Well circumscribed but unencapsulated polypoid fibrous mass, bleeds severely on manipulation and biopsy, may occlude nares. It most commonly affects adolescent males and may grow into fissures of the. The juvenile nasopharyngeal angiofibroma is a benign neoplasm of the posterior. Mri provides additional assessment of the tumors interface with adjacent soft tissue and is particularly valuable in evaluating intracranial and cavernous sinus extension. Juvenile nasopharyngeal angiofibroma jna is a rare tumour occurring. Nasopharyngeal angiofibroma an overview sciencedirect topics.
Ppt journal juvenile nasopharynx angiofibroma radiation. This is a classical presentation of juvenile nasopharyngeal angiofibroma. Juvenile nasopharyngeal angiofibroma evaluation and workup return to. 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. Although it is nonmalignant not cancerous, it can expand quickly and extensively. Given the strict epidemiology, hormonal influences seem likely as investigators have revealed the presence of androgen, testosterone, and dihydrotestosterone receptors 3, 4 and. Though it is a benign tumor, it is locally invasive and can invade the nose, cheek, orbit. Pdf juvenile nasopharyngeal angiofibroma researchgate. The most common presenting symptom is painless nasal obstruction or epistaxis. We present a case of a patient with a juvenile nasopharyngeal angiofibroma on the left side and a combined hamartoma in his left eye. Juvenile nasopharyngeal angiofibroma iowa head and neck. Juvenile nasopharyngeal angiofibroma radiology reference.
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. It most commonly affects adolescent males because it is a hormonesensitive tumor. Intricate mixture of stellate and staghorn blood vessels with variable vessel wall thickness ranging from single layer of endothelium to variable smooth muscle coat. The overlying epidermis is uninvolved but may be atrophic. Juvenile nasopharyngeal angiofibroma jna is a rare benign tumor. 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. The diagnosis of juvenile nasopharyngeal angiofibroma was based on histopathology of the excised tumor, and the diagnosis of the combined hamartoma was based on the typical ophthalmoscopic and fluorescein angiographic appearance of the fundus lesion.
Juvenile nasopharyngeal angiofibroma jna is a benign neoplasm of the nasopharynx. It is a histopathologically benign, yet locally aggressive, vascular tumor. Juvenile nasopharyngeal angiofibroma of jna 1,5,11 figure 2. This is a concise presentation for medical students especially from.
Juvenile nasopharyngeal angiofibroma jna constitutes less than 0. A rare case report of nasopharyngeal mass saleh mohebbi1, mohammad aghajanpour1 1department of rhinology and skull base surgery, ent and head and neck research center, hazrat rasoul hospital, the five senses institute, iran university of medical sciences, tehran, iran. It is almost exclusicely encountered in adolescent males 1. Recently a changing trend has been seen in its epidemiology, pathogenesis, diagnosis, medical management, preoperative care, surgical management and postoperative care including radiotherapy. A concise classification system and appropriate treatment options. Study the changing trend basically in the northeastern region of southeast.
Thompson, in head and neck pathology second edition, 20. Pdf juvenile angiofibroma jna is a relatively uncommon, highly vascular and benign tumor that presents most commonly in adolescent males. From juvenile nasopharyngeal angiofibroma to nasopharyngeal carcinoma. Juvenile nasopharyngeal angiofibroma boston childrens. Gullane pj, davidson j, odwyer t, et ah juvenile angiofibroma. 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.
As the tumor is largely spaceoccupying and highly vascular, patients typically. Juvenile primary extranasopharyngeal angiofibroma, presenting as cheek. Juvenile nasopharyngeal angiofibroma jna is a rare and benign but locally aggressive fibrovascular tumor arising from the posterolateral wall of the. Patterns of vascularization and surgical morbidity in juvenile nasopharyngeal angiofibroma. Anesthetic management of nasopharyngeal angiofibroma. Juvenile nasopharyngeal angiofibroma is an uncommon tumor found in the nasopharynx of the adolescent male. This tumor originates in the lateral wall of the nasal cavity, close to the superior border of the sphenopalatine foramen. Juvenile nasopharyngeal angiofibroma electronic journal of. Juvenile nasopharyngeal angiofibroma makhasana ja, kulkarni ma. Surgical management of juvenile nasopharyngeal angiofibroma. Juvenile nasopharyngeal angiofibroma is a very rare, highly vascular, uncapsulated and locally invasive tumor. In this video, we are going to see about juvenile nasopharyngeal angiofibroma.
Juvenile nasopharyngeal angiofibroma, surgical approach, recurrence, residual disease introduction juvenile nasopharyngeal angiofibroma jna is a relatively rare tumor occurring mainly in adolescent boys. The origin and development of jna is not fully understood. Angiofibromas cause a domeshaped mass in the superficial dermis figure 1. The juvenile nasopharyngeal angiofibroma has a characteristic growth in all directions from its origin. Juvenile nasopharyngeal angiofibroma jna is a benign vascular tumor that appears in the nasal cavity. In 1906 chareau revived the interest in the study of juvenile nasopharyngeal angiofibroma. Juvenile nasopharyngeal angiofibroma etiology, pathology. Juvenile nasopharyngeal angiofibroma wilson 1972 the. Correlating histology, surgical approach and blood loss.
Juvenile nasopharyngeal angiofibroma stages i and ii. 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. A typical clinical and radiological presentation of juvenile. The juvenile nasopharyngeal angiofibroma jna is a highly nasoangiofibroma youth is a highly vascularized tumor almost exclusively male. Fibroangioma, histopathology, juvenile nasopharyngeal angiofibroma, nasopharyngeal neoplasms, surgical approval. Thisis a reportofninecases ofangiofibroma of the nasopharynxencountered at mcpherson hospitaland duke universitymedical center from 1958 to 1963. Given its vascularity, prominent flow voids are seen on mri leading to a salt and pepper appearance. Juvenile nasopharyngeal angiofibroma radiology case. Histopathology reveals a fibrocellular stroma with spindle cells and haphazard arrangement of collagen interspersed with an irregular vascular pattern.
Review of the literature was performed to define the optimal treatment of patients with juvenile nasopharyngeal angiofibroma jna. Preoperative selective arterial embolization has decreased intraoperative blood loss and facilitated resection of larger tumors. Review article nasopharyngeal angiofibroma ashutosh hota. Pamella arteliana elisa soetanto rizky futrai abstract purpose. 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. Department of histology, qml pathology, murarrie, brisbane, department of. Juvenile nasopharyngeal angiofibroma jna is a rare vascular tumour affecting mostly adolescent males, benign by histopathology, but considered ma. Combined hamartoma of the retina and retinal pigment. 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. Pdf juvenile nasopharyngeal angiofibroma jna is a relatively. Jnas originate from the posterior choanal tissues and rapidly extend into the surrounding regions, including the nasopharynx, the orbits, and even the intracranial cavity.
This is a histologically benign lesion with very aggressive local behavior. Adolescents and young adults between 14 and 25 years are affected, and there is a distinct male predominance. Epistaxis nasopharyngeal mass in adolescent males with an average age of onset of 15 years of age. This approach with partial medial maxillectomy can give good exposure of pterygopalatine fossa and easy manipulation of maxillary artery. The typical patient is an adolescent male with a clinical history of recurrent epistaxis and nasal obstruction. Juvenile nasopharyngeal angiofibroma, histopathology embolization, treatment, radiosurgery. Juvenile nasopharyngeal angiofibroma jna is a vascular tumor of the nasopharynx that accounts for 0. 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 lester dr thompson, md. Juvenile primary extranasopharyngeal angiofibroma, presenting as.
The prognosis for this disease is extremely good if diagnosed well in time and if the tumor has not extended intracranially. The nasopharyngeal angiofibroma is an aggressive, vascular tumor that may represent a challenge for the anesthesiologist by the risk of profuse bleeding. Although the use of nonsurgical therapies is described in the literature, surgery is currently considered the ideal treatment for. Atypical clinical and radiological presentation of juvenile nasopharyngeal angiofibroma abdelrahman ezzat, hussein roshdy al azhar university airoegypt astrat juvenile nasopharyngeal angiofibroma jna constitutes less than 0. Juvenile angiofibroma rads see also with virtual slide. Shaheen in 1930 reported the first female patient with juvenile nasopharyngeal. Axial ct, soft tissue and bone windows, at the level of the maxillary sinus show characteristic features of juvenile nasopharyngeal angiofibroma. Juvenile nasopharyngeal angiofibroma knowledge for. Juvenile nasopharyngeal angiofibroma jna is a rare benign tumor arising predominantly in the nasopharynx of adolescent males.
There are several distinct clinical forms of angiofibroma which share identical histopathology fibrous papule. Juvenile nasopharyngeal angiofibroma romanian journal of. This was a retrospective cohort study of all patients with jna with intracranial extension radkowski grade iiia treated with endoscopic, endoscopicassisted, and external surgery from january of to may of consistent expression of estrogen receptor beta. Juvenile nasopharyngeal angiofibroma is a pathologically benign yet locally aggressive and destructive vascular lesion of head and neck. 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. Infact hippocrates goes on to describe a polyp in the nose which weeped blood. 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. Early postoperative ct is reliable in detecting or excluding residual disease in patients with jna 2,24. Juvenile nasopharyngeal angiofibroma jna springerlink.