RESECTION FOLLOWED BY RADIOSURGERY FOR

ADVANCED JUVENILE NASOPHARYNGEAL ANGIOFIBROMA:

REPORT OF TWO CASES
Amos O. Dare, M.D. OBJECTIVE AND IMPORTANCE: Experience with the management of juvenile naso-
Department of Neurosurgery, pharyngeal angiofibroma (JNA) by gamma knife radiosurgery is limited. We report
School of Medicine and
Biomedical Sciences, University at
control of the disease in two patients with advanced-stage JNA treated with primary
Buffalo, State University of New resection followed by gamma knife stereotactic radiosurgery of residual disease.
York, Buffalo, New York CLINICAL PRESENTATION: An 18-year-old man presented with chronic sinusitis,
worsening headaches, diplopia, and left-sided facial numbness. A second patient, a
Kevin J. Gibbons, M.D.
19-year-old man, presented with recurrent epistaxis and nasal congestion. Magnetic
Department of Neurosurgery,
School of Medicine and
resonance imaging findings and endoscopic evaluation in each patient were consistent
Biomedical Sciences, University at with advanced-stage JNA.
Buffalo, State University of New
York, Buffalo, New York
INTERVENTION: One patient underwent craniofacial resection with approximately
3.0 cm3 of residual tumor in the region of the cavernous sinus. The other patient
Gary M. Proulx, M.D. underwent preoperative embolization followed by a lateral rhinotomy for tumor
Gamma Knife Center, Roswell Park resection with approximately 4.7 cm3 of residual tumor in the right infratemporal fossa.
Cancer Institute, Buffalo, New In an attempt to limit radiation to surrounding normal brain, residual tumor in both
York patients was treated with gamma knife stereotactic radiosurgery. Control of disease
was documented by magnetic resonance imaging more than 24 months after
Robert A. Fenstermaker,
treatment.
M.D.
Department of Neurosurgery,
CONCLUSION: Short-term control of late-stage JNA was achieved by use of a strategy
School of Medicine and of primary surgical resection followed by gamma knife radiosurgery of residual tumor
Biomedical Sciences, University at in two patients. Establishing the effectiveness and safety of this strategy over conven-
Buffalo, State University of New
York, and Gamma Knife Center,
tional methods of managing advanced JNA will require future prospective studies.
Roswell Park Cancer Institute, KEY WORDS: Angiofibroma, Gamma knife radiosurgery, Surgery
Buffalo, New York
Neurosurgery 52:1207-1211, 2003 DOI: 10.1227/01.NEU.0000058022.97390.7A www.neurosurgery-online.com
Reprint requests:
Amos O. Dare, M.D., Department
of Neurosurgery, School of

Medicine and Biomedical
Sciences, University at Buffalo,
State University of New York, 3
Gates Circle, Buffalo, NY
14209-1194.
Email: dare@acsu.buffalo.edu
Received, April 2, 2002.
Accepted, January 8, 2003.
J
uvenile nasopharyngeal angiofibroma
(JNA) is a tumor arising from cells in
the region of the sphenopalatine fora-
men (25). Although histologically benign,
the tumor may develop an aggressive
growth pattern and has therefore been man-
aged by various protocols, including sur-
gery (2, 8, 10, 11, 13, 16–18, 21, 22, 25, 29),
radiation therapy (4, 7, 10, 27, 30), or a com-
bination of the two (9, 12, 19, 28). Most
authors agree that surgery is the primary
and exclusive treatment of early-stage dis-
ease when gross total resection can be
achieved (1, 6, 9, 10, 13, 18, 21, 22). However,
because of a higher rate of tumor recurrence
associated with advanced-stage lesions, con-
troversy surrounds the optimal manage-
ment of disease with widespread cranial
base extension or intracranial involvement.
The relative safety of gamma knife stereo-
tactic radiosurgery in the management of
pediatric head and neck tumors has been
documented (3, 5). However, experience
with the management of JNA by gamma
knife radiosurgery is limited. We report two
cases of late-stage JNA treated by primary
surgical resection and gamma knife stereo-
tactic radiosurgery of residual tumor, with
control of disease at follow-up evaluation 3
years later.
CASE REPORTS
Patient 1
An 18-year-old man presented with wors-
ening headaches, double vision, and left-sided
facial numbness. He had been treated for
chronic sinusitis in the past. Evaluation with
computed tomographic and magnetic reso-
nance imaging (MRI) studies revealed a left

NEUROSURGERY VOLUME 52 | NUMBER 5 | MAY 2003 | 1207

DARE ET AL.

nasal and nasopharyngeal mass with extension into the infra-

temporal fossa, the apex of the left orbit, and the left cavernous
sinus (Fig. 1, A and B). Signal characteristics of the mass were
consistent with Stage IV JNA (6). The patient underwent
craniofacial resection, with residual tumor in the region of the
cavernous sinus that measured approximately 3.0 cm3 (Fig.
2A). His preoperative symptoms of headaches, facial numb-
ness, and diplopia improved significantly. The final pathology
report confirmed the diagnosis of JNA. Four months after
surgery, the patient underwent gamma knife treatment and
received 20 Gy to the tumor margin (45% isodose line) (Fig.
3A). The patient tolerated treatment without complications. A
follow-up evaluation at 2 years revealed no change in the size
of the tumor (Fig. 2B). The patient is highly functional, with no FIGURE 2. Gadolinium-enhanced, T1-weighted postoperative MRI scans
neurological deficits. in Patient 1. A, axial view, showing residual tumor in the region of the
left cavernous sinus (arrow) measuring approximately 3.0 cm3. B, axial
Patient 2 view, obtained at follow-up examination 2 years after gamma knife radio-
surgery, revealing stable disease (arrow, left cavernous sinus).
A 19-year-old man presented with a 2-year history of recur-
rent episodes of epistaxis associated with progressive right
nasal congestion. After failed treatment for presumed sinus-
itis, the patient was evaluated by computed tomography and
MRI studies. A densely enhancing mass occupying the right
nasal and nasopharyngeal space with extension into the infra-
temporal fossa was discovered (Fig. 4, A and B). With a pre-
sumed diagnosis of Stage III JNA, the patient underwent
preoperative embolization, followed by a lateral rhinotomy
for tumor resection. Pathology results confirmed the diagnosis
of JNA. Immediate and follow-up postoperative MRI studies
revealed residual tumor that measured approximately 4.7 cm3
located in the right infratemporal fossa (Fig. 5A). Seven
months after surgery, the patient was treated with gamma
knife radiosurgery. A dose of 20 Gy was delivered to the
tumor margin (55% isodose line) (Fig. 3B). The patient toler- FIGURE 3. Gadolinium-enhanced, T1-weighted MRI scans, showing dose
ated treatment without complications. A follow-up MRI study distribution for radiosurgery. A, Patient 1 (axial view). A dose of 20 Gy
2 years later revealed no change in the size of the residual was delivered to the 45% isodose line. B, Patient 2 (axial view). A dose of
tumor (Fig. 5B). Clinically, the patient has had no further 20 Gy was delivered to the 55% isodose line.
episodes of epistaxis and is without neurological deficit.

DISCUSSION
JNA is most commonly diagnosed in adolescent males.
Histologically, the tumor is benign. Clinically, it may develop
an aggressive growth pattern. Tumor may be confined to the

FIGURE 4. Gadolinium-enhanced, T1-weighted MRI scans in Patient 2

(A, sagittal view; B, coronal view), showing a densely enhancing mass
occupying the right nasal cavity and nasopharynx with extension into the
infratemporal fossa.

nasopharynx (Stage I); extend into the nasal cavity and/or

FIGURE 1. Gadolinium-enhanced, T1-weighted MRI scans in Patient 1.
A, coronal view, showing left nasal and nasopharyngeal mass with exten-
sion into the infratemporal fossa and the apex of the left orbit. B, axial
view, showing invasion of the left cavernous sinus.
sphenoid sinus (Stage II); extend into the antrum, ethmoid
sinus, pterygomaxillary and infratemporal fossae, orbit,
and/or cheek (Stage III); or even extend intracranially (Stage
IV) (6).

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FIGURE 5. Gadolinium-enhanced, T1-weighted postoperative MRI scans
in Patient 2. A, coronal view, showing residual tumor in the right infra-
temporal fossa measuring approximately 4.7 cm3 (arrow). B, coronal view,
showing evidence of tumor necrosis 2 years after treatment with gamma
knife radiosurgery (arrow).
Traditionally, surgery has been used exclusively in the man-
agement of primary and recurrent lesions. Primary surgical
resection has been particularly effective in the control of early-
stage disease (Stage I or II) and sometimes early Stage III
disease (1, 2, 11, 16, 17, 29). Usually, a gross total resection is
achieved with a lateral rhinotomy or a transpalatal approach.
Tumor extending into the pterygopalatine and infratemporal
fossa may be resected with a LeFort I approach (11). More
recently, tumors confined to the nasopharynx, nasal cavity,
and paranasal sinuses have been removed by use of a purely
transnasal endoscopic approach (11). Advanced-stage disease
with variable cranial base involvement and intracranial exten-
sion requires more extensive surgery. Maxillectomy may be
necessary through a lateral rhinotomy or a Weber-Ferguson or
a midfacial degloving incision. Frontotemporal and orbitozy-
gomatic craniotomies and craniofacial approaches may be
used as indicated (11, 16, 18). Often, only a subtotal resection
is achieved in extensive disease, with residual tumor involv-
ing the cavernous sinus, temporal fossa, and orbital apex (11,
16, 18). In advanced disease, complications associated with
resection include intraoperative blood loss requiring the trans-
fusion of several units of blood (18). Other reported compli-
cations include neuralgia attributable to sectioning of the Vth
cranial nerve, hearing loss, trismus related to lateral infratem-
poral approaches, meningitis, amaurosis, and ophthalmople-
gia and hemiparesis with intracranial approaches (16, 18). Late
complications include nasal crusting, infraorbital nerve dys-
esthesia, lacrimal duct stenosis, otitis media, facial deformities,
and ocular abnormalities requiring further corrective surgery
(11, 16). Jafek et al. (18) reported a surgical mortality of 6.6% (1
of 15 patients) for advanced-stage disease with intracranial
extension. More recent series have reported no deaths (11, 16).
Recurrence after surgery seems to be influenced by disease
stage. Rates of 0 to 7% have been reported for early-stage
disease (Stages I and II) (1, 2, 11, 16, 17, 29). Advanced-stage
NEUROSURGERY
SURGERY AND RADIOSURGERY FOR ANGIOFIBROMA
lesions (Stages III and IV) are considerably less likely to be
controlled by surgery alone. Even with aggressive cranial base
approaches, these lesions recur at a higher rate, in the range of
5.5 to 39.5% (11, 16, 18). In a recent retrospective review of 44
patients treated primarily by surgery in which two-thirds of
the patients harbored Stage III lesions (according to the stag-
ing system proposed by Radkowski et al. [26]) or Stage IV
lesions (according to the scheme by Chandler et al. [6]), the
recurrence rate was 27.5% (16). More frequent recurrence was
correlated with higher stage of disease, which was character-
ized by widespread cranial base invasion of the infratemporal
fossa, sphenoid sinus, base of the pterygoids and clivus, me-
dial cavernous sinus, and anterior fossa. Recurrence in Stage
III disease was 39.5%, compared with a 7% recurrence in Stage
I and II disease (16). Similarly, in their review of 16 patients
managed primarily by surgery, Fagan et al. (11) concluded
that the propensity for tumor recurrence is highest when
tumor extends beyond the bony confines of the nose, ptery-
gopalatine fossa, and paranasal sinuses and invades the infra-
temporal fossa, cavernous sinus, and brain. Consequently,
most authors agree that surgery is the primary and exclusive
treatment for early-stage JNA (1, 6, 9, 10, 13, 18, 21, 22).
However, controversy still revolves around the optimal man-
agement of disease with widespread cranial base extension
(Stage III) and/or intracranial involvement (Stage IV).
Alternatively, some authors have used conventional frac-
tionated radiation therapy as a first line of treatment and
sometimes as an exclusive treatment modality in the control of
primary and recurrent JNA (4, 7). Others have advocated
radiation therapy for selected patients only. These patients
include those with intracranial extension of disease (10, 15, 30,
31), recurrent disease (9, 12, 16, 19), and postoperative intra-
cranial disease or residual tumor in areas associated with a
high morbidity for surgical resection (e.g., cavernous sinus) (9,
21, 28). Unfortunately, case series are often reported with
outcomes that have been combined for patients treated with
both primary and salvage radiation therapy. Also, few of these
reports have used the staging schemes that are often used in
the surgical series. Therefore, not only is it difficult to deter-
mine pure treatment outcome of specific radiation protocols
for different disease stages, but also, the literature does not
lend itself to direct comparison of outcomes after surgical
therapy. Nevertheless, in one of the largest series of patients
treated primarily with radiation therapy, Briant et al. (4) re-
ported initial control rates of 78% in 45 patients treated with
moderate-dose radiotherapy (3000–3500 rads). The follow-up
period ranged from 2 to 20 years, but 10 of these patients were
irradiated after surgical failure. This report was later updated
by Cummings et al. (7) in 55 patients (42 managed with
primary radiation and 13 treated with radiation therapy for
recurrent disease after surgical excision), with similar control
rates of 80% after initial therapy. More recently, Reddy et al.
(27) reported 15 patients with advanced-stage JNA treated
with radiation (6 primarily and 9 after surgical resection) and
reported an 85% rate of complete response. The total radiation
dose was 20 to 30 Gy. A 2.5-year minimum follow-up was
VOLUME 52 | NUMBER 5 | MAY 2003 | 1209
DARE ET AL.
reported. Using radiation therapy to manage recurrent dis-
ease, Kasper et al. (19) reported 100% local control of disease
in seven patients treated for recurrent advanced-stage disease
after primary resection. The follow-up period was 3 to 15
years, and 3000 cGy was used. Fields et al. (12) reported
results of megavolt radiation (3600–5200 cGy) in 13 patients.
Disease was controlled in 9 (82%) of 11 patients treated for
recurrent disease after primary surgical resection. The median
duration of follow-up was 136 months. Using radiation ther-
apy primarily to manage patients with intracranial extension
of disease (Stage IV), Economou et al. (10) reported asymp-
tomatic control in 10 (71%) of 14 patients. A 25-year experience
was reported; no follow-up period was mentioned. Wiatrak et
al. (30) reported symptomatic control of disease in a series of
three patients with intracranial extension of disease treated
with primary radiation (3660–5040 cGy), with a follow-up
period of 1.7 to 5 years. In summary, the current literature
would suggest that long-term control of recurrent and
advanced-stage disease may be achieved with radiation ther-
apy; however, a recurrence rate of 20 to 33% may be expected
from primary treatment with radiation alone (4, 7, 19, 27, 30).
The use of conventional radiotherapy in the management of
JNA in the adolescent has been criticized for its potential long-
term adverse effects. Cummings et al. (7) reported secondary
malignancies within the radiation portal in 2 of 55 patients
treated with primary radiation therapy; one patient developed
basal cell carcinoma of the face, and the second developed a
well-differentiated thyroid carcinoma. Reddy et al. (27) reported
basal cell carcinoma in 1 of 15 patients. Others have documented
malignant transformation of JNA after conventional radiother-
apy. Adverse reactions include cataracts and hypopituitarism (7,
27), glaucoma and optic atrophy (30), xerostomia, and caries (12).
Primary failure of conventional radiotherapy has been attributed
to inadequate dose or volume or geographic misses because of
the inability to deliver a highly conformal dose determined on
the basis of modern imaging modalities such as MRI (7, 10, 14,
24). With advances in radiation technology, conformal radiation
may be delivered to a desired target volume while treatment to
surrounding tissue is limited. Recent experience with intensity-
modulated radiotherapy, a form of conformal radiotherapy, has
been reported for the management of extensive and recurrent
JNA in three patients (20). The computer-assisted technology for
field placement used in intensity-modulated radiotherapy has
the potential to significantly reduce the chances for geographic
misses previously reported with conventional radiotherapy.
However, the long-term efficacy of intensity-modulated radio-
therapy for the management of JNA remains to be determined.
Recent reports have confirmed the safety of radiosurgery in
the management of pediatric head and neck tumors (3, 5).
Radiosurgery was performed in our patients in an attempt to
limit the amount of radiation to the surrounding normal brain.
A significant advantage of gamma knife radiosurgery is that it
is a single-session, 1-day treatment. This treatment was well
tolerated by the two patients presented here, and we have not
encountered any acute or delayed evidence of toxicity in either
patient. However, the follow-up period is too short to detect
1210 | VOLUME 52 | NUMBER 5 | MAY 2003
secondary malignancies, which are rarely described after ra-
diosurgery. The strategy of surgery followed by adjuvant
treatment with radiation for residual tumor in areas associated
with high morbidity of resection has been recommended pre-
viously, albeit with external-beam radiation therapy (9, 18, 21,
28). Surgery reduces the tumor to a size amenable to treatment
with gamma knife radiosurgery. Typically, JNA recurs within
6 to 36 months (mean, 17 mo) after primary therapy (23). We
have observed both patients for more than 24 months after
radiosurgery, with control of disease in one patient and radio-
graphic evidence of early tumor regression in the second.
Others have advocated the use of postoperative radiation
therapy for residual tumor only at the time of disease progres-
sion (11, 16). In the case of a delayed diagnosis of recurrence
or the loss of a patient to follow-up, a recurrent lesion may no
longer be treatable with gamma knife radiosurgery. This re-
port documents short-term control of postoperative residual
tumor in the infratemporal fossa and lateral cavernous sinus
after gamma knife radiosurgery in two patients. Long-term
control of advanced-stage JNA with this strategy and estab-
lishing the safety and effectiveness of gamma knife radiosur-
gery over conventional radiation in the management of ad-
vanced JNA will require further prospective studies.
ADDENDUM
During the period of review of this manuscript for publi-
cation, we have had the opportunity to evaluate both patients
at 36 months after treatment with radiosurgery, and we have
detected no clinical or imaging evidence of disease recurrence.
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COMMENTS
T he authors report two patients treated with resection fol-
lowed by gamma knife radiosurgery for juvenile nasopha-
ryngeal angiofibromas (JNAs). Many of these tumors can be
effectively treated with resection and radiation therapy. How-
ever, as the authors note, this is associated with the possibility
of delayed secondary tumor formation years after the fraction-
ated radiation regimen. In an attempt to restrict the radiation
dose precisely to the tumor, they performed radiosurgery. For
NEUROSURGERY
SURGERY AND RADIOSURGERY FOR ANGIOFIBROMA
a slow-growing, late-responding tissue such as an angiofi-
broma, there may be a radiobiological advantage to radiosur-
gery, as there is with arteriovenous malformations. Thus, ra-
diosurgery is a reasonable strategy in patients with small-
volume and localized nasopharyngeal angiofibromas. Other
patients who may have large, diffuse lesions for which radio-
surgery would not be appropriate may be treated effectively
with a larger-field fractionated approach. The length of
follow-up in this study (36 mo) is adequate to address neuro-
logical toxicity after radiosurgery. I think this is an interesting
way to treat such tumors and should be evaluated further.
Douglas Kondziolka
Pittsburgh, Pennsylvania
T his article details the use of gamma knife radiosurgery for
residual JNA after resection. JNA is a disease of the young.
Every attempt should be made at the first operation to achieve a
gross total resection. In our experience, for extensive JNAs as
described by the authors, a transfacial and subtemporal infra-
temporal approach is usually necessary. The residual disease
seen in the authors’ two patients could probably have been
addressed had the subtemporal infratemporal fossa view been
available during the surgery. The authors achieved a very good
resection in both of their patients. The small residuals appear to
be amenable to resection by the lateral approach. In our opinion,
this would be the management of choice rather than the use of
radiation. The primary management paradigm of gross total
resection may have to be altered in the face of recurrent disease,
although re-resection should be strongly considered. In patients
with recurrent unresectable disease, radiation therapy has been
shown to be a useful adjunct to therapy. Several reports have also
described tumor response to chemotherapy.
Franco DeMonte
Raymond Sawaya
Houston, Texas
G amma knife radiosurgery is a novel therapy for incom-
pletely resected JNA. One advantage of this approach is
that a more aggressive and potentially dangerous surgical resec-
tion may be avoided. Radiosurgery is an effective way to deliver
high-dose radiation to residual tumor while minimizing expo-
sure to surrounding healthy tissue. However, this series and
others do not have adequate follow-up (10–20 yr) to confirm the
long-term safety of using high-dose, single-fraction radiation in
pediatric patients. Larger-dose-per-fraction radiation is known to
increase the risk and severity of late effects such as cranial nerve
palsy, bone and soft tissue necrosis, and second malignancy,
especially in children. A potentially safer and equally effective
alternative may be the use of other highly conformal radiation
technologies with fractionation, such as fractionated stereotactic
radiosurgery and intensity-modulated radiation therapy.
Suzanne L. Wolden
Pediatric Radiation Oncologist
Philip H. Gutin
New York, New York
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