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  2. 2. Lesson plan • Introduction • Types • Epidemiology • Clinical presentation • Investigations • Management - Medical - Surgery: basics • Approach to a case of fibro-osseous lesion
  3. 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. 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. 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. 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. 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. 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. 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. 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. 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. 12. Clinical features
  13. 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. 14. Investigations CT SCAN
  15. 15. Investigations CT SCAN
  16. 16. Investigations CT SCAN
  17. 17. Investigations CT SCAN
  18. 18. Histopathology
  19. 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. 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. 21. Evaluation
  22. 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. 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. 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. 25. Clinical Features
  26. 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. 27. Investigation CT SCAN
  28. 28. Histopathology
  29. 29. Histopathology
  30. 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. 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. 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. 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. 34. Osteitis deformans (Paget’s disease of bone) Investigation- CT Scan
  35. 35. Management • Medical – Chemotherapy, Physiotheraphy, antiresorptive, analgesics • Combined Calcitonin & Etindronate therapy • Modern hearing devices alternatives to middle ear exploration,should be encouraged
  36. 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. 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. 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. 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. 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. 41. Investigation
  42. 42. Investigation
  43. 43. Histopathology
  44. 44. Management  Periapical and focal COD - no treatment, routine monitoring  Florid COD - close clinical follow-up for complications of osteomyelitis.
  45. 45. Metabolic bone disease
  46. 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. 47. Investigation- OPG
  48. 48. Investigation - Ct scan
  49. 49. Metabolic bone disease • Blood biochemistry -essential • Bone lesions due to secondary HP(CRF) more common. • Bone lesions of HP reverse on treatment.
  50. 50. Management  Periapical and focal COD - no treatment, routine monitoring  Florid COD - close clinical follow-up for complications of osteomyelitis.
  51. 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. 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

Editor's Notes

  • A typical lesion on the face, chest, and arm of a 5-year-old girl with McCune-Albright syndrome which demonstrates jagged "coast of Maine" borders, and the tendency for the lesions to both respect the midline and follow the developmental lines of Blaschko. B) Typical lesions that are often found on the nape of the neck and crease of the buttocks are shown (black arrows). C) A typical lesion on the lower back in an adult with McCune-Albright syndrome demonstrates jagged borders (white arrow). Note the spinal asymmetry due to fibrous dysplasia-related scoliosis.
  • The lesion shows varying amount of trabeculae of lamellar bone or woven bone, characteristically with osteoblastic rims and some osteoclasts,a few multinucleated giant cells and varying amounts of fibroblastic stroma. Cementum-like bodies (psammomatoid spherules) are not present.
  • Histologically, COF-APOF is composed of varying amounts and types of calcified material and varying amounts of fibroblastic stroma. The calcified structures may present as cementum-like (psammomatoid) basophilic deposits (round bodies or spherule forms), trabeculae of lamellar bone or woven bone, characteristically with prominent osteoblastic rims and some osteoclasts. Cementum-like bodies (psammomatoid spherules) are scattered throughout the lesion. The connective tissue consists of spindle fibroblastic cells in storiform pattern can be closely packed to nearly acellular. Mitosis can be seen. COF-APOF = cemento-ossifying fibroma– aggressive psammomatoid ossifying fibroma
  • This patient has sensorineural hearing loss and hemifacial spasm with weakness. Axial CT scan shows extensive mottled radiolucent changes of the skull base, with a coarse trabecular pattern and
    areas of sclerosis. This mosaic radiographic pattern reflects the coexistence of osteolysis and sclerosis seen in Paget’s disease. The external auditory canal, middle ear space, and otic capsule are normal bilaterally. The internal auditory canal is, however, stenotic on the side with acousticofacial neural dysfunction.

    Coronal CT scan through the otic capsule illustrates the translucent variant of Paget’s disease. The appearance is homogeneous and fuzzy. The otic capsule is eroded in a peripheral to
    central fashion. A shell of endosteal bone is preserved. Note the concomitant diffuse demineralization of the petrous pyramid.
  • Cemento-osseous dysplasia. Radiography shows lesions of mixed radiolucent and radiopaque florid cemento-osseous dysplasia in both quadrants of the mandible
  • Peri-apical COD
  • there is a single well-defined, corticated, lucent lesion in the body of the left mandible between the premolar and only remaining molar tooth, Radiolucent cyst-like lesions may be seen together
    with loss of lamina dura around tooth roots
  • 31-year-old female patient with bilateral jaw pain. CT showed bilateral mandibular brown tumours. Lesions contained stipple calcification
  • treatment of primary HP
    and respond to oral vitamin D in secondary HP.
  • Mill