in. 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. Differential diagnosis cellular angiofibroma surgical. Dermnet nz does not provide an online consultation service. It usually presents with a triad of unilateral nasal obstruction, episodes of spontaneous nasal bleeding and nasal nasopharyngeal mass. Pdf nasopharyngeal angiofibroma of the nasal cavity. Juvenile angiofibroma rads see also with virtual slide. Analysis of 345 polypoidal masses in nose and nasal sinuses with clinical diagnosis of nasal polyp, observed in 10 years, revealed 175 50. Pdf on may 1, 2010, s marc stokes and others published nasopharyngeal angiofibroma of the nasal.
Juvenile nasopharyngeal angiofibroma jna jnas are wellvascularized benign lesions typically occurring in adolescent males. Sclerosing naevus these usually do not exhibit vascular ectasia. Review article nasopharyngeal angiofibroma ashutosh hota. Department of histology, qml pathology, murarrie, brisbane, department of. Juvenile nasopharyngeal angiofibroma also known as nasopharyngeal fibroma is a distinctive tumor that is associated with nasal obstruction, drainage, and epistaxis in adolescent males. Cellular angiofibroma ca belongs to soft tissue tumours that predominantly occur in the distal genital tract of both genders. Soeprono teaches and practices dermatopathology at loma linda university, school of medicine, department of dermatology. Nasopharyngeal angiofibromas from one female and seven male adolescents. The nasal pack was removed within three days of the intervention, and the nasal inspection revealed normal postoperative healing. The juvenile nasopharyngeal angiofibroma is a benign neoplasm of the posterior. Proliferating cells ki67 reactive were restricted to endothelial cells.
They are well circumscribed and comprise spindle cells with morphol. Histologically, angiofibromas are characterized by fibrosis of the dermis and blood vessels. May, 2010 in the current case, visualization of an axial t2 weighted magnetic resonance image demonstrated an intermediate to high signal, wellcircumscribed mass noted in the right nasal cavity and chonca at the level of the condyles which extended from the posterior nasal cavity to the pharyngeal region. They are essentially rounded projections of edematous membrane 1. Juvenile nasopharyngeal angiofibroma etiology, pathology. Generally, an angiofibroma presents as 1 to 5 mm skincolored to erythematous domeshape papule on the. 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. Juvenile nasopharyngeal angiofibroma electronic journal of. Nasopharyngeal angiofibroma is dealt with separately. Schwannoma of the sinonasal tract clinically presented as. Juvenile nasopharyngeal angiofibroma jna is a rare tumour occurring.
Angiofibroma is a common tumor of the nasopharynx region but cellular type is extremely rare in head and neck. The first description of this tumour dates from nucci et al. Original article aijcr introduction various pathologies ranging from benign lesions to malignant nasal tumor may mimic a simple nasal mass. This stateoftheart, minimally invasive approach allows surgeons to access the tumor through the natural corridor of the nose, without making an open incision. A clinical, histopathological and immunohistochemical study of 42 cases with emphasis on stromal features. It is an aggressive neoplasm and shows a propensity for destructive.
Juvenile nasopharyngeal angiofibroma 420 introduction juvenile nasopharyngeal angiofibroma jna represents 0. Thompson, in head and neck pathology second edition, 20. Juvenile nasopharyngeal angiofibroma jna is a rare benign tumor arising predominantly in the nasopharynx of adolescent males. Nasopharyngeal angiofibroma naf is a highly vascular, histologically benign. Histopathological study of polypoidal lesions of the nasal. It usually presents with a triad of unilateral nasal obstruction, episodes of spontaneous nasal bleeding and nasalnasopharyngeal mass. Plain radiographs no longer play a role in the workup of a suspected juvenile nasopharyngeal angiofibroma, however, they may still be obtained in some instances during the assessment of nasal obstruction, or symptoms of sinus obstructions.
The lesion was removed some weeks later as a day procedure case and the specimen was sent to pathology. Jul 31, 20 dermatopathology reference describes angiofibroma histopathology including histologic features and provides links to additional medical references. Among the non neoplastic lesions, there were 110 cases 62. The bleeding was spontaneous, moderate in quantity, 56 attacks per year.
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