Tumor Spread
JNAs develop at the posterolateral wall of the nasal cavity. Anatomically, the site of origin is
thought to be the area of the pterygoid canal and the sphenopalatine foramen [6, 8]. From this
location tumor grows easily medially to the nasopharynx, forward to the nasal cavity, upward
to the sphenoid sinus and laterally to the pterygopalatine fossa. Approximately 20% to 40%
of patients have skull base invasion at the time of diagnosis [1, 6, 9]. In our study 10 of all 62
(16%) patients with JNA diagnosed at our institution presented with intracranial extension.
According to the literature several routes of intracranial invasion can be identified:
from the pterygo-palatine fossa via the superior orbital fissure into parasellar
compartment of the middle cranial fossa;
from the sphenoid sinus by direct erosion of its superior wall into pituitary fossa
and/or medially to the cavernous sinus;
from the pterygoid canal deep invasion of the cancellous bone of the pterygoid base
and greater wing of the sphenoid may result in involvement of the middle cranial
fossa laterally to the cavernous sinus;
from the infratemporal fossa through direct erosion of the floor of the middle cranial
fossa and/or natural channels (foramen rotundum, foramen ovale), which perforate the
middle fossa;
from the ethmoid sinus by erosion of the floor of the anterior cranial fossa [2].
Despite the limited number of patients, all above mentioned routes of spread were observed
in our study. Our results indicate, that most frequently the tumor spreads along the first way,
via superior orbital fissure, which results in parasellar extension in proximity of the
cavernous sinus. This growth pattern is often associated with invasion of the pterygo-palatine
and infratemporal fossa. Tumor growth through soft tissue channels is relatively unrestricted
in comparison to invasion of anatomical spaces confined within bony walls. It may occur in
an early stage of the disease, as natural fissures form a preferable way for tumor spread.
Extension to the anterior portion of the cranium is infrequent. It occurs in advanced tumors
with massive involvement of the sphenoid and ethmoid sinuses, and was reported by
Elsharkawy et al. [10] in 1/23 patients and by Margalit et al. [9] in 3/21 patients. In our study
1 patient had erosion of the floor of the anterior cranial fossa, without significant intracranial
extension.
Tumor Imaging
Clinical findings typical for JNA is a nasopharyngeal mass in a young male with the history
of epistaxis. However, the size of the tumor in the nasopharynx does not always represent the
size of the whole tumor, as extensive growth may be clinically silent. CT and MRI studies are
essential part of evaluation of the disease and allow precise preoperative staging of the tumor.
They are both helpful to confirm the diagnosis and visualize the exact extension of the lesion.
CT better delineates bony details of the skull base involved with the tumor, including bony
erosion, widening of natural foramina and fissures, invasion into cancellous bone of the
greater wing of the sphenoid. MRI has the advantage of showing soft tissue characteristics
and is superior in determining intraorbital and intracranial extensions. It is crucial for
assessment of tumor relation to important intracranial structures, like the internal carotid
artery, cavernous sinus, pituitary gland, as well dural involvement. The extent of cellular
invasion of the sphenoid diploe may also be reliably assessed on MR images with use of fat
saturation techniques and contrast enhancement.
Intracranial extension of JNA usually displaces the dura or may become to some degree
adherent to it. However, it is not always possible to determine radiologically the relationship
of the tumor to the dural plane. The radiological criteria of transdural growth, like the lack of
interface between the tumor and neurovascular structures or encasement of the internal
carotid artery, may lead to misinterpretation and overstaging of tumors [11, 12]. Danesi et al.
[13] reported that 50% of patients with preoperative radiological stage IV according to Fisch
classification were classified as IIIB on the basis of surgical interpretation. Partial or total
encasement of the internal carotid artery on MR images does not determine cavernous sinus
involvement and a dural plane is usually present at operation [11]. In our study cavernous
sinus involvement occurred in 1 patient. Although in the literature there are a few reports of
angiofibromas with intradural extension, in a vast majority of advanced cases tumors remain
extradural and brain infiltration has never been observed [2, 6]. Single cases of dural
penetration have been reported by Roche et al. [6] and Danesi et al. [11]. Angiography is
used to assess tumor vascular composition and blood supply, and enables preoperative
embolization. Typically, major arterial supply to this hypervascular tumor is the internal
maxillary artery. Branches of the internal carotid artery may be additional feeders in
advanced tumors. However, it is not a reliable sing of intracranial invasion. Involvement of
the sphenoid sinus may be associated with blood supply from the branches of the cavernous
portion of the ICA and invasion of the orbit or ethmoid sinus may result in arterial supply
from ethmoid arteries. The presence of cerebral parenchymal vessels with collateral feeding
branches to the tumor may be a sign of dural penetration [11].
Tumor Staging
Several staging systems relying on CT and MRI findings have been introduced for JNAs
(Tab. I.). These classifications depend on tumor involvement of particular anatomical
compartments and reflect the risk of postoperative residual/recurrent disease associated with
particular locations. Therefore, they are helpful in selection of surgical approach. The most
prevalent staging systems seem to be those of Fisch’s, Andrews’ and Radkowski’s. The
classification proposed by Fish [15] reflects the growth pattern of JNAs, with intracranial
tumors included in stage III and IV. The staging system suggested by Andrews et al. [7] is a
modified system introduced by Fish and it more precisely defines intracranial spread,
distinguishing extradural parasellar involvement (IIIb), intradural (IVa) and intradural with
infiltration of the cavernous sinus, pituitary fossa or optic chiasm (IVb). Radkowski et al.
[16] proposed a staging system, which highlighted the importance of tumor extension
posterior to the pterygoid plates (IIc) and distinction between minimal and extensive
intracranial involvement. Another recently proposed staging system by Snyderman et al. [17]
is focused on residual vascularity from the intracranial circulation following embolization
and routes of intracranial invasion, subdivided into medial or lateral to the cavernous and
paraclival segments of the ICA. This endoscopic classification reflects potential morbidity
and risk of residual disease associated with these two routes of intracranial growth.
1. Bales C., Kotapka M., Loevner L.A., Al-Ravi M., Weinstein G. et al.: Craniofacial
resection of advanced juvenile nasopharyngeal angiofibroma. Arch. Otolaryngol. Head.
Neck. Surg., 2002; 128: 1071–1078.
2. Schick B., Kahle G.: Radiological findings in angiofibroma. Acta. Radiol., 2000; 41:
585–593.
3. Szymańska A., Szymański M., Morshed K., Czekajska-Chehab E., Szczerbo-
-Trojanowska M.: Extranasopharyngeal angiofibroma: clinical and radiological
presentation. Eur. Arch. Otorhinolaryngol., 2013; 270: 655–660.
4. Windfuhr J.P., Remmert S.: Extranasopharyngeal angiofibroma: etiology, incidence and
management. Acta Otolaryngol., 2004; 124(8): 880–889.
5. Blount A., O’Riley K., Woodworth B.A.: Juvenile nasopharyngeal angiofibroma.
Otolaryngol. Clin. North Am., 2011; 44: 989–1004.
6. Roche P.H., Paris J., Regis J., Moulin G., Zanaret M. et al.: Management of invasive
juvenile nasopharyngeal angiofibromas: the role of multimodality approach.
Neurosurgery, 2007; 61: 768–777.
7. Andrews J.C., Fisch U., Valavanis A., Aeppli U., Makek M.S.: The surgical management
of extensive nasopharyngeal angiofibromas with the infratemporal fossa approach.
Laryngoscope, 1989; 99(4): 429–437.
8. Liu Z.F., Wang D.H., Sun X.C., Wang J.J., Hu L. et al.: The site of origin and expansive
routes of juvenile nasopharyngeal angiofibroma. Int. J. Pediatr. Otorhinolaryngol., 2011;
75: 1088–1092.
9. Margalit N., Wasserzug O., De-Row A., Abergel A., Fliss D.M. et al.: Surgical treatment
of juvenile nasopharyngeal angiofibroma with intracranial extension. J. Neurosurg.
Pediatrics, 2009; 4: 113–117.
10. Elsharkawy A.A., Kamal E.M., Tawfik A., Zaher A., Kasem M.: Juvenile
nasopharyngeal angiofibroma with intracranial extension: analysis of 23 Egyptian
patients. Int. J. Ped. Otorhinolaryngol., 2010; 74: 755–759.
11. Danesi G., Panciera D.T., Harvey R., Agostinis C.: Juvenile nasopharyngeal
angiofibroma: evaluation and surgical management of advanced disease. Otolaryngol
Head Neck Surg., 2008; 138: 581–586.
12. Lee J.T., Chen P., Safa A., Juillard G., Calcaterra T.C.: The role of radiation in the
treatment of advanced juvenile angiofibroma. Laryngoscope, 2002; 112: 1213–1220.
13. Danesi G., Panizza B., Mazzoni A., Calabrese V.: Anterior approaches in juvenile
nasopharyngeal angiofibroma with intracranial extension. Otolaryngol. Head Neck Surg.,
2000; 122: 277–283.
14. Budzynowska K., Pietniczka M., Dowżenko A., Borowska K., Czepiel W.: Safe
extirpating of AFJ after preoperative tumor obliteration with tissue adhesive glue.
Otolaryngol. Pol., 2008; 62: 408–411.
15. Fisch U.: The infratemporal fossa approach for nasopharyngeal tumors. Laryngoscope,
1983; 93(1): 36–44.
16. Radkowski D., McGill T., Healy G.B., Ohlms L., Jones D.T.: Angiofibroma: changes in
staging and treatment. Arch Otolaryngol Head Neck Surg, 1996; 122(2): 122–129.
17. Snyderman C.H., Pant H., Carrau R.L., Gardner P.: A new endoscopic staging system for
angiofibromas. Arch. Otolaryngol. Head Neck Surg., 2010; 136(6): 588–594.
18. Tyagi I., Syal R., Goyal A.: Staging and surgical approaches in large juvenile
angiofibroma – study of 95 cases. Int. J. Ped. Otorhinolaryngol., 2006; 70: 1619–1627.
19. Gołąbek W., Szymańska A., Siwiec H., Trojanowski P.: Transpalatal approach for
juvenile angiofibroma. Otolaryngol. Pol., 2008; 62: 16–19.
20. Balcerzak J., Gotlib T., Hamera M., Osuch-Wójcikiewicz E., Niemczyk K.:
Angiofibroma juvenile – evolution of surgical management. Otolaryngol. Pol. 2011; 65(5
Suppl): 12–16.
21. Szyfter W., Borucki Ł., Balcerowiak A.: Endoscopic surgery for selected tumors of the
nose and paranasal sinuses – practical sense of the four hands technique. Otolaryngol.
Pol., 2008; 62: 170–174.
22. Snyderman C.H., Pant H.: Endoscopic management of vascular sinonasal tumors,
including angiofibroma. Otolaryngol. Clin. North Am., 2016; 49(3): 791–807.
23. Charkaborty S., Ghoshal S., Patil V.M,. Oinam A.S., Sharma S.C.: Conformal
radiotherapy in the treatment of advanced juvenile nasopharygeal angiofibroma with
intracranial extension: an institutional experience. Int. J. Radiation Oncology Biol. Phys.,
2011; 80: 1398–1404.
24. Rekonnen S., Hagstrom J., Vuola J., Niemela M., Porras M. et al.: The changing surgical
management of juvenile nasopharyngeal angiofibroma. Eur. Arch. Otorhinolaryngol.,
2011; 268: 599–607.
25. Tewfik T.L., Tan A.K.W., Noury K.A., Chowdhury K., Tampieri D. et al.: Juvenile
nasopharyngeal angiofibroma. J LaryngolOtol, 1999; 28: 145–151.
26. Dare A.O., Gibbons K.J., Proulx G.M., Fenstermaker R.A.: Resection followed by
radiosurgery for advanced juvenile nasopharyngeal angiofibroma: report of two cases.
Neurosurgery, 2003; 52: 1207–1211.
27. McAfee W.J., Morris C.G., Amdur R.J., Werning J.W., Mendenhall W.M.: Definitive
radiotherapy for nasopharyngeal angiofibroma. Am J Clin Oncol, 2006; 29(2): 168–170.
28. Rowan N.R., Zwagerman N.T., Heft-Neal M.E., Gardner P.A., Snyderman C.H.:
Juvenile nasal angiofibroma: a comprarison of modern staging systems in an endoscopic
era. J. Neurol. Surg. B, 2017; 78(1): 63–67.
29. Rowan N.R., Stapleton A.L., Heft-Neal M.E., Gardner P.A., Snyderman C.H.: The
natural growth rate of residual juvenile angiofibroma. J Neurol Surg Skull Base, 2018;
79: 257–261.
30. Paris J., Guelfucci B., Moulin G., Zanaret M., Triglia J.M.: Diagnosis and treatment of
juvenile nasopharyngeal angiofibroma. Eur Arch Otorhinolaryngol, 2001; 258: 120–124.
