Jpn J Clin Oncol 2014;44(10)926 931

doi:10.1093/jjco/hyu100
Advance Access Publication 5 August 2014

Post-operative Radiotherapy for the Treatment of Malignant Solitary

Fibrous Tumor of the Nasal and Paranasal Area
Ying Xue, Guangjin Chai, Feng Xiao, Ning Wang, Yunfeng Mu, Yujie Wang and Mei Shi*

Department of Radiation Oncology, Xijing Hospital, The Fourth Military Medical University, Xian, China

*For reprints and all correspondence. Department of Radiation Oncology, Xijing Hospital, The Fourth Military Medical
University, Chang Le West Street, No. 17, 710032 Shaanxi, Xian, China. E-mail: meishi_xa@163.com

First author: Ying Xue, (1971.08.29), doctor, doctor-in-charge, mainly engaged in the comprehensive treatment of
chest tumor.
Received December 31, 2013; accepted July 8, 2014

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Objective: Solitary fibrous tumor is a rare tumor occurring in almost every anatomic location of
human body; however, reports of malignant solitary fibrous tumor in the nasal and paranasal
area are especially rare. In this report, we describe a case of non-recurrent malignant solitary
fibrous tumor of the nasal and paranasal area.
Methods: The patient was initially treated with nasal and paranasal tumor cytoreductive
surgery, followed by post-operative three-dimensional conformal intensity modulated radiation
therapy (dynamic MLC Varian 600CD Linac, inversely optimized by the Eclipse system) and
stereotactic body radiation therapy to provide a radical cure for residual tumor.
Results: The tumor of the nasal and paranasal area was effectively treated and the integrity of the
right eye kept. There were no signs of recurrence after four and a half years of further follow-up.
Conclusions: This is the first attempt to successfully combine cytoreductive surgery with inten-
sity modulated radiation therapy and stereotactic body radiation therapy together to treat solitary
fibrous tumor of the nasal and paranasal area, which may provide a potential strategy for the
treatment of similar cases.

Key words: solitary brous tumor radiotherapy

INTRODUCTION
Solitary brous tumor (SFT) is an uncommon spindle cell tumor
that was rst recognized and fully characterized in 1994 by
Westra et al. (1). SFT often originates from the pleura, and occa-
sionally from other parts of the body, including the extremities,
mediastinum, peritoneum, parotid gland and orbit (16). Radical
surgical resection is the preferred treatment for SFT. Here, we
present a case of malignant SFT of the nasal and paranasal area
that was treated by cytoreductive surgery combined with intensity
modulated radiation therapy (IMRT) and solitary brous tumor
(SBRT). No recurrence was seen in long-term follow-up.
CASE REPORT
An 18-year-old female was admitted to the Xijing Hospital of
Shaanxi Province, China in April due to painless proptosis,
decreased visual acuity and epiphora in the right eye. Prior to
the onset of symptoms, the uncorrected visual acuity was 4.3
and 4.8 in the right and left eyes, respectively. The patient had
no family history of similar symptoms.
An orbital computed tomography (CT) scan showed that
a large mass fully occupied the right nasal cavity, sinuses
and frontal sinuses (Fig. 1A and B). The mass impinged on
the right eye and the right maxillary sinus, and caused nasal
septum perforation with oppressing on the left ethmoidal
cellules (Fig. 1 A and B). In order to keep the integrity of
the right eye, nasal and paranasal tumor cytoreductive
surgery was performed to partially resect the paranasal
sinus tumor inside the right nasal cavity with image
navigation. During the operation, a wider range of tumor in-
vasion was observed, showing invasion of the ethmoid
bone roof, the sphenoid sinus anterior wall, nostrils and the
laminapapyrace.

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The tumor tissue was grayish-white, like sh with brittle
textures, covered with a brous bone shell. The post-operative
specimen was a dark brown nodular mass (9  6.5  5 cm)
with incomplete envelope. On the cut section, the tumor
was grayish-white, lobulated, rm and well-demarcated, with
a whorled and fasciculated surface. Light microscopic
examination of the resected tumor showed large numbers of
tumor cells that are oval and fusiform, distributed in patches
with signicant atypia by Hematoxylin and eosin staining.
The branched blood vessels, abnormal cells, mitotic
gures and nuclear polymorphisms were frequently noted
(Fig. 2A and B).
Due to the low incidence and histologic similarity to other
spindle cell tumors, early diagnosis of nasal and paranasal
SFT is difcult. The main clinical manifestation is pain-
less proptosis. The diagnosis is mainly dependent on
Figure 1. Orbital computed topographies scan of right orbit solitary brous
tumor (SFT). (A) The image shows a 5 cm-sized, well-demarcated nodule
with an eccentric nodular enhancement. (B) A coronal image shows a mass
lying in the orbit.
Figure 2. Hematoxylin and eosin-stained slide. (A) Microscopic examination of the orbit lesion shows proliferation of relatively uniform spindle cells that are
either patternless or have a focally storiform pattern. (B) Focal vascular dilatation with collagenized stroma, dense collagen nodules and perivascular hyalinization
is also observed.
Jpn J Clin Oncol 2014;44(10) 927
immunohistochemical studies. It has been documented that
SFT exhibits strong positivity with CD99 (70%), vimentin
(95%) and Bcl-2 (35%) antibodies, and is negative for S-100
(7). In addition, Ki-67 proteins are excellent markers for deter-
mining the so-called growth fraction of a given cell popula-
tion. In the present case, immunohistochemical staining
revealed that cells were positive for CD99 (Fig. 3A), Vim
(Fig. 3B) and Bcl-2 (Fig. 3C), and contained scattered
CD34-positive cells (Fig. 3D). Ki67-positive cells accounted
for almost 28% of all cells (Fig. 3E). All cells were negative
for S-100 (Fig. 3F). On the basis of the aforementioned
results, pathological evaluation ascertained the presence of a
malignant SFT with bone invasion.
Three weeks after surgery, magnetic resonance imaging
(MRI) imaging of the nasopharynx revealed an irregular, in-
homogeneous mass in the right nasal cavity and ethmoid sinus
(4.1  2.8 cm) (Fig. 4). A radiation treatment plan, three-
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dimensional conformal IMRT was developed for the patient
after CT-guided tumor localization. VRIAN 600CD was used
as the treatment machine. The Eclipse planning system was
LUNA TM 260. The gross tumor volume (GTV), including re-
sidual tumor, was visualized on CT and MRI. The clinical
tumor volume (CTV) was calculated including the right nasal
cavity, part of the left nasal cavity, right maxillary sinus, an-
terior and posterior ethmoid sinus, sphenoid sinus and part of
the nasopharyngeal mucosa. The planning target volume
(PTV) was 0.3 cm outside of the CTV. The total GTV irradi-
ation received was 60 Gy/24F, 2.5 Gy/F, one session/day and
ve sessions/week. The total PTV irradiation was 55.2 Gy/
24F, 2.3 Gy/F, one session/day, and ve sessions/week.
During the second treatment course, SBRT therapy was deliv-
ered targeting the GTV, with a total irradiation dose of 12 Gy/
3F, 4 Gy/F, one session/every other day. A 50% isodose line
surrounded the targeted region. Dose constraint for each organ
928 Radiotherapy for nasal and paranasal solitary brous tumor
Figure 3. Immunohistochemical test of the SFT. Immunohistochemical tests showed that the tumor was positive for CD99 (A), Vim (B) and Bcl-2 (C), and
contains scattered CD34-positive cells (D). Ki67-positive cells accounted for 28% of all cells (E). All cells are negative for S-100 (F).
Figure 4. Tumor image of MRI at 3 weeks after surgery.
at risk were as follows: brainstem, Dmax  54 Gy D1  60 Gy;
optic chiasma, Dmax  54 Gy D1  60 Gy; bilateral crystal,
Dmax  5 Gy D33  8 Gy; bilateral eye, Dmax  50 Gy; and
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the left optic nerve, Dmax  54 Gy D1  60 Gy. Besides, the
DVH for each organ were as follows: brainstem (Dmax 44.1 Gy;
D1 45.6 Gy), optic chiasma (Dmax 43.2 Gy; D1 44.1 Gy), bilat-
eral crystal (Dmax 5.1 Gy; D33 5.4 Gy), bilateral eye (Dmax
41.2 Gy) and the left optic nerve (Dmax 45.4 Gy; D1 46.5 Gy).
After radiation therapy, the patient complained of xerostomia,
ageusia and anosmia. Furthermore, there was no signicant
relief of right nasal cavity obstruction and binocular visual
acuity after treatment.
With reexamination for primary tumor 5 months after radio-
therapy, nasal congestion symptoms were abated in the right
nasal cavity, but still accompanied with the xerostomia,
ageusia and anosmia. The visual acuity of the right eye was
4.3, indicating no change compared with levels before radi-
ation therapy. MRI analysis showed an absence of the right
turbinate and the medial wall of the right maxillary sinus. The
lesions on the right nasal cavity and ethmoid sinus showed
hyper signals (2.9  3.4  2.1 cm) with dened border using
enhanced MRI (Fig. 5A1 and A2).
Eighteen months after radiation therapy, reexamination
showed that the xerostomia was signicantly ameliorated.
Symptoms of stuffy nose and headache had disappeared. The
 Jpn J Clin Oncol 2014;44(10) 929

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Figure 5. Follow-up reviews. The follow-up reviews after 5 months (A1, A2) and eighteen months (B1, B2) showed a reduction in the size of the mass, and four
and a half years after therapy, no change was seen in the size of the mass in the right nasal cavity (C1, C2).
930 Radiotherapy for nasal and paranasal solitary brous tumor
sense of taste and smell had almost returned to normal levels.
Visual acuity of the right eye was 4.3. MRI showed a reduc-
tion in size of the mass (2.5  3.1  1.6 cm) in the right nasal
cavity and ethmoid sinus (Fig. 5B1 and B2).
Four and a half years after radiation therapy, the symptoms
of xerostomia had completely disappeared. The left eye visual
acuity was stable (4.6) and the right eye visual acuity
decreased slightly (4.1). The senses of taste and smell were
normal. MRI showed no change in the size of the mass in the
right nasal cavity and ethmoid sinus (2.0  3.1  1.5 cm). No
local recurrences were observed (Fig. 5C1 and C2).
DISCUSSION
SFT is a rare soft tissue spindle cell tumor. It can occur in
every site of the body, including the mediastinum, lung,
pleura, liver, kidney, orbit and meninges (3, 8, 9). The age of
onset is mainly between 40 and 70 years. There was no signi-
cant difference between males and females (9, 10). Most SFT
cases are benign neoplasms; however, 10 15% are malig-
nant neoplasms, especially those in mediastinal, abdominal,
pelvic and retroperitoneal locations (6, 11). Metastases may
occur in the lungs, bones and liver. Malignancy is dened as a
signicantly increased tumor cell density, clear cell atypia,
more than four mitotic gures in every 10 HPF, and the pres-
ence of necrosis (6, 11 13).
SFT of the nasal and paranasal area was rst reported in
1994 (14) and most SFT are benign (15, 16). Inltrating
growth is not a common feature of pathology, and only a few
cases reveal malignant transformation. SFT displays high
CD34 reactivity in 79 100% of cases (14). On the contrary,
malignant SFT may show high mitotic counts and loss of
CD34 immunoreactivity (17, 18). Therefore, CD34 is used to
distinguish benign and malignant SFT. Most samples in this
case report showed immunohistochemistry results that were
negative for CD34, resulting in the diagnosis of a possible ma-
lignant entity according to the benign malignant system.
Combined with the appearance of branched blood vessels,
abnormity cells, mitotic gures and nuclear polymorphisms
under the light microscope, it is reasonable to judge this case
to be a malignant lesion.
SFT of the nasal and paranasal area is a rare neoplasm, and
there are no standard clinical treatment guidelines. Complete
surgical removal is the main treatment strategy, but some
drawbacks still exist in the clinical application in SFT of the
nasal and paranasal area. Complete resection is often restricted
due to the small volume of the nasal and paranasal tissue, the
presence of vital organs, large tumor volumes, a lack of com-
plete encapsulation and invasive growth. Recently, tumor
cytoreduction combined with post-operative adjuvant therapy
has been considered more advantageous in SFT from nasal
and paranasal area. Stereotactic radiation therapy is often used
as post-operative therapy, and has been reported to effectively
control residual tumors (19, 20). However, radiation therapy
can damage the optic nerve, optic chiasm, cornea and lens,
resulting in decreased visual acuity or visual eld defects.
Consequently, post-operative radiation therapy may need to be
improved for the treatment of SFT in the head.
IMRT is known to enable the delivery of lower doses of ra-
diation to normal tissue, while increasing or maintaining the
tumor dose, which exhibits more advantages than two-
dimensional radiotherapy (2DRT) or three-dimensional con-
formational radiotherapy (3DCRT). The potential of IMRT
for sparing organs has been demonstrated in patients with
mixed head and neck tumors (21) and nasopharyngeal cancers
(22). In this study, the patient had a large localized nasal and
paranasal tumor with diffuse local extension. To preserve the
right eye, as required by the patient, the tumor was partially
resected. In order to better control the primary tumor and
avoid recurrence and metastases, a post-operative IMRT was
used to eliminate residual tumors. In this patient, IMRT was
well used and the healthy tissues of the left side received non-
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toxic doses of radiation, which preserved the visual acuity and
visual eld of the patients left eye. In the subsequent follow-
up, the vision of the patient was maintained at a stable level
after treatment, conrming that our strategy was effective and
can be referenced. However, the eye, lens and optic nerve of
the right eye were closely related to the tumor, so IMRT could
not be strictly targeted.
SBRT is a relatively new type of radiosurgery that is
capable of the precise delivery of converging beams of radi-
ation on a small target in almost any location in the body (23,
24). Studies have shown that SBRT after external irradiation
improves the local control and survival rates in nasopharyn-
geal cancer. SBRT could effectively enhance the protection
of healthy tissues, which is important in the treatment of
head and neck cancers (25, 26). In this case, SBRT was used
in combination with IMRT in the residual tumor to remedy
the deciency of IMRT and protect contralateral healthy
organs and reduce the acute and late-stage side effects of
radiation. In the subsequent follow-up, the right nasal cavity
and paranasal sinus tumors progressively decreased in size.
The patient experienced no obvious decline in visual acuity
or visual eld defects, even though the maximum dose deliv-
ered to the right optic nerve and right eye were up to 64 and
62 Gy, respectively. The symptoms of nasal obstruction, xer-
ostomia, ageusia and anosmia disappeared gradually during
follow-up.
CONCLUSION
In this report, we described a case of malignant SFT originat-
ing from the nasal and paranasal area. Following cytoreduc-
tive surgery, two post-operative radiation therapies of IMRT
and SBRT were performed; no recurrences or metastases oc-
curred during long-term follow-up. Therefore, this report indi-
cates an effective strategy for the treatment of malignant SFT
of the nasal and paranasal area. More research and longer
follow-up should be explored in the future based on the
recommended strategy.

Conict of interest statement
None declared.
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