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| ORIGINAL ARTICLE |
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| Year : 2019 | Volume
: 21
| Issue : 1 | Page : 6-8 |
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Atypical Clinical and Radiological Presentation of Juvenile Nasopharyngeal Angiofibroma; A case report
Ali Al-Zraei1, Abdullah AlSkaini2, Tawfiq Khurayzi3
1 Otorhinolaryngology Consultant, Head of local Committee South Region, Aseer Central Hospital, Aseer, Saudi Arabia
2 Otorhinolaryngology Department at the Armed Forces Hospital Southern Region, KhamisMushauet, Saudi Arabia
3 Otorhinolaryngology Department at King Fahad Central Hospital, Jizan, Saudi Arabia
| Date of Web Publication | 6-Feb-2020 |
Correspondence Address:
Ali Al-Zraei
Aseer Central Hospital, Aseer
Saudi Arabia
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/1319-8491.277843
Juvenile nasopharyngeal angiofibroma (JNA) is a highly aggressive locally invasive vascular neoplasm found almost exclusively in prepubescent and pubescent boys. It accounts for 0.05% of all head and neck tumors and is the most common benign tumor involving the pterygopalatine and infratemporal fossa[1]. Although histologically benign, it is locally invasive and, if left untreated, has the potential to erode through the skull base[2]. Here we present a case report of a 13 years old boy came with history of obstruction in the right nasal cavity associated with epistaxis and discharge. Computed tomography with contrast imaging revealed an enhancing heterogeneous mass lesion in the nasopharynx and posterior choana extending to pterygopalatine and infratemporal fossa in the right side.
Keywords: juvenile nasopharyngeal angiofibroma, pterygopalatine, infratemporal, epstaxis,
How to cite this article:
Al-Zraei A, AlSkaini A, Khurayzi T. Atypical Clinical and Radiological Presentation of Juvenile Nasopharyngeal Angiofibroma; A case report. Saudi J Otorhinolaryngol Head Neck Surg 2019;21:6-8 |
How to cite this URL:
Al-Zraei A, AlSkaini A, Khurayzi T. Atypical Clinical and Radiological Presentation of Juvenile Nasopharyngeal Angiofibroma; A case report. Saudi J Otorhinolaryngol Head Neck Surg [serial online] 2019 [cited 2023 Jun 4];21:6-8. Available from: https://www.sjohns.org/text.asp?2019/21/1/6/277843 |
| Case Report | |  |
In November 2017,A 13 years old boy was seen in our Otolaryngology clinic at Aseer Central Hospital with right nasal obstruction for long time, progressive, intermittent right-sided epistaxis and discharge in the past 2 years. There was no history of allergy or facial headache or other health problems. On clinical examination patient looks well with no active bleeding or left / right symmetry feature changes. Oral cavity examination was also normal. Endoscopic evaluation of the right nostril showed a fragile, lobular, red -colored, smooth with active serous discharge, arising posteriomedialy to the right inferior turbinate [Figure 1].The coagulation profile was found to be within the normal limits.
Computed tomography (CT) scan of the nose and paranasal sinuses showed soft tissue mass lesion showing heterogenous predominantly peripheral contrast enhancement.[Figure 2](A), [Figure 2](B) The patient was admitted to hospital, diagnosed with juvenile nasopharyngeal angiofibroma and prepared to have surgery. Surgical consent, preoperative preparations and investigations appropriate for this type of surgery were token according to agreed protocol. | Figure 2: Axial (A) and coronal (B) Computed tomography (CT) scan of the nose and paranasal sinuses showed soft tissue mass lesion within the posterior aspect of the right nasal cavity; centered upon the right sphenopalatine foramen with mild heterogenous contrast enhancement
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Surgical Procedure
Under general hypotensive anesthesia, nasal packing was done with cotton swabs socked in topical 1:20000 adrenaline to help in local vasoconstriction, after 15 minutes packing removed then infiltration of lateral nasal wall of the right nostril using lidocaine 2% with 1: 100,000 adrenaline. Fine-needle aspiration was carried out showed only blood, also the mass was bleed easily with manipulation. Endoscopic Dissection of the tumor from the surrounding anatomical structures until identification of the pedicle of the tumor which was originated from the sphenopalatine foramen and posterior and lateral wall of nasopharynx. Under constant traction of the second surgeon, Tumor was resected in 4 parts with the aid of Kerrison forceps.
After removal of the tumor [Figure 3], the site of origin was bleeding profusely but was controlled after packing with Gauze socked in topical adrenaline and cauterized with monopolar cautery. | Figure 3: Show Irregular soft tissue pieces measuring 4x2x2 (A), and Histopathological examination of the excised masses (B) composed of mixture of large and small thin-walled blood vessels and fibrous stroma
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Histopathological examination of the excised masses showed regular soft tissue pieces measuring 4x2x2 cm composed of mixture of large and small thin-walled blood vessels and fibrous stroma [Figure 3].
| Discussion | | |
Juvenile nasopharyngeal angiofibroma is an uncommon tumor presenting mostly in adolescent males. The prevalence age differs between 11- 20 years with an average of presentation of this tumor is 14 years[3]. The occurrence in young men is most likely identified with androgen receptors[4]. The tumor starts close to the sphenopalatine foramen area, later it grows in all directions through numerous projection[5]. Patients usually came at late phase of the disease with history of nasal obstruction and intermittent epistaxis and rarely as swelling on face. Extensivegrowth of tumor may cause facial swelling, proptosis, diplopia with disturbance in speech and conductive hearing loss[6]. The patient should be inspected using nasal endoscopy, which generally demonstrates an expansive, lobulated mass behind the middle turbinate filling the choana, smooth- surfaced with signs of hypervascularity.[7]. Diagnostic imaging should be performed before an invasive method since it might propose the finding, in this way decreasing the danger of disastrous hemorrhage related with biopsy. CT with contrast is a most important preoperative test since it is valuable for demonstrating the destruction of bony structures and widening of foramen and fissures at the skull base because of spread of tumor.[8].
Demonstration of the anterior bowing of the maxillary wall due to presence of a mass in the pterygomaxillary space on axial CT slices known as the Holman-Miller sign is a characteristic finding of JNA[9].
Recurrence results because of more expansion of the tumor resulting in residual tissue after surgery. The vascular supply to JNAs is essentially from distal Internal Maxillary Artery (IMA) branches, especially the sphenopalatine, descending palatine and posterior superior alveolar branches. JNAs may also be supplied by the ascending pharyngeal artery. So, preoperative embolization of feeder vessels i.e., super selective embolization of external carotid artery can be done to reduce blood loss without significant neurological complications[10].
Surgery is the mainstay of treatment of JNA. Preoperative embolization and newer surgical approaches result in less hemorrhage and complete resection of the tumor. Aggressive re-resection should be done for resectable tumor to prevent recurrences. Radiotherapy must be reserved for unresectable, recurrent/residual disease[11].
| References | | |
| 1. | Gullane PJ, Davidson J, O’Dwyer T, Forte V. Juvenile angiofibroma: a review of the literature and a case series report. Laryngoscope. 1992;102(8):928-933  |
| 2. | Granato L. Juvenile nasopharyngeal angiofibroma. In: Otolaryn- gology Textbook. Sao Paulo: Roca; 2002: 701-710. |
| 3. | Antonelli AR, Cappiello J, Di Lorenzo D, Donajo CA, Nicolai P, Olandi A Diagnosis, staging, and treatment of juvenile nasopharyngeal angiofibroma(JNA). Laryngoscope. 1987;97:1319-1325. |
| 4. | Brentani MM, Butugan O, Oshima CT, Torloni H, Paiva LJ ; Multiple steroid receptors in nasopharyngeal angiofibroma. Laryngoscope, 1989; 99:398-401 |
| 5. | A. Peric, N. Baletic, S. Cerovic et al., “Middle turbinate angiofibroma in an elderly woman,” Vojnosanitetski Pregled, vol. 66, no. 7, pp. 583-586, 2009. |
| 6. | Maurice M, Milad M. Pathogenesis ofjuvenile nasopharyngeal angiofibroma. Braz J Otorhinolaryngol. 1981; 95:1121-6. |
| 7. | Nicolai P, Schreiber A, Bolzoni Villaret A. Juvenile angiofibroma: Evolution of management. Int J Pediatr. 2012:412545. |
| 8. | Lloyd G, Howard D, Phelps P, et al. Juvenile angiofibroma: The lessons of 20 years of modern imaging. J Laryngol Otol. 1999; 113: 127-134. [PMID: 10396561] |
| 9. | Maurice M, Milad M. Pathogenesis ofjuvenile nasopharyngeal angiofibroma. Braz J Otorhinolaryngol. 1981; 95:1121-6. [PubMed] |
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| 11. | Mistry RC, Qureshi SS, Gupta S, Gupta S. Juvenile nasopharyngeal angiofibroma: A single institutions study. Indian J Cancer. 2005; 42:35-9. |
[Figure 1], [Figure 2], [Figure 3]
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