Volume 1, Issue 2 , Pages 132-134, June 2006
Extranasopharyngeal angiofibroma of the epiglottis in an 8-year-old girl
Article Outline
Summary
Nasopharyngeal angiofibroma (NA) is a benign tumour which occurs typically in adolescent males. It originates from the nasopharynx and usually extends to the adjacent areas: nose, paranasal sinuses, infratemporal fossa, cranium. Compared to nasopharyngeal angiofibromas primary extranasopharyngeal angiofibromas are very rare. We present a unique case of angiofibroma of the epiglottis in an 8-year-old girl. The tumour was successfully removed by endoscopic CO2 laser surgery under general anesthesia. The diagnosis was histopathologically confirmed. In 1 year follow-up the patient is free of disease.
Keywords: Extranasopharyngeal angiofibroma, Larynx
1. Introduction
Nasopharyngeal angiofibroma (NA) accounts for 0.05% of all head and neck tumours and is the most common benign neoplasm of the nasopharynx [4]. Although histologically benign it shows locally aggressive growth with bone destruction, extension to adjacent areas and possible fatal complications such as intracranial invasion or haemorrhage. The pathogenesis of the NA is still unclear. It occurs almost exclusively in young males, mean age 15–16 years according to different authors [4], [8], [13]. The presentation of NA in females and other age groups has been reported, but remains controversial.
Head and neck angiofibromas arising from outside the nasopharynx are rare. These tumours present different clinical features from the nasopharyngeal angiofibromas, as they more often occur in adults and in females. We report a very rare case of the laryngeal angiofibroma originating from the epiglottis. To our knowledge this is a first described case of angiofibroma arising in such location.
2. Case report
An 8-year-old girl was referred to our institution in June 2004 with a 4-week history of mild dysphagia. Examination revealed a smooth, round, reddish lesion, which was located on the top of the epiglottis and partially obscured the entrance to the larynx. The tumour was covered with mucosa and showed some mobility during breathing and phonation. No other pathological findings were detected.
Endoscopic CO2 laser surgery was performed under general anesthesia. The tumour was excised completely with a small rim of epiglottic cartilage and normal mucosa. There was no major bleeding during the operation. The tumour had 1.5
cm in diameter and had a wide stalk, which was attached to the edge of the epiglottis.
Histopathological examination revealed a benign angiofibroma with histological composition reminding cellular angiofibroma of vulva or pelviperineal region. The tumour was composed of uniform spindle cells with indistinct cytoplasm and wavy nuclei arranged in short fascicles with wispy collagen bundles, and numerous small, often thick-walled vessels (Fig. 1).
Fig. 1.
Microscopically the tumour is composed of uniform spindle cells, collagen bundles and numerous small vessels (H&E, magnification ×400).
The postoperative course was uneventful and the patient left the hospital 4 days after the operation. During 12 months follow-up the patient remains tumour-free.
3. Discussion
Benign neoplasms of the larynx are rare compared to malignant tumours of this origin and benign vocal fold mucosal disorders, like polyps or nodules. Benign vascular neoplasms involving the larynx account for less than 1% of all laryngeal neoplasms. Most common vascular tumours of the larynx are polypoid granulation tissue and hemangiomas [2], [3]. Other vascular lesions that may be seen in adult larynx include hemangiopericytoma, paraganglioma and angiofibroma [10].
Angiofibromas typically occur in the nasopharynx [4]. In this location they show a number of characteristic features including strong sex and age correlation, clinical symptoms, histopathological structure and radiological findings. Laryngeal angiofibroma is a very rare finding. Windfuhr and Remmert in a review of 65 cases of the extranasopharyngeal angiofibromas (ENA) found only four tumours in this location [12]. The angiofibroma of the epiglottis to our knowledge has not been reported.
Clinical manifestation of laryngeal lesions include hoarsness, dysphagia, dyspnea, stridor [7]. Angiofibroma of the larynx shows no significant clinical features and may mimic other pathologies. Unlike NAs, angiofibromas of extranasopharyngeal origin occur in females. They also develop more frequently at older ages. The mean age of tumour diagnosis in extranasopharyngeal angiofibromas is 22 years, comparing to 17 years in NAs [5]. In the review of the literature only 8 of 65 extranasopharyngeal angiofibromas were observed in the first decade of life [1]. Therefore, with regard to tumour location and the patient's age this case is uncommon.
Radiological imaging is a valuable diagnostic tool in the preoperative evaluation of extranasopharyngeal angiofibromas. On CT and MRI tumours show marked contrast enhancement [5], [10], [11]. Signs of expansion and bony erosion may also be present [1], [11]. Selective arteriography confirms the diagnosis of vascular tumour, defines its blood supply, vascular composition and venous drainage. An intensive, inhomogenous blush is typical for angiofibroma. Arteriography allows preoperative embolization, which is an accepted method of reducing intraoperative bleeding [8], [9]. This method has been successfully used in the management of patients with extranasopharyngeal angiofibromas [1], [6], [10]. In one of the reported cases of laryngeal angiofibroma the patient underwent angiography, which revealed tumour blood supply from the superior laryngeal artery [10]. In our case the tumour was small and well visible in clinical examination. Due to tumour size, location and easy surgical access preoperative radiological imaging of the lesion was not necessary. Taking into account the age of the patient we considered that exposition of the child to X-rays or invasive diagnostic procedures was not justified by little additional practical information that could be added. Histopathological examination confirmed the diagnosis of angiofibroma.
Angiofibroma of the larynx is an extremely rare finding. However, one should remember that extranasopharyngeal angiofibromas may occur in young children in various locations.
References
- . Juvenile angiofibroma: report of a case with intraoral presentation. Oral Surg. Oral Med. Oral Pathol. Oral Radiol. Endod. 2002;94:228–232
- . Benign mucosal and saccular disorders; benign laryngeal tumours. In: Cummings CW editors. Otolaryngology—Head and Neck Surgery. St. Louis: Mosby Year Book; 1993;p. 1897–1924
- . Tumours of the Head and Neck, Clinical and Pathological Considerations. second ed.. Baltimore: Wiliams and Wilkins; 1979;
- . Angiofibroma: treatment trends in 150 patients during 40 years. Laryngoscope. 1986;96:1321–1329
- . Extranasopharyngeal angiofibroma arising from the nasal septum. Int. J. Pediatr. Otorhinolaryngol. 2001;58:163–166
- . Extranasopharyngeal angiofibroma. Int. J. Pediatr. Otorhinolaryngol. 2000;56:59–64
- . Superselective embolization in two cases of laryngeal paraganglioma. Otolaryngol. Head Neck Surg. 1995;113:126–130
- Analysis of intr-operative leeding and recurrence of juvenile nasopharyngeal angiofibroma. Clin. Otolaryngol. 2002;27:536–540
- . Diagnosis and treatment of the juvenile nasopharyngeal angiofibroma. Eur. Arch. Otorhinolaryngol. 2001;258:120–124
- . Angiofibroma of the larynx: report of a case with clinical and pathologic literature review. Head Neck. 2002;24:805–809
- . Juvenile angiofibroma limited to the sphenoid sinus. J. Otolaryngol. 1998;1:37–39
- . Extranasopharyngeal angiofibroma: ethiology, incidence and management. Acta Otolaryngol. 2004;124:880–889
- . Nasopharyngeal angiofibroma. J. Otolaryngol. 2002;6:346–350
PII: S1871-4048(06)00039-6
doi:10.1016/j.pedex.2006.03.001
© 2006 Elsevier Ireland Ltd. All rights reserved.
Volume 1, Issue 2 , Pages 132-134, June 2006
