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Nature of Head & Neck Tumours

Case report

Juvenile psammomatoid ossifying fibroma of the paranasal

sinuses: A report of two cases
JN Bharti1*, B Dey1, P Gautam1, S Kaushal1

Abstract trabecular (TJOF) variants.2,3 Distinctive features of PJOF

Introduction
Juvenile psammomatoid ossifying fibroma (JPOF) is
characterized by a fibroblastic stroma containing small
ossicles resembling psammoma bodies. The stroma varies
from being loose and fibro-blastic to intensely cellular with
minimal intervening collagen. These ossicles should not be
confused with the cementum- like deposits that are
present in conventional OF. We report two cases in female,
include predilection for the sinonasal complex and orbit in
young people, an aggressive infiltrative growth pattern and
propensity for recurrence.4 We report two cases of PJOF
involving the paranasal sinuses.
Case report
Case 1
A 15 year old female presented with a complaint of

first case of fronto-ethmiod sinus and second one of
maxillary sinus.
Case Report
Case 1
A 15 year old female presented with 5.5 x 5 cm in sized,
hard forehead swelling extended to bridge of nose. MRI of
the paranasal sinuses revealed a well-defined,
spherical expansile lesion in the frontal bone.
Case 2
All authors contributed to conception and design, manuscript preparation, read and approved the final manuscript.
forehead swelling for three years. On examination, the
swelling was 5.5x5 cm in size, hard in consistency and
extended till the bridge of the nose. MRI PNS revealed a
well-defined expansile radiolucent lesion in the frontal
bone involving both the frontal sinuses with cortical
thinning however no cortical breach was present. (Figure
1a) The mass was surgically resected by giving Lynch
howarth incision and we received multiple grey white soft
tissue bits. Histopathological examination revealed stroma
comprised of bland fibroblastic spindle cells along with

A 38 year old female presented with right sided facial
swelling with a mucosa covered mass was obstructing the
right nasal cavity. Coronal non contrast CT showed a well-
defined, spherical expansile lesion involving the right nasal
cavity. Histopathological examination revealed stroma
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spherical and curved ossicles (Figure 2a) having concentric
pattern of lamination (Figure 2b) and focal osteoblastic
rimming. No necrosis or atypia were identified however
rare mitotic figures were seen. The histopathological

Competing interests: None declared. Conflict of interests: None declared.

comprised of bland fibroblastic spindle cells along with features along with clinic- radiological finding supported a
spherical and curved ossicles having concentric pattern of diagnosis of Juvenile psammomatoid ossifying fibroma
lamination and focal osteoblastic rimming. (JPOF).
Conclusion
PJOF is a very aggressive lesion which mimics malignancy Case 2
and due to its benign nature, early diagnosis can prevent A 38 year female presented with right sided facial swelling
over management and helpful in long term for six years and nasal obstruction for six months. On local
examination, a mucosa covered mass was seen obstructing
Introduction
Juvenile psammomatoid ossifying fibroma (JPOF) is an
uncommon benign bone-forming neoplasm in the
craniofacial skeleton of juvenile patients and defined as a
variant of ossifying fibroma (OF). It has been distinguished
from a larger group of ossifying fibromas on the basis of
age of the patients, site of involvement and clinical
behaviour.1,2
OF is highly cellular neoplasm and contains cementum-
like deposits which have a smooth contour with a radiating
fringe of collagen fibres.2 On the basis of
histomorphological features, juvenile ossifying fibromas
are further categorized into psammomatoid (PJOF) and
Figure 1: a) MRI PNS (Fronto - ethmiod sinus) shows
hypointense central soft tissue component seen in frontoethmoid
*Corresponding author
sinus causing expansion with thinning of the overlying skull
Email: jyotsnamamc@gmail.com
tables. No intracranial extension was observed. b) CT Maxilla
1
MAULANA AZAD MEDICAL COLLEGE, NEW DELHI, INDIA revealed a partially ossified mass deviating the nasal septum and
lateralizing the medial wall of the right maxillary sinus and
effacing the right maxillary cavity.

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FOR CITATION PURPOSES: Bharti JN, Dey B, Gautam P, Kaushal S. Juvenile psammomatoid ossifying fibroma of the
paranasal sinuses: A report of two cases. Head Neck Oncol 2014 Jul 18;6(3):25.

the right nasal cavity. CT of paranasal sinus revealed a
well-defined ovoid mass measuring 7x6.8 cm in the right
nasal cavity. The partially ossified mass caused deviation of
the nasal septum, lateralizing the medial wall of the right
maxillary sinus and causing effacement of the right
maxillary cavity. (Figure 1b) The mass was surgically
excised by lateral rhinotomy and sent for histopathological
examination. We received multiple soft tissue bits along
with bony bits. The histopathology section revealed
irregular ossicles exhibiting varying degrees of calcification
which were interspersed among fibroblastic stroma
(Figure 2c and Figure 2d). No atypia, necrosis and mitotic
figures were observed in the section examined. The final
diagnosis of psammomatoid ossifying fibroma was
supported by histomorphological features in correlation
with radiological and clinical findings.
Discussion
The classification of odontogenic tumours in the second
edition of the WHO, used the term juvenile (aggressive)
ossifying fibroma.3 It included two distinct histological
types of maxillofacial ossifying fibroma which were PJOF
and TJOF. In 1938, Benjamins designated PJOF as osteoid
fibroma with atypical ossification of the frontal sinus 5 and
in 1949 Golg termed it as psammomatoid ossifying fibroma
of the nose and paranasal sinuses.6 Finally in the year
1985, Margo et al. introduced the termed psammomatoid
juvenile ossifying fibroma.7
Reports vary on gender predilection.2,8 The average age of
occurrence for PJOF is 16 to 33 years.2 Both of our cases
were females and ages were beyond the average range as
reported in literature, although in some reports patients
age ranged from 3 months to 72 years.8
PJOF develops predominantly in the orbit and paranasal
sinuses mainly in the frontal and ethmoid sinuses.8 The
pathogenesis for these maxillofacial lesions is related to
the maldevelopment of basal generative mechanism that is
essential for root formation.
Diagnosis of PJOF is based on clinical-radiological and
microscopic findings. Clinically PJOF manifests as an
asymptomatic bony-hard swelling, the duration and extent
of which may vary depending on the site and
aggressiveness of the lesion.9 The symptoms such as pain,
paresthesia, sinusitis and proptosis can also occur.10
Radiologically PJOF appears as radiolucent, mixed or
radiopaque unilocular, oval or spherical lesion, depending
on the degree of calcification.10 A ‘ground-glass’
appearance on radiographs has also been reported in the
literature.2
Microscopically the pathognomonic feature is small
spherules resembling psammoma bodies, which are
referred to as psammoma-like bodies or psammomatoid
ossicles. The intervening stroma is highly cellular
consisting of spindle shaped fibroblasts with minimum
intervening collagen. The other features that can be seen
are pseudocystic stromal degeneration and haemorrhages
with aneurysmal bone cyst like spaces.4
Licensee OAPL (UK) 2014. Creative Commons Attribution License (CC-BY)
FOR CITATION PURPOSES: Bharti JN, Dey B, Gautam P, Kaushal S. Juvenile psammomatoid ossifying fibroma of the
paranasal sinuses: A report of two cases. Head Neck Oncol 2014 Jul 18;6(3):25.
Page 2 of 3
Case report
Figure 2: a) Cellular fibroblastic stroma admixed with
All authors contributed to conception and design, manuscript preparation, read and approved the final manuscript.
spherical and curved ossicles. (H&E 400x). b) Acellular
psammoma body-like ossicles with concentric pattern of
lamination (H&E 400x). c) Irregular ossicles exhibiting varying
degrees of calcification which were interspersed among
fibroblastic stroma (H&E 400x). d) Stratified squamous
epithelium, subepithelium showed spindle shaped fibroblastic
cells and irregular ossicles (H&E 100x).
Prognostically PJOF has an aggressive course with
tendency to recur.2,9 The aggressive nature of this entity
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and its high recurrence rate, surgical resection is the
preferred line of treatment.9,10
Conclusion
PJOF is very aggressive lesion which mimics malignancy
Competing interests: None declared. Conflict of interests: None declared.
and common in childhood, it rarely occurs in maxilla.
Diagnosis can be aided by awareness of the entity,
characteristic histologic features and clinico- radiological
finding. Due to its benign nature, early diagnosis can
prevent over management and helpful in long term
management.
References
1.Brannon RB, Fowler CB. Benign fibro-osseous lesions: a
review of current concepts. Adv Anat Pathol. 2001; 8:126–
143.
2.El-Mofty S. Psammamatoid and trabecular juveline
ossifying fibroma of trabecular juveline ossifying fibroma
of the craniofacial skeleton: Two distinct
clinicopathological entities. Oral Surg Oral Med Oral Path
Oral Radiol Endod. 2002; 93: 296–304.
3.Kramer IR, Pindborg JJ, Shear M. Histological typing of
odontogenic tumors. 2nd ed. Berlin: Springer-Verlag; 1992.
p. 28.
4.Nwizu NN, George MD, Chen F. An aggressive
psammomatoid ossifying fibroma presenting as a
sphenoethmoidal mass. N Am J Med Sci. 2010; 3:24-27.
5.Benjamins CE. Das osteoid-fibroma mit atypischer
Verkalkung im sinus frontalis. Acta Otolaryngol.1938;
26:26–43.
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Case report

6.Golg H. Das Psammoma Osteoid fibroma der nase und

ihrer Nebenhohlen. Monatsschr Ohrenheilkd
Laryngorhinol. 1949; 83:1–10.
7.Margo CE, Ragsdale BD, Perman KI, Zimmerman LE,
Sweet DE. Psammomatoid (juvenile) ossifying fibroma of
the orbit. Ophthalmology. 1985; 92:150–159.
8.Johnson LC, Yousefi M, Vinh TN, Heffner DK, Hymans VJ,
Hartman KS. Juvenile active ossifying fibroma, its nature,
dynamics and origin. Acta Otolaryngol. 1991; 488:1–40.
9.Thankappan S, Nair S, Thomas V, Sharafudeen KP.
Psammomatoid and trabecular variants of juvenile
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10.Offiah C, Hall E. The rapidly enlarging chin mass. Br J
Radiol. 2005; 78:175–176.

All authors contributed to conception and design, manuscript preparation, read and approved the final manuscript.
All authors abide by the Association for Medical Ethics (AME) ethical rules of disclosure.
Competing interests: None declared. Conflict of interests: None declared.

Licensee OAPL (UK) 2014. Creative Commons Attribution License (CC-BY)

FOR CITATION PURPOSES: Bharti JN, Dey B, Gautam P, Kaushal S. Juvenile psammomatoid ossifying fibroma of the
paranasal sinuses: A report of two cases. Head Neck Oncol 2014 Jul 18;6(3):25.