Juvenile nasopharyngeal angiofibroma: spontaneous resolution.
Juvenile nasopharyngeal angiofibroma is a rare, benign tumor that occurs most often in adolescent males. Common practice is to excise the tumor with open or endoscopic surgery. We report the case of a 17-year-old male who presented in 1995 with a mass filling the left posterior nasal cavity. A diagnosis of juvenile nasopharyngeal angiofibroma was obtained with computed tomography and magnetic resonance imaging. The patient elected to have no treatment. On annual scans, the lesion changed little until 1998, when it began to gradually decrease in size. Although it is not well proven, the natural history of these tumors seems to be regression over time. This case supports the argument that a policy of watchful waiting with regular imaging studies may postpone or eliminate the need for surgery and its attendant risks.
Juvenile nasopharyngeal angiofibroma (JNA) is a benign vascular lesion that accounts for 0.5% of all neoplasms of the head and neck. (1) It mainly affects young males; the mean age at diagnosis is 14 years. (2,3) The highest prevalence is in India and Egypt. (4) The most common presenting symptoms are epistaxis and nasal obstruction. (5) The tumor most often arises from the lateral wall or roof of the nasopharynx (3) and receives a blood supply predominantly from the internal maxillary artery, with variable contributions from the external and internal carotid arteries. JNA is diagnosed clinically with computed tomography (CT) or magnetic resonance imaging (MRI).
There is a perception among otolaryngologists that JNA undergoes natural regression, but the literature shows little support for the idea. This lack of evidence is due partly to the lack of sufficiently sophisticated imaging until the advent of CT. Reports predating CT described improvement in symptoms alone. Furthermore, most JNA is treated with excision, embolization, radiotherapy, or a combination thereof, and the effects of both embolization and radiotherapy are well known to continue for many years. (6) Differentiating these effects from natural disease regression can be difficult.
We present a case of spontaneous regression of JNA with no active treatment which, to our knowledge is only the second such case described in the literature.
A 17-year-old male presented in October 1995 with a 6-month history of recurrent left-sided nasal obstruction and repeated minor epistaxis. On examination, a mass was found filling the left posterior nasal cavity. A CT scan was arranged, with a view to excision, and it demonstrated a large, deep facial mass centered on the pterygopalatine fissure, with erosion of the basisphenoid and basioccipital bone. MRI showed a markedly enhancing lesion extending into the pterygomaxillary and infratemporal fossae and nasal cavity through the sphenopalatine foramen (figure, A). A diagnosis of JNA was made.
The patient was offered resection and/or radiotherapy, but after consideration, he elected to have no treatment. Little change in the lesion was seen on annual scanning until 1998. Since then, it has gradually decreased in size (figure, B and C) and now is largely confined to the nasopharynx, with limited extension into the infratemporal fossa.
The literature contains 4 other case reports of regression of JNA demonstrated radiologically, but only 1 of them was untreated and can be considered a true spontaneous regression.
[FIGURE A OMITTED]
In the first case, the patient underwent surgical excision, and on serial CT, residual tumor was found to involute over a 5-year period. (7) In the second case, a 15-year-old boy presented with right cheek swelling and epistaxis. (8) JNA was diagnosed and treated with arterial embolization, followed by two attempts at surgical resection. After recurrence and intracranial progression occurred, a conservative approach was adopted. Serial CT scans showed complete involution over the following 8 years. In the third case, a 13-year-old boy had nasal obstruction and recurrent epistaxis. (9) JNA was proven on biopsy, and he underwent three surgical resections and one arterial embolization. Each time there was local recurrence. Again, a conservative approach was taken and complete resolution was seen on CT 11 years later.
The only case of true spontaneous regression involved an 11-year-old boy with nasal obstruction and frequent episodes of epistaxis. (5) JNA was proven on biopsy. The parents declined surgical intervention, so the patient was followed with serial CT scans, which showed total involution of the lesion.
Surgical resection is the widely recommended treatment for JNA, with the approach based on the location and extent of tumor. Total excision often requires a long procedure, is accompanied by significant blood loss, and can have serious complications, including cranial nerve damage, optic neurovascular bundle injury, and meningitis. Preoperative embolization of the tumor reduces morbidity, recurrence, and intraoperative complications. (10) Endoscopic and endoscopic-assisted surgery have become popular, (11) and several authors advocate this approach for tumors confined to the nasal cavity, sinuses, and pterygopalatine fossa. (12,13) Surgical resection is not always possible for advanced cases (i.e., those with intracranial extension), however.
External-beam radiotherapy is an effective alternative, (14) and tumors usually regress slowly over several months. Complications of radiotherapy can include growth retardation, panhypopituitarism, cataracts, and development of malignant tumors, notably basal cell carcinoma and thyroid carcinoma. (15)
Unfortunately, JNA has a high recurrence rate of up to 40%, (16) usually within 2 years of surgery. (3) Some authors argue that recurrence is unrelated to tumor stage or surgical approach, (17-19) but recurrence rates are reduced through meticulous surgical removal and may be related to residual disease in the basisphenoid bone. (16) Patients presenting at an early age are more likely to have recurrent disease. (20)
With the advent of CT and, especially, MRI scanning, a case can be made for the use of regular imaging and removal or treatment of these benign tumors only if they increase in size or give rise to disabling symptoms. The well-documented problems associated with surgical removal or radiotherapy and the growing evidence that regression is the natural history of these tumors lend support to this argument. Such a policy would, in select cases, avoid treatment and its associated morbidity.
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Patrick M. Spielmann, MRCSEd; Richard Adamson, FRCS; Kenneth Cheng, MRCS; Robert J. Sanderson, FRCS ([dagger])
From the Department of Otolaryngology, Western General Hospital, Edinburgh, Scotland (Mr. Spielmann, Mr. Adamson, and Mr. Sanderson), and the Department of Otolaryngology, Ninewells Hospital, Dundee, Scotland (Dr. Cheng).
Corresponding author: Patrick M. Spielmann, Department of Otolaryngology, Raigmore Hospital, Inverness, Scotland. Phone: 01-463704000; e-mail: firstname.lastname@example.org
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|Title Annotation:||ORIGINAL ARTICLE|
|Author:||Spielmann, Patrick M.; Adamson, Richard; Cheng, Kenneth; Sanderson, Robert J.|
|Publication:||Ear, Nose and Throat Journal|
|Article Type:||Clinical report|
|Date:||Sep 1, 2008|
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