benign fibro-osseous lesions of the craniofacial area in
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Benign Fibro-Osseous Lesions of the Craniofacial
Area in Children and Adolescents
Доброкачественные фиброзно-костные
поражения краниофациальной зоны у детей и
подростков
Russian Children’s Clinical Hospital, Moscow
Рогожин Дмитрий Викторович.
Москва 2016
Waldron’s Modified Classification of BFOL (1993):
1. Fibrous Dysplasia.
2. Cemento-Osseous Dysplasia (periapical cement-osseous
dysplasia, focal cement-osseous dysplasia, florid
cement-osseous dysplasia).
3. Fibro-Osseous Neoplasm (cementifying fibroma,
ossifying fibroma, cemento-ossifying fibroma).
The classification system of Waldron has suggested that the BFOL originate
from the periodontal ligament which contains multipotent cells which are known
to differentiate into fibrous tissue cells, cementum and bone*.
[Karan Rajpal et al., IOSR Journal of Dental and Medical Sciences. Volume 13, Issue 2 Ver. IV. (Feb. 2014), p. 99-103].
Classification of benign fibro-osseous lesions of the
craniofacial complex (Roy Eversole et al., 2008):
I. Bone dysplasia
a. Fibrous dysplasia (monostotic, polyostotic, polyostotic with endocrinopathy
(McCune-Albright) and osteofibrous dysplasia).
b. Osteitis deformans
c. Pagetoid heritable bone dysplasias of childhood
d. Segmental odontomaxillary dysplasia
II. Cemento-osseous dysplasias
a. Focal cemento-osseous dysplasia
b. Florid cemento-osseous dysplasia
III. Inflammatory/reactive processes
a. Focal sclerosing osteomyelitis
b. Diffuse sclerosing osteomyeliitis
c. Proliferative periostitis
IV. Metabolic Disease: hyperparathyroidism
V. Neoplastic lesions (Ossifying fibromas)
a. Ossifying fibroma NOS
b. Hyperparathyroidism jaw lesion syndrom
c. Juvenile ossifying fibroma (trabecular and psammomatoid type)
d. Gigantiform cementomas
Benign fibro-osseous lesions (BFOLs) of the craniofacial
area are characterized by:
FD – 40 (68.9%)
Juvenile Ossifying Fibroma (JOF) – 15
(25.9%)
BFOL, unclassifiable, with overlapping
features – 3 (5.2%)
Pathologic ossifications and calcifications
A hypercellular fibroblastic marrow element.
Definition: FD is a benign medullary, sporadic fibro-osseous lesion, which may
involve one or more bones (monostotic FD or polyostotic FD)*.
Fibrous Dysplasia:
*Christopher D.M. Fletcher, Julia A. Bridge, Pancras C.W. Hogendoorn, Fredrik Mertens. WHO Classification of Tumors of Soft Tissue and Bone. 4th
Edition, 2013. P. 352-3.
**G. Petur Nielsen, Andrew E. Rosenberg, Vikram Deshpande, Francis J. Hornicek, Susan V. Kattapuram, Daniel I. Rosenthal. Diagnostic Pathology
Bone. 2013, p. 6/2-13.
***K. Krishnan Unni, Carrie Y. Inwards. Dahlin’s Bone Tumors. 2010, p. 317-327.
All forms of FD are associated with a non-germ cell acquired mutation at
codon 201 within exon 8 of the GNAS1 gene located on chromosome 20q**.
Patients with FD were divided into four distinct groups:
1. FD in the jaws,
2. FD in the bones of the skull,
3. FD in the ribs,
4. FD in all other bones.
The largest single group is the last one. Growth in the jawbone accounted for
the second largest group, and of these, the maxilla was involved much more
commonly than the mandible***.
We have analyzed 798 cases of bone tumors
and tumor-like lesions in children and
adolescents from 2009 to 2016.
FD was found in 58 patients (7.2%).
Craniofacial localization was diagnosed in 40
children (68.9%).
Male/Female ratio was 31/27.
Initial diagnoses of all the cases were based on
their clinical, radiological, and histological
features.
Fibrous Dysplasia (FD) in Children and Adolescents.
40
9
2
6
2
Sites of involvement, FD:
Maxilla – 18 cases.
Frontal bone and orbit – 9 cases.
Mandible – 5 cases.
Temporal and occipital bone – 4 cases.
Ethmoid and nasal cavity – 4 cases.
Two patients had multiple
craniofacial lesions (5%).
18
9
5
4
4
The main complaints and symptoms of patients
with FD are:
Painless swelling often leading to facial asymmetry.
Maxillary and mandibular involvement leads to
displacement of teeth and/or malocclusion.
In FD affecting the paranasal sinuses nasal obstruction
occurs.
Sometimes facial pain, headaches or facial numbness
develops.
The time from the appearance of symptoms to
treatment varied from 1 month to 8 years, with a mean
time of 24.7 months.
The size of the lesions varied from 10 to 70 mm.
Imaging findings:
Well-defined margins (can be sclerotic and
have rind-like appearance).
Expanded bone.
Classically produces ground-glass
appearance, and degree of radiolucency or
density is variable.
No prominent periosteal reaction.
Macroscopic findings:
Well-defined margin.
The tissue cuts with a gritty
consistency and is grayish white.
The cortical bone often is thinned
and expanded.
Classic microscopic pathology, FD:
Early formative lesions of FD lay down woven bone trabeculae with extensive
osteoblastic rimming of trabeculae and marked stromal hypercellularity whereas “older
mature” lesions show both woven and lamellar bone with trabecular that lacks osteoblasts*.
*Roy Eversole, Lan Su, Samir ElMoftu. Benign fibro-osseus lesions of the craniofacial complex. A review. Head and Neck Pathol (2008) 2:177-202.
The “maturation” processes in FD:
Differential Diagnosis, overlapping features:
FD
GCRG JPOF
JTOF
Osteomyelitis Low-grade
osteosarcoma
Desmoplasti
c fibroma
In 23 cases we have performed immunohistochemical staining
with CDK4 and MDM2:
All cases were negative for MDM2:
One case was slightly positive for
CDK4 (nuclear reaction):
But: MDM2
cdk4
Juvenile Ossifying Fibroma:
Definition: OF is well-demarcated lesion composed of fibrocellular tissue and
mineralized material of varying appearances.*
According to the WHO Classification (2005) most OFs commonly occurs in
the 2nd to 4th decades and shows a predilection for females*.
The second edition of the WHO classification of odontogenic tumors defines
juvenile (aggressive) ossifying fibroma as an actively growing lesion [**] and
consist of two histologic types:
1. Juvenile trabecular ossifying fibroma (JTOF).
1. Juvenile psammomatoid ossifying fibroma (JPOF).
*Leon Barnes, John W. Eveson, Peter Reichart, David Sidransky. Pathology and Genetics of Head and Neck Tumors. Lyon, 2005. P. 321-2, 323.
**Rashi Bahl, Sumeet Sandhu, Mohita Gupta. Benign fibro-osseus lesions of jaws – a review. International Dental Journal of Student's Research. June-Sep 2012, vol.1, issue 2, p. 56-68.
Image findings:
A round, well-circumscribed expansive mass with a
thick wall of bone density.
Sometimes corticated osteolytic.
Sclerotic changes are evident in the lesion, that show
a ground-glass appearance.
In CT scans set on bone window, the lesion appear
less dense than normal bone and can vary in size
from 2 to 8 cm in diameter.
Areas of low CT density may be noted due to cystic
changes.
Microscopic pathology of JTOF :
JTOF is unencapsulated and shows
infiltration of the surrounding bone.
The stroma is cell-rich, with spindle or
polyhedral cells that produce little
collagen.
The immature cellular osteoid is not
always easily distinguished from the
cellular stroma.
Maturation to lamellar bone is not
observed.
Local aggregates of osteoclastic giant
cells are invariably present in the
stroma*.
Mitoses are present, especially in the
cell-rich areas.
*Roy Eversole, Lan Su, Samir ElMoftu. Benign fibro-osseus lesions of the craniofacial
complex. A review. Head and Neck Pathol (2008) 2:177-202.
Microscopic pathology of JPOF :
The lesion consists of multiple round
uniform small ossicles
(psammomatoid bodies) embedded
in a relatively cellular stroma
composed of uniform, stellate, and
spindle shaped cells.
The psammomatoid bodies are
basophilic and bear superficial
resemblance to dental cementum,
but may have an osteoid rim.
Mitotic activity is extremely rare in
the stromal cells.
Cystic degeneration and aneurismal
bone cyst formation has been
reported in some cases.*
Roy Eversole, Lan Su, Samir ElMoftu. Benign fibro-osseus lesions of the craniofacial complex. A review. Head and Neck Pathol (2008) 2:177-202.
Secondary aneurysmal bone cyst registrated in 2 cases (5%).
Differential Diagnosis:
Psammomatoid meningioma (PM)
Primary extracranial meningiomas are rare and constitute 2% of all
meningiomas.
PM has been reported in the nasal cavity, paranasal sinuses and
nasopharynx, arising from heterotopic rests of arachnoid cells
(meningiocytes, meningothelial cells).
Histopathologically, JPOF and PM may be difficult to distinguish at the time
of intraoperative consultation (frozen sections), or even after obtaining
routine H&E sections*.
*Olga L. Bohn, John R. Kalmar, Carl M. Allen, Claudia Kirsch, Dayna Williams, Marino E. Leon Trabecular and Psammomatoid Juvenile Ossifying Fibroma of the
Skull Base Mimicking Psammomatoid Meningioma. Head and Neck Pathol (2011) 5:71–75.
Certain features favor PM rather
that JPOF, including:
1. The lack of associated osteoclasts
and osteoblasts rimming the
psammoma bodies and
1. The haphazard distribution of
psammoma bodies in PM.
Differential Diagnosis:
Psammomatoid meningioma (PM)
Differential Diagnosis:
EMA S-100 CD10 SMA
JPOF - - +/- +
PM + + +/- -
EMA SMA
JOF JOF
Differential Diagnosis:
Inflammatory/Reactive Processes
(Osteomyelitis)
Poorly circumscribed lesions
Often tooth involvement
May involve angulus mandibulae,
ramus mandibulae and processus
condylaris
Osteomyelitis:
Conclusions:
1. BFOLs are one of the commonest entities reported in the head and
neck region (39,4% among tumors of craniofacial area).
1. Craniofacial FD is the most common benign fibro-osseous lesion in
children and adolescents (67.4%).
2. Histologically we revealed extensive osteoblastic rimming of
trabeculae and marked stromal cellularity in the FD of craniofacial
area in comparison with FD of other sites.
3. FD should be differentiated from low-grade osteosarcoma, juvenile
ossifying fibroma, desmoplastic fibroma and giant cell reparative
granuloma.
4. FD and JOF can be differentiated from one another on the basis of
molecular detection of Gs-alpha mutation in FD of the jaws while
JOFs are negative.
Thank you for your attention!
Спасибо за внимание!
Рогожин Д.В.
Pathol.777@mail.ru
Patanatomia.rdkb@gmail.com
Russian Children's Clinical Hospital,
Moscow (Russian Federation).
Research Center of Pediatric Hematology, Oncology and Immunology,
Moscow (Russian Federation).
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