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Juvenile psammomatoid ossifying fibroma of the orbit and paranasal sinuses.

A case report

Article  in  Acta Neurochirurgica · August 2011

DOI: 10.1007/s00701-011-1115-1 · Source: PubMed

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Juvenile psammomatoid ossifying fibroma
of the orbit and paranasal sinuses. A case
report

Paulo Linhares, Eugénia Pires, Bruno

Carvalho & Rui Vaz

Acta Neurochirurgica
The European Journal of
Neurosurgery

ISSN 0001-6268

Acta Neurochir
DOI 10.1007/s00701-011-1115-1

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 Author's personal copy
Acta Neurochir
DOI 10.1007/s00701-011-1115-1
CASE REPORT
Juvenile psammomatoid ossifying fibroma of the orbit
and paranasal sinuses. A case report
Paulo Linhares & Eugénia Pires & Bruno Carvalho &
Rui Vaz
Received: 12 April 2011 / Accepted: 20 July 2011
# Springer-Verlag 2011
Abstract Juvenile psammomatoid ossifying fibroma
(JPOF) is an uncommon benign fibro-osseous lesion
predominantly arising in the paranasal sinuses and
orbits of children and young adults. We report a case
of JPOF involving the paranasal sinuses and orbit in a
15-year-old boy that presented due to progressive
proptosis and downward displacement of the left eye.
The lesion, first described as fibrous dysplasia (FD),
was totally removed surgically, and then proved to be a
JPOF, by histopathology. We discuss its differential
diagnosis with other fibro-osseous lesions, histopathological
features, and treatment options.
Keywords Juvenile psammomatoid ossifying fibroma .
Fibro-osseous lesions . Paranasal sinus neoplasms .
Fibrous dysplasia . Children
Introduction
Ossifying fibromas (OF) of the craniofacial skeleton are
benign fibro-osseous neoplasms characterized by the
Prior publication/presentation: This work or part of it has never been
published or presented in any other journal or at a conference or
seminar.
P. Linhares : B. Carvalho (*) : R. Vaz
Department of Neurosurgery, Hospital de São João,
Alameda Prof. Hernâni Monteiro,
4200–319 Porto, Portugal
e-mail: bmfcarvalho@gmail.com
E. Pires
Department of Anesthesiology, Hospital de São João,
Alameda Prof. Hernâni Monteiro,
4200–319 Porto, Portugal
replacement of normal bone by a fibrous cellular stroma
containing foci of mineralized bone trabeculae and
cementum-like material that vary in amount and appearance
[4, 5, 10, 16, 22, 28]. According to their pattern of
mineralization, three overlapping clinicopathological
entities have been identified: juvenile psammomatous
ossifying fibroma (JPOF), juvenile trabecular ossifying
fibroma (JTOF), and cemento-ossifying fibroma (COF).
The juvenile variants of ossifying fibromas share many
similarities, but they have been distinguished on the
basis of their histopathological features, site, and age of
recurrence [4–6, 13, 16, 21, 22, 25, 28]. Their location is
also different: JPOF arises mainly around paranasal
sinuses and orbits, whereas JTOF usually affects the
maxilla. The last entity, COF, is an odontogenic neoplasm
arising from the periodontal ligament and affects the
tooth-bearing areas of the jaws, mandible, and the maxilla;
the cementicles are the characteristic feature instead of the
bone elements; the highest incidence occurs between the
ages of 20 and 40 years [4, 8, 9, 12, 14, 16, 19].
Notwithstanding these entities, it must be emphasized
the contrast of JPOF with the much more common fibrous
dysplasia (FD), a developmental hamartomatous fibro-osseous
lesion, from which the differential is difficult based solely on
clinical or radiographic criteria [4, 6, 16].
The definite diagnosis of OF requires correlation of
the histopathologic features with the patient history,
clinical findings, radiographic/imaging analysis, and
operative findings because of the huge similarities
among the diverse group of diseases with fibrous and
osseous components [4, 10].
It is the purpose of this paper to report a case of
JPOF, in a 15-year-old boy in which the first diagnosis
was compatible with fibrous dysplasia (FD) based on
clinical and radiographic findings. The definite diagno-
 Author's personal copy
sis of JPOF was made after surgical resection of the
lesion based on histological criteria. We will review and
compare the clinical, radiological, and histopathological
features of these two entities.
Case report
History and examination A previously healthy 15-year-old
boy presented to our department with a 3-year history of
proptosis and downward displacement of the left eye
without any associated pain or visual loss. The neurological
examination was unremarkable. The family history was
irrelevant. The CT scan and the MRI were consistent with
the diagnosis of FD so the patient maintained clinical and
imagiological surveillance (Figs. 1, 2). Over a 3-year
period, there was progressive worsening of proptosis and
eye displacement and the patient underwent surgical
removal of the lesion.
The patient underwent a bicoronal approach. After the
scalp incision, care was taken to preserve the periosteum
over the frontal cranium. A left frontal craniotomy was
done. A small incision in the dura mater and arachnoid was
done for drainage of cerebrospinal fluid allowing a safe
retraction of the left frontal lobe to expose the orbital
roof. This was removed, exposing a huge cystic lesion
involving the periorbit. Since the lesion was completely
Fig. 1 Pre-operative gadolinium-enhanced axial T1-weighted (a) and T2-weighted (b) magnetic resonance imaging (MRI) showing an expansive
cystic lesion invading the left periorbit and ethmoid sinus
Acta Neurochir
extradural, we were able to preserve dura mater integrity
by careful dissection from the skull base and the remnant
thin bone without dural tear or CSF leak. The orbit walls,
except for a small portion of the orbital floor, were also
removed to prevent regrowth. The orbital bar was not
removed. The roof of the orbit was reconstructed with a
titanium plate and covered with pericranium and fibrin
glue. Two years later, there is no evidence of regrowth of
the lesion (Fig. 3).
Pathological examination
Macroscopic examination Two samples were available. The
first sample showed a nodular patch partially covered by
membranous white tissue. Other areas were red, with clots
and blood spaces congested. The tissue was friable. Some
bone odds and ends were identified in the middle portion of
the lesion. The second sample had many patches of white
and brown tissue, some with membranous aspect, with
lacerated cavity and hematic content.
Microscopic examination The pathological exam was
consistent with a benign fibro-osseous lesion, known as
juvenile psammomatous ossifying fibroma (JPOF). The
osseous component was predominantly made by eosinophilic,
spherical, and calcified structures. The fibrous component was
densely cellular, without atypia and with a very low mitotic
 Author's personal copy
Acta Neurochir

Fig. 2 Pre-operative axial CT scan showing a fibrous-osseous lesion of the left orbit and ethmoid sinus

Fig. 3 Post-operative gadolinium-enhanced axial T1-weighted (a) and T2 – weighted (b) magnetic resonance imaging (MRI) at 2-year follow-up
demonstrating total resection with no evidence of regrowth
Table 1 Literature-reported cases of JPOF/OF with intracranial extension [1–3, 6, 7, 11, 14–18, 20, 21, 23, 24, 26, 27]

Author Year Journal Age/Sex Clinical features Extension Approach Resection

Scott 1971 J Neurosurg 22/F Headache and anosmia Orbits, ethmoids, upper nasal cavity, Bifrontal craniotomy Subtotal
and the right maxillary antrum
Tomita 1981 Acta Neurochir 12/M Tumor recurrence Paranasal sinuses and intracranially Craniofacial approach Total
through the cribriform plate
Villemure 1983 J Neurosurg 22/M Swelling, headaches, Left parietal bone mass extending Bilateral craniotomy + Total
blurring of vision across the midline with autogenous split-rib
intracranial extension cranioplasty
Shields 1985 Br J Ophthalmol 14/F Painless, proptosis and upward Maxillary sinus with extension Lynch incision Total
displacement of the left eye into the sphenoid sinus,
ethmoid sinus, and orbit
Nakagawa 1995 Neurosurgery 29/M Diplopia, exophthalmos Paranasal sinuses, orbit, and Bicoronal approach Total
anterior cranial fossa
Tobey 1996 Am J Roentgenol 51/M Tension Left maxillary sinus, ethmoid Bifrontal craniotomy Subtotal
pneumocephalus sinus, left orbit, planum sphenoidale
into the anterior cranial fossa
Saito 1998 J Neurosurg 15/M Meningitis and deterioration Skull base - the planum sphenoidale, Bifrontal craniotomy with Total
of left visual acuity sella turcica, upper clivus, and right temporal extension
right middle cranial base
El-Mofty 2002 Oral Surg Oral Med Oral Pathol 53/F Nasal obstruction Left ethmoid sinus Biopsy Biopsy
Oral Radiol Endod
Vandekerckhove 2003 Bull. Soc. belge Ophtalmol 16/F Displacement of the left globe Invading through lamina Not described Total
papyracea and orbit
Baumann 2005 Otolaryngol Head Neck Surg 41/F Facial pain, swelling, and Ethmoid, frontal sinus, orbit, Transethmoidal via a Total
exophthalmos and the skull base Killian incision
Hasselblatt 2005 J. Neurosurg 15/M Incidental Ethmoid and sphenoid sinuses, Lateral rhinotomy and a Total
anterior cranial fossa bifrontal craniotomy
2005 J. Neurosurg 23/M Swelling Temporal bone mass infiltrating Temporal craniotomy Total
dura-mater
Bannykh 2007 J Neurooncol 26/F Diplopia, vertigo, proptosis Right sphenoid sinus and Trans-sphenoidal biopsy Biopsy
Author's personal copy

orbit, anterior cranial fossa

Gezici 2008 Neurol Neurochir Pol 18/M Headache and deterioration Sphenoid body, greater and lesser Bifrontal craniotomy Subtotal
of left visual acuity wings extended to the right
fronto-basal region
Nasser 2009 Childs Nerv Syst 12/M Left eye proptosis POF and aneurysmal bone cyst Left frontal craniotomy Total
(ABC) of the frontal sinus
with expansion into the ethmoid
and orbital cavities
Noudel 2009 Childs Nerv Syst 12/M Epilepsy, airway obstruction, Right maxilla, orbit, and Bifrontal craniotomy Total
proptosis anterior skull base
Nwizu 2010 N A J Med Sci 26/F Headache Spheno-ethmoidal mass involving Endoscopic resections Subtotal
the tuberculum sellae, left anterior
clinoid process and eroding
anterior cranial fossa
Bohn 2011 Head and Neck Pathol 15/M Proptosis and headache Nasal cavity and the anterior Bifrontal craniotomy Subtotal
cranial fossa and transnasal approach
Acta Neurochir
 Author's personal copy
Acta Neurochir
rate. There were no necrotic areas. There were also foci of old
hemorrhage and extensive areas of cavitary evolution. Bone
trabeculae covered by respiratory epithelium were identified.
Discussion
The commonest fibrous-osseous lesions of the orbit and
sinonasal tract are OF (variant JPOF) and FD. The
diagnosis between these two entities may be challenging,
as stated in our case report, because they share similar
features. There are four main clinical subtypes of FD:
monostotic (affects one bone, and accounts for 85% cases
of FD), polyostotic (affects multiple bones), McCune–
Albright syndrome in which multiple disseminated lesions
of bone are accompanied by skin hyperpigmentation and
endocrine disturbances; and a form that is confined to
bones of the craniofacial complex [4, 22].
JPOF has been distinguished because of its location,
clinical behavior, and age of occurrence. It typically arises
in the orbit and paranasal sinuses, accounting for over 72%
of reported cases [4, 12], followed by the calvarium (11%),
maxilla (10%), and mandible (7%), and affects mainly the
young, predominantly before the age of 15 [6, 16].
Symptoms include bulbar displacement, proptosis, impaired
vision, facial swelling, nasal obstruction, periorbital pain,
headache, and sinusitis. The lesions most often display a
steady progressive growth, but they can rapidly grow and
become locally aggressive, maybe because of a younger
age of occurrence and the concurrent development of
aneurysmal bone cysts [4, 6, 14, 28]. This was the case of
our patient. Clinically, craniofacial FD presents as a
painless enlargement of the affected bone, most often
during the first and second decades of life. Table 1
describes literature-reported cases of JPOF, and previously
undifferentiated OF, with intracranial extension.
Radiographically, the JPOF is usually well delineated at
the periphery with varying degrees of radiolucency and
radiopacity [4]. Lesions that are more aggressive may show
cortical thinning and perforation. Being well demarcated
distinguishes it from other fibro-osseous lesions such as
FD. Sometimes JPOF has a “ground glass” appearance [6,
16, 28]. The radiographic appearance of the FD reflects its
histological features: ground-glass opacity without defined
borders, in contrast to JPOF that is usually separated from
the surrounding bone by a lytic border. Enlargement of cranio-
facial FD occurs during active skeletal growth and ceases with
skeletal maturation. However, periods of regrowth may be
experienced in adulthood for unknown reasons [4, 12].
Microscopically, the JPOF is composed of a cell-rich
fibrous stroma that may have a whorled appearance.
Variable numbers of relative acellular rounded calcifications
resembling psammoma bodies are embedded in stroma. In
some tumors these round ossicles (cementicles) may be
very closely packed with little intervening stroma. An
eosinophilic rim is present at the periphery of most of the
ossicles. In some areas, the ossicles may connect to form
trabecular structures. Some lesions may also contain
acellular mineralized deposits with a bizarre shape, seen
most often in the myxoid portion of the tumor. This myxoid
area is considered to be an integral part of the tumor and
may undergo cystic change with edema, hemorrhage, and
clusters of multinucleated giant cells; these areas bear some
resemblance to aneurysmal bone cyst. Mitotic figures may
be seen, but are never prominent [12, 16]. In fact, the
microscopic examination of the lesion of our case is similar
to the description above and the final result was consistent
with JPOF. Histologically, FD is a benign, nonneoplastic,
intramedullary, cellular proliferation of fibroblasts, with
formation of irregular trabeculae of bone or ovoid calcifica-
tions that show indistinct, nonencapsulated borders; residing
in a cellular, fibrous connective tissue stroma, these delicate
trabeculae lack osteoid rims and have minimal to no
osteoblastic rimming [2, 4, 10, 12, 25].
Differential diagnosis between these benign fibrous
osseous lesions may be very difficult because there are no
definite established diagnostic criteria. As seen in our case
report, the first diagnosis, based on clinical and radiologic
criteria, was FD. However, after surgery, the definite
diagnosis of JPOF was made based on the distinctive
histological picture of this entity.
Complete excision of a JPOF lesion is the treatment of
choice and is usually curative. Despite its benign features,
JPOF can be locally invasive, causing significant morbidity,
and fatal consequences may be induced by intracranial
extension [2, 3, 5, 10, 14, 21, 22]. However, a surgical
approach is dictated more by anatomic location and tumor size
than by histologic subtype. In our case, the location of the
lesion at the orbit and the progressive enlargement over
3 years, were determinant in deciding a surgical approach
even before the histological diagnosis. Radiotherapy is
contraindicated because of the risk of malignant transforma-
tion and the potentially harmful late effects in children [14,
16]. Recurrence after resection is reported to range from 30
to 56% and is likely to be caused by incomplete excision
resulting from the infiltrative nature of the tumor borders
more than to any intrinsic biological properties [4, 12, 16].
Conflicts of interest None.
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