Volume 1, Issue 2 , Pages 128-131, June 2006
Usefulness of histological evaluation of excised nasopharyngeal tumour in a 12-year-old Nigerian boy mimicking adenoids
Article Outline
Summary
Background/aim: Juvenile nasopharyngeal angiofibroma (JNA) is the commonest benign nasopharyngeal tumour, which tends to bleed is found exclusively in male adolescents. This case was seen at the Federal Medical Centre, Gusau, Nigeria, a relatively new tertiary health institution in North Western Nigeria in May 2005, this is to stress the importance of excision biopsy for tumours rather than incisional biopsy in an environment with limited facilities. Results: This 12-year-old male Nigerian boy presented with nasal obstruction, obstructive sleep apnoea and nasal speech previously treated by the native doctors. The tumour was diagnosed based on clinical, posterior rhinoscopy and plain radiological findings and it was surgically excised via a transpalatal approach and histological evaluation of the excised tumour revealed juvenile angiofibroma. Conclusions: The management is discussed with reference to the literature and the limitation of diagnostic facilities is highlighted in a developing country setting and the need for complete surgical excision rather than incisional biopsy especially in the unwary.
Keywords: Juvenile nasopharyngeal angiofibroma, Male adolescents, Histology, Posterior rhinoscopy, Transpalatal approach
1. Introduction
This histologically benign tumour comprising fibrous with vascular tissues, usually arise from the posterolateral wall of the nasal cavity and superolateral nasopharyngeal wall [1]. It occurs predominantly and perhaps exclusively in young adult males, Hippocrates described the tumour in the fifth century BC, but Friedberg first used the term angiofibroma in 1940. It forms 0.05% of all head and neck tumours [2]. A frequency of 1:5000–1:60,000 in ORL patients have been reported. Juvenile nasopharyngeal angiofibroma occurs exclusively in males; females with the disease should undergo genetic testing. Onset is commonly in the second decade, in the range 7–19 years, rare in patients older than 25 years. Hormond theory has been suggested for its aetiology because it occurs in adolescent males [2]. Pediatric nasopharyngeal tumours are rare, and few clinicians possess more than anecdotal experience [3]. The differential diagnosis includes a diverse group of benign and malignant tumours, but can be narrowed further based on the clinical and radiographic appearance of the mass [3]. Diagnosis of juvenile nasopharyngeal angiofibroma is based on history, physical examinations and radiographic studies [4]. It is the commonest benign tumour of the nasopharynx and the most common presenting symptoms are nasal obstruction and epistaxis, others are hearing loss, rhinorhoea, nasal speech, obstructive sleep apnoea, mouth breathing and headaches [4]. On examination, virtually all patients will have a nasopharyngeal mass, usually pink to purple in colour and nodular. Plain X-rays of the sinuses usually show anterior bowing of the posterior wall of the maxillary sinus “antral bowing” sign (Holmar–Miller sign) in most patients. CT scanning in the main stay of diagnosis, some authors recently have explored the merits of MRI in delineating the margins of tumours and unrevealing vascularity [4]. A high index of suspicion for any nasopharyngeal mass is essential because angiofibroma should not as a rule be biopsed and must not be confused with a large adenoid pad [5]. Juvenile nasopharyngeal angiofibroma is a benign tumour that tends to bleed and occurs in the nasopharynx of prepubertal and adolescent males [5].
Histopathological investigation should only be omitted if there is complete agreement between symptoms, intra-operative findings and macroscopic findings [5]. Histopathology is advised for very large adenoids, alteration of consistency and in older children or young adolescents [5]. Nasopharyngeal angiofibroma has such characteristics angiographic and CT imaging that many authors suggest biopsy is not essential in the evaluation of this lesion [3].
We present a case of a peadiatric nasopharyngeal neoplasm with radiological (plain radiographs), and clinical findings consistent with angiofibroma in a medium sized tertiary health institution in North Western Nigeria with limited diagnostic tools. The management is discussed with reference to the literature stressing the importance of complete excision rather than incisional biopsy of this tumour.
2. Case-report
AW, is a 12-year-old male primary school pupil that presented at the Ear, Nose and Throat Clinic, Federal Medical Center, Gusau, Nigeria, May 2005 with a 5 year history of snoring obstructive sleep apnoea, nasal obstruction mouth breathing nasal speech and rhinorrhoea.
His complaints dated back to 2 years ago when the above symptoms started and he was treated for asthma and chronic catarrh by the traditional doctors and GPS without satisfactory improvement. Examination revealed a male child, small for age, afebrile, anicteric, not pale with submandibular and submental adenopathy. It and rhinolalia aperta. It main findings were in the nose/nasopharynx which showed reduced nasal patency bilaterally, pinkish globular mass in the nasopharynx via posterior rhinoscopy. The ears, throat and other systems were grossly normal. The general investigations that included full blood count, clotting profile and electrolyte/urea were within normal limits. Plan radiographs of the paranasal sinuses and nasopharynx showed enlarged inferior turbinates, antral and nasopharyngeal opacity with narrowing of the nasopharyngeal air-column (Fig. 1, Fig. 2). A request for CT-scan of the paranasal sinuses, the facility that is available in another referral centre 120
km away could not be done due to financial constraints by the parents. Blood was taken for grouping and three units of blood was crossmatched and he was scheduled for examination under anaesthesia of the nasopharynx under general aneasthesia via oral endotracheal intubations with excision of the tumour. Posterior rhinoscopy showed a nasopharyngeal polypoidal mass obstructing the choanae bilaterally on the posterior superior margin of the nasal septum. Transoral digital palpation revealed a firm globular mass 3 by 3cm firm and attached to the posterior end of the nasal septum (Fig. 3). There was no contact bleeding.
Fig. 1.
Plain radiographs of postnasal space (lateral view).
Fig. 2.
Plain radiographs of paranasal sinuses (occipito frontal view).
Fig. 3.
Excised nasopharyngeal tumour (juvenile angiofibroma).
The tumour was completely excised via a transpalatal approach with exposure of the oropharynx with Boyle–Davis mouth gag with a tongue plate. Estimated blood loss was 150ml. The postoperative condition was uneventful and he was discharged home sixth day postoperatively. Histology of the mass revealed angiofibroma. The presenting symptoms had disappeared and outpatient visits had largely being uneventful.
3. Discussion
Juvenile nasopharyngeal angiofibroma (JNA) is a rare benign but aggressive tumour of the nasopharynx which occurs primarily in male adolescents and the usual presentation is either nasal obstruction or epistaxis [6]. Nasal obstruction (80–90%) is the most frequency symptoms in initial stages. Epistaxis (45–60%), unilateral mostly, severe headaches (25%) with snoring. Others are rhinorhoea, rhinolalia, with deafness [7]. The Signs are nasal/nasopharyngeal mass (80%), orbital (15%), proptosis (10–15%) others features of eustachian tube obstruction at otoscopic [7]. This case is a male child within the age bracket and the commonest feature was nasal obstruction that necessitated persistent mouth opening with prominent incisors and high arched palate as found in adenoids. There was no mention of epistaxis in this case probably due to tumour being at the early stages or possibly due to reduced proportion of vascular tissues in relation to fibrous tissues. The main nasopharyngeal findings of a polypoidal mass was achieved via posterior rhinoscopy due to lack of flexible fibreoptic nasopharyngoscopes, despite this inadequacy, it was quite useful in predicting the possible surgical technique(s) to be used. The plain radiographs showed opacification of the nasopharynx and the paranasal sinuses, CT-scan which is the main stay of diagnosis could not be done, due to limited resources on the part of the parents who largely bear the cost of the treatment as the National Health Insurance Scheme is not yet fully operational in Nigeria, although some nearby tertiary health institutions have these diagnostics facilities which are not easily affordable by the patients whose average monthly income is less than $50 [8]. The therapeutic modalities of treatment of juvenile nasopharyngeal angiofibroma over the years have been surgery and radiotherapy, several adjunctive measures of embolization, hormonal therapy and chemotherapy [4]. The main concern of radiotherapy delivered by external beam is malignancies in the irradiated field and the danger of inhibiting normal facial growths in your patients. Most authors prefer surgical reaction in all patients with extracranial diseases and reserve radiation for unresectable intracranial tumours [4]. The surgical technique include lateralrhinotomy,transpalatal, transmaxillary for small (fisch stage I and II), infratemporal for large tumours, midfacial degloving approach and newly introduced intranasal endoscopic laser assisted removal has recently been used [2]. Walderman et al. at the Cleveland clinic USA, showed in their series that adequate surgical approach greatly minimized complications with no recurrence in 10 patients seen over a 3 year period and that radiotherapy should be reserved for cases of intracranial extensions [9]. This tumour was excised surgically via a transpalatal approach, which afforded an adequate exposure. It is striking that the intraoperative blood loss was quite low considering the fact that it is a very vascular tumour; the reason for this could not be easily ascertained. Malignant transformation are rare, in a patient followed up over 20 years after four excisions and two courses of radiotherapy had sarcomatous change, radiation is believed to have played a role. Hence confirmation through histopathology is compulsory [10]. With the availability of surgical procedures that can completely and safely resect these tumours, radiotherapy can be avoided [11]. It is important that complete excision biopsy is done rather partial removal or incisional biopsy and excised tissues should as a rule be sent for histological evaluation and not be discarded as the management of adenoids and juvenile angiofibroma are completely different.
We believe this report reaffirms the use of histological study whenever possible, in addition to radiographic imaging, in the diagnosis of pediatric nasopharyngeal tumours.
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PII: S1871-4048(06)00035-9
doi:10.1016/j.pedex.2006.02.001
© 2006 Elsevier Ireland Ltd. All rights reserved.
Volume 1, Issue 2 , Pages 128-131, June 2006
