-
Central Annals of Otolaryngology and Rhinology
Cite this article: Black K, Naseri I, Aldana P, Goldstein J,
Josephson GD (2015) Juvenile Ossifying Fibroma: Successful
Endoscopic Gross Total Resection of a Rare Sinonasal Tumor in an
Adolescent Male Ann Otolaryngol Rhinol 2(3): 1026.
*Corresponding authorGary Josephson, MD, Nemours Children’s
Clinic, 807 Children’s Way, Jacksonville, Florida 32207, USA, Tel:
904-226-1231; Email:
Submitted: 21 February 2015
Accepted: 24 March 2015
Published: 26 March 2015
Copyright© 2015 Josephson et al.
OPEN ACCESS
Keywords•Endoscopic•Rhinology•Skull base•Tumor•Surgery
Case Report
Juvenile Ossifying Fibroma: Successful Endoscopic Gross Total
Resection of a Rare Sinonasal Tumor in an Adolescent MaleKaelan
Black1, Iman Naseri2, Philipp Aldana3, Jeffrey Goldstein4 and Gary
D. Josephson5*1Department of Otolaryngology-Head and Neck Surgery,
University of South Florida, USA2Department of Otolaryngology-Head
and Neck Surgery, Baptist Health, USA3Division of Pediatric
Neurosurgery, University of Florida, USA4Department of Pathology,
Wolfson Children’s Hospital, USA5Division of Pediatric
Otolaryngology-Head and Neck Surgery, Nemours Children’s Clinic,
USA
Abstract
Juvenile ossifying fibroma is a rare tumor of the sinonasal
cavity in pediatric patients. Large tumors involving the orbit and
cranial fossa have traditionally been resected using an open
craniofacial approach, with an open transcranial and endonasal
endoscopic approach for smaller tumors. We describe a case in which
visualization with straight and angled telescopes and endoscopic
instrumentation allowed high confidence resulting in a gross total
resection of this very large tumor. We believe this approach by an
experienced endoscopist can offer equal success in treatment
outcome with lower morbidity and quicker recovery than the
traditional open procedures for this tumor.
ABBREVIATIONSJOF: Juvenile Ossifying Fibroma; OF: Ossifying
Fibromas;
JPOF: Juvenile Psammomatous Ossifying Fibroma; JTOF: Juvenile
Trabecular Ossifying Fibroma
INTRODUCTIONOssifying fibromas, first described in 1872 by
Menzel [1],
are rare benign lesions commonly found in the tibia and fibula
in children. However, these tumors have also been described in the
head and neck region. Most are found in the mandible (62-89%) but
lesions in the maxilla, the orbit, the skull base, and the
calvarium have also been reported [1].
Ossifying Fibromas in the sinonasal tract tend to occur in older
patients in the third or fourth decades and are more prevalent in
women than men [1]. They are usually characterized as slow growing
painless tumors. In contrast, Juvenile Ossifying Fibroma (JOF)
occurs in a much younger patient population. Although benign, these
tumors are locally aggressive and can cause bony and soft tissue
destruction and impingement on the surrounding organs such as the
eyes and brain. Presenting symptoms include
those related to mass effect, mainly pain, pressure, changes in
vision, and headache depending on the size and location of the
lesion.
Typically, a combined transcranial and transfacial approach for
removal and repair has been used especially for very large tumors.
With the advent of angled telescopes and more advanced equipment to
successfully remove large skull base tumors, extended endoscopic
approaches have become increasingly popular. We present an
adolescent male with a large Juvenile Ossifying Fibroma of the
nasal cavity extending superiorly into the anterior cranial fossa,
and lateral into the orbit. A transnasal endoscopic approach
allowed gross total resection of this tumor. Reconstruction with a
nasal-septal flap was successful to close the skull base defect. We
believe this to be the first case of such a large tumor in a
pediatric patient successfully removed endoscopically. The child
remains disease free 18 months since his resection.
CASE PRESENTATIONA 14-year-old white male was referred to the
Pediatric
Otolaryngology clinic for evaluation of nasal congestion
worse
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Josephson et al. (2015)Email:
Ann Otolaryngol Rhinol 2(3): 1026 (2015) 2/4
on the right for several years. Previous skin testing by the
allergy clinic revealed response to trees, grasses, weeds, and
molds. . Fluticasone and Olopatadine nasal sprays were used with no
response for six months. Otolaryngology evaluation on presentation
noted mild right proptosis. Nasal endoscopy identified a mass in
the right nasal cavity. CT scan with contrast was performed (Figure
1) followed by an MRI to further delineate the extent of the
intracranial disease. Operative nasal endoscopy with biopsy
provided the diagnosis of Juvenile Ossifying Fibroma (Figure 2).
The child returned to the operative theater three weeks later for
definitive surgical extirpation. Endoscopic endonasal resection of
the tumor included ipsilateral middle turbinate resection, total
ethmoidectomy, orbital decompression, maxillary antrostomy,
sphenoidotomy, and frontal sinusotomy, all together allowing
appropriate instrument access for removal of the tumor from the
anterior cranial fossa. The defect was successfully closed with a
nasal-septal flap based on the posterior septonasal branch of the
sphenopalatine artery. The child was admitted to the neurosurgical
service in the Pediatric ICU. He was discharged on Post-operative
day seven. The child remains disease free for eighteen months as
followed by office nasal endoscopy and follow up CT scan (Figure
3).
SURGICAL PROCEDUREPreoperative embolization was performed 24
hours prior
to the surgical procedure by the Interventional Neuroradiology
Division. The following day the child was brought to the operating
theater and general anesthesia was provided. The Medtronic Fusion®
image guidance system was registered and used throughout the
procedure to verify the tumor confines. Endonasal endoscopic
approach was performed using straight and angled rigid telescopes.
A large portion of the tumor was within the nasal cavity, with mass
effect into the ipsi lateral paranasal sinuses. The tumor surface
was smooth and mucosalized. It extended into the anterior cranial
fossa medially and superiorly, and also extended laterally
displacing the right orbit.
The intranasal component of the tumor was removed with the
Arthrocare® Coblation device with a sino-nasal handpiece (Procise
EZ View Plasma wand™ EICA 8875-01) set on coagulation 9, and
ablation 5 (Figure 4). This allowed for tumor debulking while
maintaining adequate hemostasis.
Full access to the tumor confines for visualization and
instrumentation required sinusotomies and resection of the middle
and superior turbinates. The uncinate process was identified and
removed and a large maxillary antrostomy was performed. A total
ethmoidectomy was performed to allow both medial and lateral tumor
access. The lateral aspect of the tumor displaced the medial
orbital wall laterally. Tumor was removed laterally to include
removal of the lamina papyracea while leaving the periorbita
intact. This cleared the lateral extent of the tumor margin.
Posteriorly, a large sphenoidotomy was required to clear
Figure 1 Coronal CT scan with contrast of nasal tumor extending
lateral into orbit and superior into anterior cranial fossa.
Figure 2 A haphazard arrangement of cytologically benign,
spindled fibroblasts surrounds small, round and irregular shaped,
psamommatoid ossicles composed of a cellular osteoid matrix (pink)
with calcified centers (purple).
Figure 3 Eighteen month follow up coronal CT scan showing no
evidence of recurrent disease.
Figure 4 Endoscopic view of tumor in the right nasal cavity
after partial removal, showing tumor mesenchymal contents and
vascularity.
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access to the posterior tumor margin. Superiorly, the frontal
sinus ostium was identified and enlarged. The superior aspect of
the tumor extended into the anterior cranial fossa and was removed
off the dura with neurosurgical curettes and dissectors (Figure 5).
Different angled telescopes allowed visualization during dissection
and removal of this tumor that extended across the midline in the
anterior cranial fossa, along the olfactory cleft and the
cribriform plate. The superior dome cortex of the tumor formed a
thin shell, being the superior margin of the tumor, which was
fairly adherent to the overlying dural. This required meticulous
dissection in order to minimize the extent of dural defect. Several
small durotomies were required to remove the entire adherent tumor,
assuring a gross total resection (Figure 6). The final dural defect
was less than one centimeter in greatest dimension, and was
repaired using a single layer onlay reconstruction using a
vascularized naso-septal flap based on the posterior nasoseptal
vascular bundle of the sphenopalatine artery (Figure 7).
DISCUSSIONOssifying Fibromas (OF) are rare benign lesions of
the
craniofacial skeleton, usually found in patients twenty to
thirty years old. They appear to affect women more than men1. The
cause of these tumors is currently unknown however theories include
derivatives of periodontal ligaments, trauma, or developmental
causes [1]. These neoplasms replace the normal bone of the face
with fibrous cellular stroma that usually presents as a large
mass with deficits related to mass effect on surrounding
structures.
Juvenile Ossifying Fibromas (JOF), a variant of this disease,
occurs in patients of a much younger age [2]. JOF is split into two
different groups: Juvenile Psammomatous Ossifying Fibroma (JPOF)
and Juvenile Trabecular Ossifying Fibroma (JTOF) [2]. Differential
diagnosis includes fibrous dysplasia, sinonasal Psammomatous
meningioma, and osteosarcoma [1]. The patient in this case was
found to have JPOF which will be the focus of this discussion.
JPOF was first described by Benjamins in 1938 [3]. Most are
diagnosed before the age of 15 and has a slight male predominance
[3]. JPOF usually occurs in the orbit and paranasal sinuses but has
also been found in the mandible, maxilla, and calvarium. JTOF, on
the other hand, is usually found on the maxilla or mandible.
Symptoms of JPOF are mainly caused by mass effect and include
facial pain, facial pressure, sinusitis, nasal congestion,
displacement of the orbit, blurry vision, headaches, proptosis, and
facial swelling [2]. The lesions are slow growing but can be
locally aggressive and destructive.
On imaging, JPOF is a well-circumscribed lesion with varying
degrees of radiolucency and radiodensity [2]. These lesions are
usually very well demarcated from the surrounding bone with a lytic
border [2]. Depending on the size and the aggressiveness of the
lesion, bony destruction can be found and extension into the
intracranial space, although rare, has been described. On MRI, the
lesions are hypointense on T1 and T2, however they enhance with
gadolinium [4].
Histology of the JPOF show small masses of uniform calcified
osteoid bodies that classified as psammomatoid found in a
fibroblastic stroma with spindle cells [5]. These psamommatoid
particles have a central basophilic area with an eosiniphillic
border. Osteoclastic giant cells are also a common finding in these
tumors [6]. Aneurysmal bone cysts are a common feature of JPOF and
can distinguish these tumors from other types of fibrous anomalies
[5]. JTOF, for instance, is also composed of fibroblastic spindle
cell stroma with multiple multinucleated giant cells. Unlike JPOF,
however, cystic structures are rare in this type of lesion [7].
Total surgical resection at the earliest stage is the treatment
of choice in patients with JPOF. This can be done in an
endoscopic
Figure 5 Tumor completely removed laterally from the right
periorbital. Instrumentation depicting tumor excision along the
anterior skull base. Adherent tumor along olfactory region
requiring meticulous dissection to free tumor from dura.
Figure 6 Anterior cranial fossa dural defect after complete
removal of tumor from this region.
Figure 7 Nasal Septal Flap used to close skull base defect.
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Ann Otolaryngol Rhinol 2(3): 1026 (2015) 4/4
Black K, Naseri I, Aldana P, Goldstein J, Josephson GD (2015)
Juvenile Ossifying Fibroma: Successful Endoscopic Gross Total
Resection of a Rare Sinonasal Tumor in an Adolescent Male Ann
Otolaryngol Rhinol 2(3): 1026.
Cite this article
or an open approach. Smaller tumors are usually easily resected
by an endoscopic approach. Larger tumors, however, that involve
multiple sinuses are commonly approached in an open manner. The
extent and location of the tumor will determine the approach in
these tumors.
Transfacial or cranial approaches have been used for open
resection. These include the transfrontal nasal approach and the
transfrontal nasoorbital approaches [8]. Advantages of open
resection include large exposure of the lesion for careful
dissection [9]. These methods, however, are extensive surgeries
that can cause external scarring, long hospital stays and recovery
times for young patients. They require coordination amongst
multiple surgeons and disciplines including Otolaryngologists and
Neurosurgeons. Endoscopic approaches have been described in small
tumors but not in tumors involving the anterior cranial fossa and
orbit. This case demonstrates the ability to resect this tumor with
careful dissection using endoscopic methods only.
Recurrence of JPOF has been reported as high as 30-56% [8] and
this is likely due to tumor that is inadequately removed.
Intracranial extension is rare but few cases are reported. These
lesions may require larger resections [7]. After total resection,
the patient must have close follow up with endoscopic and imaging
exams to evaluate for recurrence. Radiation therapy is not
indicated in juvenile ossifying fibromas as it may increase
malignant transformation from 0.4% to 40% [1].
Juvenile ossifying fibroma is a rare tumor of the sinonasal
cavity and even more unusual in the pediatric age group. Large
tumors involving the orbit and cranial fossa have traditionally
been resected using an open transnasal/ transcranial approach, with
an open transcranial and endonasal endoscopic approach for smaller
tumors. We describe a case in which visualization with straight and
angled telescopes and endoscopic instrumentation allowed high
confidence resulting in a gross total resection of this very large
tumor. We believe this approach by an experienced
endoscopist can offer equal success in treatment outcome with
lower morbidity and quicker recovery than the traditional
approaches for this tumor. Our case report appears to be the first
report of such a large tumor with intracranial and orbital
involvement in a pediatric patient removed completely through a
transnasal endoscopic approach. The child remains disease free
after eighteen months.
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Juvenile Ossifying Fibroma: Successful Endoscopic Gross Total
Resection of a Rare Sinonasal Tumor
inAbstractAbbreviationsIntroductionCase PresentationSurgical
ProcedureDiscussionReferencesFigure 1Figure 2Figure 3Figure 4Figure
5Figure 6Figure 7