Atormac
briv
Neurology India
menu-bar5 Open access journal indexed with Index Medicus
  Users online: 13568  
 Home | Login 
About Editorial board Articlesmenu-bullet NSI Publicationsmenu-bullet Search Instructions Online Submission Subscribe Videos Etcetera Contact
  Navigate Here 
 Search
 
  
 Resource Links
    Similar in PUBMED
    Article in PDF (1,987 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this Article
   References
   Article Figures
   Article Tables

 Article Access Statistics
    Viewed192    
    Printed2    
    Emailed0    
    PDF Downloaded8    
    Comments [Add]    

Recommend this journal

 


 
Table of Contents    
LETTER TO EDITOR
Year : 2021  |  Volume : 69  |  Issue : 5  |  Page : 1438-1441

Adjuvant Gamma Knife Radiosurgery for Advanced Juvenile Nasopharyngeal Angiofibroma


1 Department of Neurosurgery, All India Institute of Medical Sciences, Ansari Nagar, India
2 Department of Neurosurgery, Mayo Clinic Health System, Mankato, Minnesota, USA
3 Department of Otorhinolaryngology, All India Institute of Medical Sciences, Ansari Nagar, India

Date of Submission26-Sep-2017
Date of Decision01-Apr-2018
Date of Acceptance08-Nov-2019
Date of Web Publication30-Oct-2021

Correspondence Address:
Shashank S Kale
Department of Neurosurgery, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.329611

Rights and Permissions



How to cite this article:
Raheja A, Sharma MS, Singh M, Agrawal D, Kale SS, Sharma SC. Adjuvant Gamma Knife Radiosurgery for Advanced Juvenile Nasopharyngeal Angiofibroma. Neurol India 2021;69:1438-41

How to cite this URL:
Raheja A, Sharma MS, Singh M, Agrawal D, Kale SS, Sharma SC. Adjuvant Gamma Knife Radiosurgery for Advanced Juvenile Nasopharyngeal Angiofibroma. Neurol India [serial online] 2021 [cited 2021 Nov 30];69:1438-41. Available from: https://www.neurologyindia.com/text.asp?2021/69/5/1438/329611




Sir,

Juvenile nasopharyngeal angiofibroma (JNA) is a rare, benign tumor found typically in adolescent males. Although histologically benign, this tumor is locally invasive and becomes symptomatic via local tissue destruction causing nasal obstruction, epistaxis, chronic sinusitis, vision loss, headache, and cranial neuropathies.[1],[2] It classically originates in the nasopharynx at the postero-superior margin of the sphenopalatine foramen.[3] Surgery may be curative for smaller lesions and is the mainstay of treatment. Locally advanced tumors (Fisch stages III, IV) have higher rates of subtotal resection and therefore recurrence. Adjuvant modalities of treatment include preoperative embolization, external beam radiotherapy (conventional and stereotactic), and more recently, gamma knife radiosurgery (GKRS).[2],[4],[5],[6],[7] The role of GKRS remains poorly defined. Only five studies (17 cases) are detailed in the literature.[4],[5],[6],[7],[8] We report 2 cases of residual/recurrent advanced stage JNA who received secondary GKRS after being operated twice [Figure 1],[Figure 2],[Figure 3],[Figure 4] and [Table 1], [Table 2]. Both patients showed good functional and radiological outcome in long-term follow-up. Traditionally, surgery is considered to be the primary modality of treatment, especially for stage I, II, and IIIa tumors.[7],[8],[9],[10],[11],[12],[13],[14],[15],[16] Despite aggressive surgical resection, recurrence rates as high as 39.5% have been described in advanced-stage disease.[17] Historically, an operative mortality of 6.6% has been described.[18] Hence, the optimal management of advanced JNA (IIIb and IV), often involving cavernous sinus, remains controversial. Surgery for lesions within the cavernous sinus is certainly not without risk. Although a non-meningiomatous aetiology has been associated with a better outcome, mortality still ranges from 2.4% to 9.5% in series performed by leading international experts in the field.[19],[20],[21],[22],[23] Morbidity remains even higher because of the anatomical proximity of the vital neurovascular structures. Unfortunately, the chances of leaving a residual tumor behind remain high (20–86%).[21],[22],[23],[24],[25],[26],[27] Thus, the need for some form of adjuvant therapy often arises.
Figure 1: Patient 1: Treatment planning–Axial, contrast-enhanced stereotactic axial MR image, reveals a 4 cc residual juvenile nasopharyngeal angiofibroma in the region of the left pterygopalatine fossa extending into the cavernous sinus. The 50% isodose line (marked in yellow) corresponded to a 20 Gy marginal dose and covered 97% of the tumor (marked in red). The brainstem was segmented in magenta

Click here to view
Figure 2: Patient 1: Complete 3D treatment plan demonstrating the principles of isodose line (marked in yellow), marginal dose, and tumor coverage. Planning is done in accordance to avoid radiation dose of >10 Gy to adjacent radiosensitive structures such as brainstem (marked in magenta) and optic apparatus (marked in blue). Conformity between radiation dose pattern and tumor morphology is well maintained by adjusting the number and size of isocenters. Shielding technique is further utilized do further tailor the treatment plan

Click here to view
Figure 3: Pre (a) and post (b) Gamma Knife MR images for patient 2-(a) Treatment planning. Stereotactic, axial, contrast-enhanced, MR image, acquired at the level of the VIIth–VIIIth complex, reveals a large (24 cc) residual juvenile nasopharyngeal angiofibroma in the region of the right cavernous sinus producing ipsilateral proptosis. The 14 Gy (50%) isodose line (marked in yellow) covered 99% of the tumor, which was segmented in red. The 10 Gy isodose line (green) does not intersect the brainstem, which was segmented in magenta. (b) Corresponding, 78th month follow-up, axial contrast-enhanced MR image reveals a significant reduction in tumor volume and resolution of proptosis

Click here to view
Figure 4: Patient 2: Complete 3D treatment plan demonstrating the principles of isodose lines (50% marked in yellow and 10 Gy marked in green), marginal dose, and tumor coverage (marked in red). Planning is done in accordance to avoid radiation dose of >10 Gy to adjacent radiosensitive structures such as brainstem (marked in magenta) and optic apparatus (marked in green). Conformity between radiation dose pattern and tumor morphology is well maintained by adjusting the number the size of isocenters. Shielding technique is further utilized do further tailor the treatment plan

Click here to view
Table 1: Treatment planning

Click here to view
Table 2: Review of literature

Click here to view


GKRS involves the use of multiple confocal beams of Gamma rays with a steep dose fall-off and the surrounding, critical, neurovascular structures are consequently spared. It has a proven role in the management of cavernous sinus pathology and the use of high prescription doses can overcome the supposed radio-resistance of benign tumors. One of the largest series reports a nil mortality, tumor growth control rates of 98% at 47 months,[28] actuarial 10-year tumor control rates of 91.6–93%,[29],[30],[31] complication rates from 6–12%, and a serious morbidity rate of 0.8%.[28] In the series by Hasegawa et al., GKRS as the initial treatment was associated with a significant improvement of symptoms (P = 0.006).[31] The relative safety of GKRS in benign pediatric brain tumors has not been extensively documented because of their relative rarity and, possibly, the fear of secondary malignancies.[32],[33] On the other hand, GKRS has been used far more frequently in children with intracranial arteriovenous malformations (AVMs).[34],[35],[36],[37],[38],[39] Although the risk of developing a cranial malignancy after GKRS has not been reported, two patients did develop asymptomatic meningiomas 10 and 12 years post-procedure.[39] The role of GKRS for JNA remains poorly defined though intuitively logical. Till date, only 5 studies (17 cases) have shown a beneficial role of secondary GKRS for residual lesions [Table 2]. To these, we add two more cases, with a favorable long-term outcome and no permanent complications. This represents an advance over conventional radiotherapy, which is limited by potential long-term side effects such as the emergence of new malignancies, malignant transformation, cataracts, hypopituitarism, glaucoma, optic atrophy, xerostomia, temporal lobe necrosis, osteonecrosis, osteomyelitis, and caries.[4],[5],[6],[7],[40] The convenience of the same-day treatment, rare association with secondary neoplasms,[33] low complication rates, and an overall favorable outcome with good tumor control rates makes this a suitable modality for the treatment of advanced JNA after primary surgery has been done [Table 1] and [Table 2]. However, SRS is primarily reserved as an adjunct treatment option. Traditionally size limitation for instituting GKRS therapy is 3 cm, beyond which only hypofractionated GKRS using 5 or fewer fractions have been tried. Besides, there is a risk for “marginal miss” with GKRS because of necessarily tight dose distribution leading to potentially higher recurrence rates as compared to conventional radiotherapy along the tumor margins.

To sum up the cumulative data of all 19 patients, a median prescription dose of 20 Gy (range 14–30 Gy) delivered at a median isodose of 50% (range 45–55%) resulted in tumor control rates of 100% at a mean follow-up of 77.5 months (range 8–180 months). One-third of these tumors demonstrated a volume reduction after GKRS and received a prescription dose of less than 24 Gy. Three tumors disappeared (15.8%) after GKRS.[5],[6],[8] There were no neurological complications attributable to GKRS. We were unable to determine a dose-volume-outcome relationship because of insufficient data. These excellent results seem to mirror a pattern that we observed when using GKRS for glomus jugulare and pediatric intracranial AVMs.[34],[40] JNA too is a vascular tumor. It is possible that conformal, high dose, single fraction GKRS may induce obliterative endarteritis within the abnormal blood vessels of the tumor and yet spare adjacent normal structures because of the steep dose fall-off.[5],[41] Interestingly, Hayashi et al. reported the greatest shrinkage in tumor volume in patients with cavernous sinus hemangiomas after GKRS.[28] We believe that it is important to highlight the sensitivity of even large JNAs to GKRS as is evident in this report (Patient 2, tumor volume 24 cc, marginal dose 14 Gy; [Table 1] and [Figure 3], [Figure 4]. We were able to treat such a large tumor by reducing the marginal dose to 14 Gy and limiting the maximum exposure of the visual pathways to less than 10 Gy. This decision was taken after a multidisciplinary team of experts deemed further surgery as high risk. Surprisingly, despite the lower marginal dose, the tumor volume reduced significantly within a few months of the procedure and his proptosis recovered significantly, and there was no tumor recurrence noted during long-term 78-month follow-up for this patient. This report again raises a controversial question—is there a role for a planned sub-total resection with elective GKRS for strategically located residual tumors in patients with advanced JNA?[7],[41] On the one hand, this could potentially save a young patient from undergoing multiple, potentially mutilating procedures only to have him/her eventually undergo adjuvant GKRS for a large residual tumor, as seen in this series. On the other hand, it is possible that GKRS may induce a late recurrence/malignant transformation, which could jeopardize further definitive surgery. Certainly, recommendations cannot be made on the basis of available data at this point in time till a longer follow-up of a greater number of patients is available. To conclude, GKRS is a safe and effective adjuvant modality of treatment for residual tumors in and around the cavernous sinus following surgery for advanced JNA. Long-term tumor control/regression was seen in this series despite treating large volumes with a 14 Gy (50% isodose) marginal dose.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Hofmann T, Bernal-Sprekelsen M, Koele W, Reittner P, Klein E, Stammberger H. Endoscopic resection of juvenile angiofibromas--Long term results. Rhinology 2005;43:282-9.  Back to cited text no. 1
    
2.
Scholtz AW, Appenroth E, Kammen-Jolly K, Scholtz LU, Thumfart WF. Juvenile nasopharyngeal angiofibroma: Management and therapy. Laryngoscope 2001;111:681-7.  Back to cited text no. 2
    
3.
Liu ZF, Wang DH, Sun XC, Wang JJ, Hu L, Li H, et al. The site of origin and expansive routes of juvenile nasopharyngeal angiofibroma (JNA). Int J Pediatr Otorhinolaryngol 2011;75:1088-92.  Back to cited text no. 3
    
4.
Dare AO, Gibbons KJ, Proulx GM, Fenstermaker RA. Resection followed by radiosurgery for advanced juvenile nasopharyngeal angiofibroma: Report of two cases. Neurosurgery 2003;52:1207-11.  Back to cited text no. 4
    
5.
Park CK, Kim DG, Paek SH, Chung HT, Jung HW. Recurrent juvenile nasopharyngeal angiofibroma treated with gamma knife surgery. J Korean Med Sci 2006;21:773-7.  Back to cited text no. 5
    
6.
Roche PH, Paris J, Régis J, Moulin G, Zanaret M, Thomassin JM, et al. Management of invasive juvenile nasopharyngeal angiofibromas: The role of a multimodality approach. Neurosurgery 2007;61:768-77.  Back to cited text no. 6
    
7.
Alvarez FL, Suárez V, Suárez C, Llorente JL. Multimodality approach for advanced-stage juvenile angiofibromas. Head Neck 2013;35:209-13.  Back to cited text no. 7
    
8.
Min HJ, Chung HJ, Kim CH. Delayed cerebrospinal fluid rhinorrhea four years after gamma knife surgery for juvenile angiofibroma. J Craniofac Surg 2014;25:e565-7.  Back to cited text no. 8
    
9.
Fagan JJ, Snyderman CH, Carrau RL, Janecka IP. Nasopharyngeal angiofibromas: Selecting a surgical approach. Head Neck 1997;19:391-9.  Back to cited text no. 9
    
10.
Jones GC, DeSanto LW, Bremer JW, Neel HB 3rd. Juvenile angiofibromas. Behavior and treatment of extensive and residual tumors. Arch Otolaryngol Head Neck Surg 1986;112:1191-3.  Back to cited text no. 10
    
11.
Lloyd G, Howard D, Phelps P, Cheesman A. Juvenile angiofibroma: The lessons of 20 years of modern imaging. J Laryngol Otol 1999;113:127-34.  Back to cited text no. 11
    
12.
Andrews JC, FischU, Valavanis A, Aeppli U, Makek M. The surgical management of extensive nasopharyngeal angiofibromas with the infratemporal fossa approach. Laryngoscope 1989;99:429-37.  Back to cited text no. 12
    
13.
Fisch U. The infratemporal fossa approach for nasopharyngeal tumors. Laryngoscope 1983;93:36-44.  Back to cited text no. 13
    
14.
Sekhar LN, Schramm VL Jr, Jones NF. Subtemporal-preauricularinfratemporal fossa approach to large lateral and posterior cranial base neoplasms. J Neurosurg 1987;67:488-99.  Back to cited text no. 14
    
15.
Zhang M, Garvis W, Linder T, Fisch U. Update on the infratemporal fossa approaches to nasopharyngeal angiofibroma. Laryngoscope 1998;108:1717-23.  Back to cited text no. 15
    
16.
Di Rienzo A, Ricci A, Scogna A, Zotta D, Stati G, Galzio R, et al. The open-mouth fronto-orbitotemporozygomatic approach for extensive benign tumors with coexisting splanchnocranial and neurocranial involvement. Neurosurgery 2004;54:1170-80.  Back to cited text no. 16
    
17.
Herman P, Lot G, Chapot R, Salvan D, Huy PT. Long-term follow-up of juvenile nasopharyngeal angiofibromas: Analysis of recurrences. Laryngoscope 1999;109:140-7.  Back to cited text no. 17
    
18.
Jafek BW, Krekorian EA, Kirsch WM, Wood RP. Juvenile nasopharyngeal angiofibroma: Management of intracranial extension. Head Neck Surg 1979;2:119-28.  Back to cited text no. 18
    
19.
Dolenc VV, Kregar T, Ferluga M, Fettich M, Morina A. Treatment of tumors invading the cavernous sinus. In: Dolenc VV, editor. The Cavernous Sinus. A Multidisciplinary Approach to Vascular and Tumorous Lesions. Wien: Springer-Verlag; 1987. p. 377-91.  Back to cited text no. 19
    
20.
Sepehrnia A, Samii M, Tatagiba M. Management of intracavernoustumours: An 11-year experience. ActaNeurochir (Suppl) 1991;53:122-6.  Back to cited text no. 20
    
21.
DeMonte F, Smith HK, Al-Mefty O. Outcome of aggressive removal of cavernous sinus meningiomas. J Neurosurg 1994;81:245-51.  Back to cited text no. 21
    
22.
Kim DK, Grieve J, Archer DJ, Uttley D. Meningiomas in the region of the cavernous sinus: A review of 21 patients. Br J Neurosurg 1996;10:439-44.  Back to cited text no. 22
    
23.
Newman S. A prospective study of cavernous sinus surgery for meningiomas and resultant common ophthalmic complications (an American Ophthalmological Society thesis). Trans Am Ophthalmol Soc 2007;105:392-447.  Back to cited text no. 23
    
24.
Cusimano MD, Sekhar LN, Sen CN, Pomonis S, Wright DC, Biglan AW, et al. The results of surgery for benign tumors of the cavernous sinus. Neurosurgery 1995;37:1-9.  Back to cited text no. 24
    
25.
Lesoin F, Pellerin P, Autricque A, Clarisse J, Jomin M. The direct microsurgical approach to intracavernous tumors. In: Dolenc VV, editor. The Cavernous Sinus. A Multidisciplinary Approach to Vascular and Tumorous Lesions. Wien: Springer-Verlag; 1987. p. 323-31.  Back to cited text no. 25
    
26.
O'Sullivan MG, van Loveren HR, Tew JM Jr. The surgical resectability of meningiomas of the cavernous sinus. Neurosurgery 1997;40:238-44.  Back to cited text no. 26
    
27.
Knosp E, Perneczky A, Koos WT, Fries G, MatulaC. Meningiomas of the space of the cavernous sinus. Neurosurgery 1996;38:434-42.  Back to cited text no. 27
    
28.
Hayashi M, Chernov M, Tamura N, Tamura M, Horiba A, Konishi Y, et al. Gamma knife radiosurgery for benign cavernous sinus tumors: Treatment concept and outcomes in 120 cases. Neurol Med Chir (Tokyo) 2012;52:714-23.  Back to cited text no. 28
    
29.
Pollock BE, Stafford SL, Link MJ, Garces YI, Foote RL. Single-fraction radiosurgery of benign cavernous sinus meningiomas. J Neurosurg 2013;119:675-82.  Back to cited text no. 29
    
30.
Skeie BS, Enger PO, Skeie GO, Thorsen F, Pedersen PH. Gamma knife surgery of meningiomas involving the cavernous sinus: Long-term follow-up of 100 patients. Neurosurgery 2010;66:661-8.  Back to cited text no. 30
    
31.
Hasegawa T, Kida Y, Yoshimoto M, Koike J, Iizuka H, Ishii D. Long-term outcomes of Gamma Knife surgery for cavernous sinus meningioma. J Neurosurg 2007;107:745-51.  Back to cited text no. 31
    
32.
Hirth A, Pedersen PH, Baardsen R, Larsen JL, Krossnes BK, Helgestad J. Gamma-knife radiosurgery in pediatric cerebral and skull base tumors. Med Pediatr Oncol 2003;40:99-103.  Back to cited text no. 32
    
33.
Rowe J, Grainger A, Walton L, Silcocks P, Radatz M, Kemeny A. Risk of malignancy after gamma knife stereotactic radiosurgery. Neurosurgery 2007;60:60-5.  Back to cited text no. 33
    
34.
Kiran NA, Kale SS, Vaishya S, Kasliwal MK, Gupta A, Sharma MS, et al. Gamma Knife surgery for intracranial arteriovenous malformations in children: A retrospective study in 103 patients. J Neurosurg 2007;107 (6 Suppl):479-84.  Back to cited text no. 34
    
35.
Pan DH, Kuo YH, Guo WY, Chung WY, Wu HM, Liu KD, et al. Gamma Knife surgery for cerebral arteriovenous malformations in children: A 13-year experience. J Neurosurg Pediatr 2008;1:296-304.  Back to cited text no. 35
    
36.
Foy AB, Wetjen N, Pollock BE. Stereotactic radiosurgery for pediatric arteriovenous malformations. Neurosurg Clin N Am 2010;21:457-61.  Back to cited text no. 36
    
37.
Dinca EB, de Lacy P, Yianni J, Rowe J, Radatz MW, Preotiuc-Pietro D, et al. Gamma knife surgery for pediatric arteriovenous malformations: A 25-year retrospective study. J Neurosurg Pediatr 2012;10:445-50.  Back to cited text no. 37
    
38.
Kano H, Kondziolka D, Flickinger JC, Yang HC, Flannery TJ, Awan NR, et al. Stereotactic radiosurgery for arteriovenous malformations, part 2: Management of pediatric patients. J Neurosurg Pediatr 2012;9:1-10.  Back to cited text no. 38
    
39.
Yen CP, Monteith SJ, Nguyen JH, Rainey J, Schlesinger DJ, Sheehan JP. Gamma Knife surgery for arteriovenous malformations in children. J Neurosurg Pediatr 2010;6:426-34.  Back to cited text no. 39
    
40.
Sharma MS, Gupta A, Kale SS, Agrawal D, Mahapatra AK, Sharma BS. Gamma knife radiosurgery for glomus jugulare tumors: Therapeutic advantages of minimalism in the skull base. Neurol India 2008;56:57-61.  Back to cited text no. 40
[PUBMED]  [Full text]  
41.
Lee JT, Chen P, Safa A, Juillard G, Calcaterra TC. The role of radiation in the treatment of advanced juvenile angiofibroma. Laryngoscope 2002;112:1213-20.  Back to cited text no. 41
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1], [Table 2]



 

Top
Print this article  Email this article
   
Online since 20th March '04
Published by Wolters Kluwer - Medknow