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NASOPHARYNGEAL ANGIOFIBROMA WITH CAVERNOUS SINUS INVOLVEMENT
 An Unusual Presentation
Dinaz K Irani*, Makarand V Damle**,Bachi T Hathiram***, N L Hiranadani****
*Lecturer; **Senior Resident; ***Associate Professor; ****Hon. Professor, Department of Otorhinolaryngology, TN Medical College and BYL Nair Ch. Hospital, Mumbai 400 008.
INTRODUCTION

Juvenile nasopharyngeal angiofibroma (JNA) is an uncommon benign vascular tumour occurring almost exclusively in pre-pubescent or pubescent males. The triad of epistaxis, nasal obstruction and the presence of a nasopharyngeal mass strongly ind`icates an angiofibroma, especially when seen in an adolescent male. It accounts for less than 0.05% of head and neck tumours (Waldman et al, 1981). Its incidence has been stated to be as low as one in 50,000 otolaryngological new patients.[1] Intracranial extension has been observed in 20-30% patients with JNA.[2,3]

It is a benign tumour and is locally aggressive eroding adjacent bone and growing through natural foramina and fissures thus gaining an easy access into the cranium and the infratemporal region. The intracranial extension is either through erosion of the sphenoid sinus through the sella medial to the carotid artery and lateral to the pituitary gland or via erosion of the greater wing of sphenoid through the middle cranial fossa anterior to the foramen lacerum and lateral to the cavernous sinus and carotid artery.

In this paper we report our experience of an unusual presentation of a huge JNA with intracranial extension into the middle cranial fossa, which encroached the cavernous sinus. We treated it surgically by the conventional combined lateral rhinotomy and transpalatal approaches. The entire tumour including the intracranial extent was removed without opening the cranium.

CASE REPORT

A 17 year old boy was referred to us with chief complaints of right-sided nasal obstruction, repeated episodes of epistaxis and a nasal twang since a period of 1 year. The patient was alright 1 year back when he started experiencing a right-sided nasal obstruction which was gradually increasing. He also complained of repeated episodes of epistaxis, which increased to 10-15 ml of fresh blood daily. The epistaxis was controlled by the patient by lying in the supine position. There was no headache, vomiting, diplopia or any other symptoms suggestive of raised intracranial tension or involvement of the cavernous sinus. The patient occasionally complained of bleeding from the mouth.

On general examination, the patient was anaemic. There was widening of the bridge of the nose. Anterior rhinoscopy showed purulent discharge in the right nasal cavity and a huge reddish lobulated mass seen extending into the oropharynx behind the palate for about 2 cm.

Haematological investigations of the patient were as follows

Haemoglobin 6 gm%, PCV 42 gm%, MVC 65 fl., MCH 25 pg., MCHC 25%, BUN 15 mg%, Serum creatinine 0.5 mg%, Serum Na 133 mEq/L, Serum K 3.5 mEq/L, ESR 9 mm at the end of the Serum Ca 9 mg%.

On radiographic investigations the X-ray chest was normal. The X-ray paranasal sinuses Water’s and Caldwell’s view showed haziness in the right nasal cavity and right maxillary sinus without bony destruction. The X-ray nasopharynx revealed a mass occupying the entire nasal cavity, nasopharynx and upper part of the oropharynx.

The contrast CT scan axial and coronal views revealed a large well defined moderately enhancing nasopharyngeal mass extending into the right nasal cavity with widening of the pterygopalatine fossa laterally, into the sphenoid sinus, right optic canal and middle cranial fossa encroaching on the posterior and medial aspect of the right cavernous sinus superiorly and into the oropharynx inferiorly (Figs 1a,b,c).

The diagnosis of JNA with intracranial extension (Cavernous sinus) was made and the patient was advised surgery - combined lateral rhinotomy with transpalatal approach. Pre-operatively 5 units of blood were transfused to the patient to bring the haemoglobin to 11 gm%; general anaesthesia was given. The right external carotid artery was ligated. A lateral rhinotomy and transpalatal incisions were taken. The lateral rhinotomy incision was extended sublabially. The flaps were elevated and the maxillary antrum was opened and inspected. The medial wall of the maxilla, especially the anterosuperior part was removed partially to expose the tumour which was then inspected and it was gently dissected out by finger dissection and gentle traction from all its anterior and superior attachments including infratemporal, nasopharyngeal and lastly the intracranial part was removed by guarded traction and dissection. Brisk bleeding from the venous plexus of the right cavernous sinus ensued but was controlled with gentle pressure applied over gelatin sponge, reinforced with moistened cottonoids. After removal of the intracranial part the defect in the periosteum and medial wall of the right cavernous sinus was seen.

Fig1a.
Fig.1a Contrast CT scan axial view showing a large well defined moderately enhancing mass involving the right nasal cavity nasopharynx, pterygopalatine fossa and infratemporal fossa

Fig.1b
Fig.1b Contrast CT scan coronal view showing a large well defined moderately enhancing mass involving the nasopharynx,infratemporal fossa and extending into the sphenoid sinus and middle cranial fossa

Fig1c.
Fig.1c Contrast CT scan coronal view showing the enhancing mass involving the right cavernous sinus.

 

No CSF leak was found. No attempt was made to repair the defect. Simultaneously through the transpalatal incision removal of the nasopharyngeal attachments of the tumour was done by finger dissection.

Inspite of ligation of the right external carotid artery, the patient bled profusely and we had to transfuse 3 pints of blood and 2 pints of fresh frozen plasma intraoperatively and 2 pints of blood in the immediate post-operative period. Anterior nasal packing was done and the incisions were sutured. The pack was removed in the operation theatre after 72 hours under general anaesthesia and we encountered no active bleeding.

The gross appearances of the tumour was a round, nodular, nonencapsulated pink mass which measured 8 cm x 4 cm in dimension. Histopathological examination revealed fibrous connective tissue with endothelium lined/spaces confirming the diagnosis of angiofibroma (Figs. 2 and 3).

DISCUSSION

Juvenile nasopharyngeal angiofibroma (JNA) is a locally aggressive benign vascular tumour. It affects mainly adolescent males and the diagnosis is often made at a relatively late stage, after the patients have had symptoms for several months. The typical initial symptoms include nasal obstruction, epistaxis, pain, nasal discharge and hearing impairment.[4]

The diagnosis and assessment of tumour extension are made on the basis of clinical symptoms and radiographic investigations, including computed tomography (CT) and angiography.

A staging system similar to that proposed for cancer by the American Joint Committee has been suggested by the Chandler et al in 1984. According to this classification, in Stage I tumour is confined to the nasopharynx and in Stage II tumour extends into the nasal cavity and/or sphenoid sinus. In Stage III tumour extends into one or more of the followings: maxillary antrum, ethmoid sinus, pterygomaxillary and infratemporal fossae, orbit and/or cheek. Tumour extension intracranially is stage IV.

Treatment modalities include pre-operative arterial embolization[5] and Surgery.[6] In cases with intracranial extension of the tumour which occurs in 10% to 20%7 different modalities of treatment have been tried such as irradiation6 or intracranial surgery.[7]

Tumours invading the cavernous sinus have generally been considered inoperable because of the high likelihood of bleeding from the cavernous venous plexus, or potential injury to the internal carotid artery or one of the cranial nerves.[8] In addition, surgical defects in the dura surrounding the cavernous sinus can be difficult to repair, leading to the possibility of an uncontrollable cerebrospinal fluid leak.[9] Based largely on these concerns, most surgeons addressing the treatment of intracranial angiofibromas have advocated either non-surgical treatment or incomplete resection of tumour involving this structure.[10,11]

The unusual and interesting finding in our case are that inspite of being a large and extensive JNA the patient had no symptoms of intracranial and/or cavernous sinus involvement. Since the tumour is benign, encapsulated and extradural it was dissected out by gentle traction and finger dissection by combined transpalatal and lateral rhinotomy approach.

The advantage of this approach is that it is less morbid than intracranial surgery which involves traction on the cerebrum with risk of anaesthesia and other complications of intracranial surgery. Therefore we recommend it for JNA with intracranial extension in selected cases.

Fig.2
Fig2. Gross appearance of the specimen

Fig.3
Fig.3 Microphotograph showing features of angiofibroma ( H and E ) x 40.

 

ACKNOWLEDGEMENT

We are grateful to Dean, Dr. (Mrs) NA Kshirsagar for allowing us to publish this paper.

REFERENCES

  1. Chandler JR, Goulding R, Moscowitz L, et al. Nasopharyngeal angiofibromas : Staging and Management. Annals of Otology Rhinology Laryngology 1984; 93 : 323-20.
  2. Jafek BW, Krekorian EA, Kirsch WM, Wood RP. Juvenile nasopharyngeal angiofibroma : Management of intracranial extension. Otolaryngology 1979; 2 : 119-28.
  3. Cummings BJ, Blend R, Keane T, et al. Primary radiation therapy for juvenile nasopharyngral angiofibroma. Laryngoscope 1984; 94 : 1599-605.
  4. Jacobsson M, Petruson B, Svendsen P, et al. Juvenile nasopharyngeal angiofibroma : A report of 18 cases. Acta Otolaryngologica 1988; 105 : 132-39.
  5. Bryan RN, Sessions RB, Horowitz BL. Radiographic management of Juvenile angiofibromas. American Journal of Radiology 1981; 2 : 157-66.
  6. Ward PH. The evolving management of Juvenile Nasopharyngeal angiofibroma. Journal of Laryngology Otology 1983; Suppl 8 : 103-4.
  7. Jafek BW, Nahum AM, Butler RM, et al. Surgical treatment of Juvenile Nasopharyngeal Angiofibroma. Laryngoscope 1973; 83 : 707-20.
  8. Sekhar LN, Moller AR. Operative management of tumours involving the cavernous sinus. Journal of Neurosurgery 1986; 64 : 879-89.
  9. Mickey B, Close LG, Schaefer SD, Samson D. A combined frontotemporal and lateral infratemporal fossa approach to the skull base. Journal of Neurosurgery 1988; 68 : 678-83.
  10. Fisch U. The infratemporal fossa approach for nasopharyngeal tumours. Laryngoscope 1983; 93 : 36-44.
  11. Goepfert H, Cangir A, Lee Y. Chemotherapy for aggressive Juvenile nasopharyngeal angiofibroma. Archives of Otolaryngology and head neck surgery. 1985; 111 : 285-89.
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