Fibro Lesions

1. FIBRO-OSSEOUS LESIONS IN ENT
PRESENTER- Dr Ahalya

2. Lesson plan
• Introduction
• Types
• Epidemiology
• Clinical presentation
• Investigations
• Management - Medical
- Surgery: basics
• Approach to a case of fibro-osseous lesion

3. Fibro- osseous lesions
• Poorly defined group of lesions affecting jaws &
craniofacial bones, Normal bone fibroblastic
stromaPathological ossification/ calcification.
• clear developmental etiology, genetic mutations,
inflammatory/infectious conditions & benign
neoplasms.

4. Classification
Pathological Process Disease/condition
Bone Dysplasias Fibrous Dysplasia
Osteitis deformans (Paget’s disease)
Cemento- osseous dysplasias (COD) Focal COD
Florid COD
Inflammatory/reactive processes Focal Sclerosing osteomyelitis
Diffuse Sclerosing osteomyelitis
Proliferative periostitis
Metabolic Bone diseases Hyperparathyroidism
Genetic Cherubism
Neoplastic Ossifying fibroma

5. Fibrous dysplasia
• “Osteitis fibrosa generalisata” von Recklinghausen -1891
• Lichtenstein termed “fibrous dysplasia” in 1938
• Slow growing, tumour-like lesion of bone, stabilise after
skeletal maturity
 3 types -monostotic
-polyostotic
-polyostotic with endocrinopathy (Mc Cune Albright
Syndrome)

6. Fibrous dysplasia
 5% to 10% of all bone tumors
 Incidence - 1:4000-1:10,000, no gender predilection
 Usually unilateral -mostly ribs and femur
 Craniofacial skeleton-
SkullBase>frontal>sphenoid>ethmoid>parietal,>tem
poral>occipital bones.
 Overall temporal bone involvement18% of
cases affecting skull

7. Fibrous dysplasia - Types
 Monostotic (80%) –craniofacial involvement-
25%, maxilla and mandible- M/C.
 Polystotic (20%) – craniofacial involvement
present in 40% to 50% of cases.
 Monostotic FD (MFD) is 6- to 10-fold > Polyostotic
FD (PFD)> MAS
 MFD - first 3 decades of life, PFD and MAS
present earlier in childhood

8. Fibrous dysplasia - types
 Polyostotic FD, endocrine hyperfunction- McCune-Albright
syndrome. rare condition primarily affects females.
 Term “craniofacial fibrous dysplasia” (CFD) -fibrous
dysplasia where the lesions are confined to contiguous
bones of the craniofacial skeleton
-slight female predilection,
-first 3 decades,
-stabilize when the patient reaches skeletal maturity

9. Somatic missense mutation
Gene GNAS -1 chr- 20
Encodes for a-subunit of gsa
Replacement of Arg with Cys/ His
Inhibition of Int GTPase Activity
Ligand independent activation & accumulation of cAMP
Differentiation arrest
Fibro-osseous Mass
Pathophysiology

10. Pathophysiology
• Lichtenstein -“perverted activity of the specific bone-
forming mesenchyme,” -1938
• Somatic mis-sense mutation
• Burden of mutated cells
influenced by growth factors and hormones
frequently declines with age
resulting in tumor arrest.

11. Clinical features
 Painless bony enlargement.
 EAC stenosis, progressive hearing loss, postauricular
swelling
 McCune-Albright syndrome- café-au-lait skin
pigmentation & endocrine abnormalities
 FD - low rate of malignant transformation, 0.5% of
polyostotic forms and in 4% of lesions with McCune-
Albright syndrome

12. Clinical features

13. Investigations
DIAGNOSIS
CT Scan
50%- Ground glass
pattern
25 %- Dense Pattern
20%- Cystic variety
Initially Radiolucent
mottled
ground glass/orange
peel
opaque
MRI
Depends on bony
trabeculation
& degree of
cellularity
Bone biopsy
For HP
diagnosis
Scintigraphy
Assess the burden of
disease

14. Investigations
CT SCAN

15. Investigations
CT SCAN

16. Investigations
CT SCAN

17. Investigations
CT SCAN

18. Histopathology

19. Management
 In most cases, the lesions stabilize with skeletal
maturation.
 Simple contouring - affected facial or skull bones to
normal dimension has proven to be adequate.
 Conservative medical management advised.

20. Management
Surgical Management
 Maintenance of a patent external auditory canal
 Radical resection is not warranted
 EAC recontouring (split thickness skin graft bolstered
by silastic sheeting)
 Surgery of dysplastic temporal bone

21. Evaluation

22. Ossifying fibroma
• Benign fibro-osseous neoplasms  jaws & craniofacial skeleton.
• 3 clinicopathological variants –
1.Cemento ossifying fibroma (COF) ossifying fibroma of
odontogenic origin
2. Juvenile trabecular ossifying fibroma (JTOF)
3. Juvenile psammomatoid ossifying fibroma (JPOF)

23. Ossifying Fibroma
 COF is rare, peak incidence-30-40yrs; F:M::5:1
 JTOF - rare, predominantly children and adolescents, 8.5-
12 yrs(age grp), no sex predilection
 JPOF - rare tumour,16 to 33(age grp,no sex predilection
Genetics
 Multiple ossifying fibromas  hyperparathyroidism-jaw
tumour syndrome COC73 (also called HRPT2)
mutations.

24. Localization
 Tooth-bearing areas- mandible and maxilla.
 Mandible(premolar & molar area-m/c)>Maxilla.
 JTOF - maxilla and mandible, (maxilla -M/C).
Extragnathic occurrence - extremely rare.
 JPOF - Predominantly affects extragnathic craniofacial
bones, particularly periorbital, frontal and ethmoid
bones

25. Clinical Features

26. Clinical Features
 JTOF’s - progressive and sometimes rapid expansion
 JPOFs - bony expansions-involve orbit, nasal bones &
sinuses. Tumour expansion can result in proptosis, visual
symptoms, and nasal obstruction.

27. Investigation
CT SCAN

28. Histopathology

29. Histopathology

30. Management
OF suspected on imaging
Biopsy to confirm diagnosis
Exclude any malignancy
Complete surgical excision depending on site/ symptom
Extensive disease
Asymptomatic Craniofacial resection
conservative

31. Osteitis deformans (Paget’s disease of bone)
• Second most frequent metabolic bone disorder.
• Predilection for jawbones (mandible M/c)
• Onset uncommon prior to 40 years of age
• overall incidence is 3%,  rises to 10% by eighth decade.
• 20% -asymptomatic
• Mostly sporadic, 15%-AD Inheritance pattern evident
• Immunoregulatory defect on chromosome 6.

32. Osteitis deformans (Paget’s disease of bone)
Etiology
Genectic + Environmental Factors
Viral Infection  Bone cells
Encodes P62
SQSTM1 Gene affected
Increases bone turnover

33. Osteitis deformans (Paget’s disease of bone)
Clinical features
 Pain, stiffness and fatigability
 Hearing loss- in PDB(13%-40%) High Frequency
sensorineural
 Tinnitus more common
 Giddiness (20–30% of individuals with PDB suffer
from vestibular dysfunction)

34. Osteitis deformans (Paget’s disease of bone)
Investigation- CT Scan

35. Management
• Medical – Chemotherapy, Physiotheraphy, antiresorptive,
analgesics
• Combined Calcitonin & Etindronate therapy
• Modern hearing devices alternatives to middle ear
exploration,should be encouraged

36. Management
• Surgical
• hearing loss and cranial neuropathy entertained only
after full course chemotherapeutic trial
• Persistent symptomatic IAC stenosis with SNHL & facial
nerve dysfunction

37. Complication
 Osteosarcoma-(< 0.1%)
 Skull expansion- disfigurement(frontal bossing)
 Extensive involvement of skull base
 Cranial nerve involvement
 Platybasia
 High-output cardiac failure (rare).

38. Cemento-osseous dysplasia
• M/C fibro-osseous lesion of the jaws.
• Middle-aged Black women- Strong Predeliction
• Three variants ( basis of anatomical location):
-Periapical COD- apical areas of mandible
-Focal COD- single tooth
-Florid COD-multifocal (multi-quadrant)

39. Cemento-osseous dysplasia
Clinical Features
 Asymptomatic, discovered on routine dental
radiographs.
 Generally non-expansive
 Florid cases- expansile; present with pain and
discharge secondary to infection.

40. Investigation
 Lesions -identified clinically and radiographically, without
the need for biopsy
 Serial radiographs - increased density and calcification as a
lesion matures.
 Focus of COD  well defined & demonstrates a thin
radiolucent rim.

41. Investigation

42. Investigation

43. Histopathology

44. Management
 Periapical and focal COD - no treatment, routine
monitoring
 Florid COD - close clinical follow-up for complications
of osteomyelitis.

45. Metabolic bone disease

46. Metabolic bone disease
• Jaws affected  multifocal bone replacement- brown
tumours.
• collections -osteoclasts within haemosiderin-rich stroma
• Primary hyperparathyroidism (HP)- parathyroid
hyperplasia or adenoma mainly affects post-menopausal
females.

47. Investigation- OPG

48. Investigation - Ct scan

49. Metabolic bone disease
• Blood biochemistry -essential
• Bone lesions due to secondary HP(CRF) more common.
• Bone lesions of HP reverse on treatment.

50. Management
 Periapical and focal COD - no treatment, routine
monitoring
 Florid COD - close clinical follow-up for complications
of osteomyelitis.

51. Approach to a case of suspected Fibro-osseous lesion
Approach to a case of suspected Fibro-osseous lesion
Fibrous Dysplasia
Cotton wool
Appearance
Monostotic
Café au lait spots
& endocrine
abnormalities
>1 bone
involved
Only 1
bone
involved
Biochem
Parameters-
^Ca,^PTH, vPO
Single/multiple
lesions-lytic/mixed
Incidental finding in
jaw, asymptomatic
COD
Ossifying Fibroma
Metabolic Bone
Diseases
Paget’s Disease
Unilocular Mixed Radio-lucent
& radio-opaque with sharply
demarcated borders
Homogenous
radiodense opacities
(ground glass pattern)
Polyostotic
Mc Cune Albright
Syndrome

52. • Scott-Brown's Otorhinolaryngology, Head and Neck Surgery,
8th ed . 2015 .
• World Health Organization Classification of Tumours -4th
edition
• Neurotology (Robert Jackler & Derald Md ) -2 nd edition
• Mills’s & Sternberg Diagnostic Surgical Pathology 7 th edition
References