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Fibroosseous lesions

Fibroosseous lesions of the jaw

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Fibroosseous lesions

  1. 1. FIBRO- OSSEOUS LESIONS OF THE JAWS PRESENTER: DR. KALPAJYOTI BHATTACHARJEE
  2. 2. CONTENTS • INTRODUCTION • CLASSIFICATIONS • OSSIFYING FIBROMA • FIBROUS DYSPLASIA • CEMENTO- OSSEOUS DYSPLASIA • CENTRAL GIANT CELL GRANULOMA • CHERUBISM • ANEURISMAL BONE CYST • SOLITARY BONE CYST
  3. 3. INTRODUCTION • “THE TERM FIBRO-OSSEOUS LESION (FOL) IS A GENERIC DESIGNATION OF A GROUP OF JAW DISORDERS” CHARACTERIZED BY THE REPLACEMENT OF BONE BY A BENIGN CONNECTIVE TISSUE MATRIX. • THIS MATRIX DISPLAYS VARYING DEGREES OF MINERALIZATION IN THE FORM OF WOVEN BONE OR OF CEMENTUM-LIKE ROUND ACELLULAR INTENSELY BASOPHILIC STRUCTURES.
  4. 4. • DIAGNOSIS OF THESE LESIONS BASED ON HISTOLOGIC APPEARANCE ALONE HAS CONSIDERABLE LIMITATIONS. • BENIGN FIBRO-OSSEOUS LESIONS (BFOL) OF THE JAW, FACIAL AND SKULL BONES ARE A VARIANT GROUP OF INTRAOSSEOUS DISEASE PROCESSES THAT SHARE MICROSCOPIC FEATURES, WHEREAS SOME ARE DIAGNOSABLE HISTOLOGICALLY. • MOST REQUIRE A COMBINED ASSESSMENT OF CLINICAL, MICROSCOPIC AND RADIOLOGIC FEATURES.
  5. 5. • CHARLES WALDRON WROTE “IN ABSENCE OF GOOD CLINICAL AND RADIOLOGIC INFORMATION A PATHOLOGIST CAN ONLY STATE THAT A GIVEN BIOPSY IS CONSISTENT WITH A FOL. WITH ADEQUATE CLINICAL AND RADIOLOGIC INFORMATION MOST LESIONS CAN BE ASSIGNED WITH REASONABLE CERTAINTY INTO ONE OF SEVERAL CATEGORIES”.
  6. 6. CLASSIFICATION WHO CLASSIFICATION OF FIBROOSSEOUS LESIONS OF JAWS (2005) 1) OSSIFYING FIBROMA (OF) 2) FIBROUS DYSPLASIA 3) OSSEOUS DYSPLASIA A. PERIAPICAL OSSEOUS DYSPLASIA B. FOCAL OSSEOUS DYSPLASIA C. FLORID OSSEOUS DYSPLASIA D. FAMILIAL GIGANTIFORM CEMENTOMA 4) CENTRAL GIANT CELL GRANULOMA 5) CHERUBISM 6) ANEURISMAL BONE CYST 7) SOLITARY BONE CYST
  7. 7. PAUL M. SPEIGHT & ROMAN CARLOS CLASSIFICATION (2006) 1. FIBROUS DYSPLASIA A. MONOSTOTIC FD B. POLYOSTOTIC FD C. CRANIOFACIAL FD 2. OSSEOUS DYSPLASIA A. PERIAPICAL OSSEOUS DYSPLASIA B. FOCAL OSSEOUS DYSPLASIA C. FLORID OSSEOUS DYSPLASIA D. FAMILIAL GIGANTIFORM CEMENTOMA 3. OSSIFYING FIBROMA A. CONVENTIONAL OSSIFYING FIBROMA B. JUVENILE TRABECULAR OSSIFYING FIBROMA C. JUVENILE PSAMMOMATOID OSSIFYING FIBROMA
  8. 8. • IT WAS FIRST REPORTED IN 1921 BY FRANGHENHEIM. • THE WORLD HEALTH ORGANIZATION (WHO) IN 1971 CLASSIFIED OSSIFYING FIBROMA AS A TYPE OF CEMENTIFYING FIBROMA. • ACCORDING TO THE 1992 WORLD HEALTH ORGANIZATION (WHO)CLASSIFICATION, COF IS A "DEMARCATED OR RARELY ENCAPSULATED NEOPLASM CONSISTING OF FIBROUS TISSUE CONTAINING VARYING AMOUNTS OF MINERALIZED MATERIAL RESEMBLING BONE AN/OR CEMENTUM." OSSIFYING FIBROMA (OF)
  9. 9. ETIOLOGY 1. ORIGIN: CLOSE PROXIMITY TO THE PERIODONTAL LIGAMENT HAS LED TO A PRESUMPTION THAT COFS ORIGINATE IN THE PERIODONTAL LIGAMENT WITH THE POTENTIAL FOR BOTH OSSEOUS AND CEMENTAL DIFFERENTIATION. 2. PROBABLY THEY ARISE FROM THE MULTIPOTENT MESENCHYMAL BLAST CELLS PRESENT IN THE PERIODONTAL MEMBRANE AND HAVE A CAPACITY TO PRODUCE CEMENTUM, ALVEOLAR BONE AND FIBROUS TISSUE. 3. RECENTLY MUTATIONS IN THE TUMOR SUPRESSOR GENE HRPT2 WERE IDENTIFIED.
  10. 10. OF IS FURTHER DIVIDED INTO SUBTYPES: CONVENTIONAL AND JUVENILE (JOF) :  TRABECULAR (JTOF)  PSAMMOMATOID (JPOF)
  11. 11. CLINICAL FEATURES • AGE: 3RD AND 4TH DECADES → MEAN AGE OF 32 YRS • SEX: FEMALE PREDILECTION • SITE: MANDIBLE ˃MAXILLA. MAINLY ARISES IN THE TOOTH BEARING AREAS. • MOST COMMON SITES : MANDIBULAR PREMOLAR AND MOLAR AREAS. • ASYMPTOMATIC AND EXPANSILE LESION • PAIN AND PARESTHESIA ARE RARELY ASSOCIATED. • SMALL LESIONS → DETECTED ONLY ON RADIOGRAPHIC EXAMINATION. • LARGER TUMORS → PAINLESS SWELLING OF THE INVOLVED BONE → FACIAL ASYMMETRY. • GROWTH RATE IS UNPREDICTABLE AND MAY BE SLOW AND STEADY OR RAPID
  12. 12. RADIOGRAPHIC FEATURES • EVERSOLE ET AL FOUND 2 MAJOR PATTERNS 1. WELL-DEFINED UNILOCULAR, ROUND, OR OVAL STRUCTURES. 2. LARGER TUMORS →MULTILOCULAR RADIOGRAPHIC APPEARANCE. • ACCORDING TO MACFRONALD-JANKOWSKI' INITIAL RADIOLUCENT PROGRESSIVELY RADIOPAQUE INDIVIDUAL RADIOPACITIES COALESCE
  13. 13. GROSS PATHOLOGY • CUT SURFACE → WHITISH YELLOW, • CONSISTENCY → VARIES WITH THE AMOUNT OF CALCIFIED MATERIAL. • WELL CIRCUMSCRIBED • RELATIVELY SMALL LESIONS OFTEN EXCISED COMPLETE WITH SOME SURROUNDING NORMAL BONE.
  14. 14. HISTOPATHOLOGIC FEATURES • SU ET AL FOUND THREE HISTOLOGIC SUBTYPES: 1. EQUAL AMOUNT OF CALCIFIED MATERIAL AND FIBROBLASTIC STROMA. • CALCIFIED STRUCTURES → BOTH SEPARATE AND RETIFORM BONY TRABECULAE WITH A PROMINENT OSTEOBLASTIC RIM AND OCCASIONAL OSTEOCLASTS. ROUNDED OR LOBULATED CEMENTUM- LIKE BODIES MAY BE SCATTERED THROUGHOUT THE LESION • CONNECTIVE TISSUE → SHEETS OF SPINDLE-SHAPED, FIBROBLASTIC, OR STELLATE CELLS WITH FOCAL AREAS OF STORIFORM PATTERN 2. LEAST COMMON SUBTYPE → CHARACTERIZED BY PREDOMINANTLY STORIFORM CELLULARITY IN THE STROMA CONTAINING SCANT SEPARATE OSTEOID OR BONY TRABECULAE, OFTEN WITHOUT OSTEOBLASTIC RIMMING. 3. COMBINATION OF THE FIRST TWO, WHICH ARE EACH SEEN IN DIFFERENT AREAS OF LARGE LESIONS.
  15. 15. • SPHERULES OF CEMENTUM-LIKE MATERIAL → PERIPHERAL ‘BRUSH BORDERS’. THAT BLEND INTO THE ADJACENT CONNECTIVE TISSUE → PARTICULAR ARRANGEMENT OF EXTRINSIC COLLAGEN FIBER BUNDLES OF ACELLULAR CEMENTUM. • FEATURE IN DISTINGUISHING OF FROM FD: COF IS A SHARPLY DEMARCATED LESION. THE HARD TISSUES OF THE TUMOR DO NOT FUSE WITH THE SURROUNDING BONE, EXCEPT OCCASIONALLY IN LIMITED AREAS PATTERN OF MINERALIZATION VARIES FROM PLACE TO PLACE WITHIN THE LESION, WHEREAS IN FIBROUS DYSPLASIA, THE PATTERN TENDS TO BE UNIFORM THROUGHOUT THE LESION
  16. 16. DIFFERENTIAL DIAGNOSIS • FIBROUS DYSPLASIA • JUVENILE OSSIFYING FIBROMA • OSTEOBLASTOMA • BENIGN CEMENTOBLASTOMA
  17. 17. TREATMENT AND PROGNOSIS • UNCOMPLICATED JAW CASES → SIMPLE ENUCLEATION/CURETTAGE. • AGGRESSIVE LESIONS → MORE EXTENSIVE SURGICAL RESECTION. • PROGNOSIS → VERY GOOD • RECURRENCE →RARELY ENCOUNTERED
  18. 18. JUVENILE OSSIFYING FIBROMA (ACTIVE OSSIFYING FIBROMA OR AGGRESSIVE OSSIFYING FIBROMA) • AN ACTIVELY GROWING LESION CONSISTING OF A CELL RICH FIBROUS STROMA, CONTAINING BANDS OF CELLULAR OSTEOID WITHOUT OSTEOBLASTIC RIMMING TOGETHER WITH TRABECULAE OF MORE TYPICAL WOVEN BONE. • SMALL FOCI OF GIANT CELLS MAY ALSO BE PRESENT, AND IN SOME PARTS THERE MAY BE ABUNDANT OSTEOCLASTS RELATED TO THE WOVEN BONE. • USUALLY NO FIBROUS CAPSULE CAN BE DEMONSTRATED, • WELL DEMARCATED FROM THE SURROUNDING BONE. • 2 PATTERNS: 1. PSAMMOMATOID AND 2. TRABECULAR- JUVENILE OSSIFYING FIBROMA
  19. 19. PSAMMOMATOID FIBROMA • FIRST REPORTED BY BENJAMINS, IN 1938. • THE TERM WAS COINED BY GOGL, IN 1949 • JOHNSON ET AL, IN 1952 COINED THE TERM JUVENILE ACTIVE OF → “CELLULAR MASS WHICH GENERATES INNUMERABLE SMALL UNIFORM-SIZED OSTEOID BODIES.” • REPORTED MORE FREQUENTLY IN THE LITERATURE
  20. 20. ETIOLOGY • OVERPRODUCTION OF THE MYXOFIBROUS CELLULAR STROMA NORMALLY INVOLVED IN THE GROWTH OF THE SEPTA IN THE PARANASAL SINUSES AS THEY ENLARGE AND PNEUMATIZE. • THESE STROMAL CELLS SECRETE HYALINE MATERIAL THAT OSSIFIES AND CONNECTIVE TISSUE MUCIN THAT INITIATES THE CYSTIC AREAS (SARODE, SARODE ET AL. 2011).
  21. 21. CLINICAL FEATURES PSAMMOMATOID JUVENILE OSSIFYING FIBROMA (PJOF) • MEAN AGE: 22 YEARS • SEX: MALE˃ FEMALE • SITE: 75% → DEVELOP IN THE ORBIT, PARANASAL SINUSES AND CALVARIA WHEREAS ONLY ABOUT 25% OF ALL CASES INVOLVE THE MAXILLA OR MANDIBLE. • MAXILLARY PREDOMINANCE.
  22. 22. TRABECULAR JUVENILE OSSIFYING FIBROMA (TJOF) • AGE: MEAN AGE; 11 YEARS • SEX: MALE˃ FEMALE. • SITE: 95% OF THE DOCUMENTED CASES OF TJOF HAVE DEVELOPED WITHIN THE JAW BONES. • MAXILLARY PREDOMINANCE. • IN MOST INSTANCES, THE NEOPLASMS GROW SLOWLY, ARE WELL - CIRCUMSCRIBED AND LACK CONTINUITY WITH THE ADJACENT NORMAL BONE
  23. 23. COMPLICATIONS • DUE TO IMPINGEMENT ON NEIGHBORING STRUCTURES. • WITH PERSISTENT GROWTH, LESIONS ARISING IN THE PARANASAL SINUSES PENETRATE THE ORBITAL, NASAL AND CRANIAL CAVITIES. NASAL OBSTRUCTION, EXOPHTHALMOS, . INTRACRANIAL EXTENSION → MENINGITIS
  24. 24. RADIOGRAPHIC FEATURES • BOTH TUMORS →`WELL-DEMARCATED, UNILOCULAR OR MULTILOCULAR RADIOLUCENCIES WITH A VARIABLE AMOUNT OF RADIOPACITY, USUALLY MANIFESTING AS FINE SPECKS OR AS GROUND-GLASS OPACIFICATION. • MANY TUMORS ARE INITIALLY DISCOVERED UPON ROUTINE RADIOGRAPHIC EXAMINATION, CORTICAL EXPANSION MAY RESULT IN CLINICALLY DETECTABLE FACIAL ENLARGEMENT. • CIRCUMSCRIBED RADIOLUCENCIES BUT IN SOME CASES, CENTRAL RADIOPACITIES CAN BE SEEN. • MAXILLARY TUMORS → OFTEN FILL AND OBLITERATE THE MAXILLARY SINUS, • MANDIBULAR TUMORS → USUALLY INVOLVE THE RAMUS AND ANGLE.
  25. 25. nasolabial fold as well as vestibule obliteration
  26. 26. GROSS PATHOLOGY • CONSISTENCY → FIRM TO HARD • COLOR → TAN-WHITE, GRAYISH-WHITE OR GRAYISH-BROWN • WELL DEMARCATED FROM THE SURROUNDING BONE, THOUGH NOT ENCAPSULATED. • ALSO DISPLAYS LARGE CYSTIC AREAS
  27. 27. HISTOPATHOLOGIC FEATURES • NONENCAPSULATED BUT WELL DEMARCATED FROM THE SURROUNDING BONE. • CELLULAR FIBROUS CONNECTIVE TISSUE → EXHIBITS AREAS THAT ARE LOOSE AND OTHER ZONES THAT ARE SO CELLULAR THAT THE CYTOPLASM OF INDIVIDUAL CELLS IS HARD TO DISCERN BECAUSE OF NUCLEAR CROWDING. • MYXOMATOUS FOCI ARE NOT RARE AND OFTEN ARE ASSOCIATED WITH PSEUDOCYSTIC DEGENERATION. • MITOTIC FIGURES CAN BE FOUND BUT ARE NOT NUMEROUS. • AREAS OF HEMORRHAGE AND SMALL CLUSTERS OF MULTI NUCLEATED GIANT CELLS ARE USUALLY SEEN.
  28. 28. TRABECULAR VARIANT • IRREGULAR STRANDS OF HIGHLY CELLULAR OSTEOID ENCASING PLUMP AND IRREGULAR OSTEOCYTES
  29. 29. PSAMMOMATOID PATTERN FORMS • CONCENTRIC LAMELLATED AND SPHERICAL OSSICLES THAT VARY IN SHAPE AND TYPICALLY HAVE BASOPHILIC CENTERS WITH PERIPHERAL EOSINOPHILIC OSTEOID. • A PERIPHERAL BRUSH BORDER BLENDING INTO THE SURROUNDING STROMA IS NOTED IN MANY OF THE OSSICLCS. OCCASIONALLY. INDIVIDUAL OSSICLES UNDERGO REMODELING AND FORM CRESCENTIC SHAPES.
  30. 30. DIFFERENTIAL DIAGNOSIS • CEMENTIFYING FIBROMAS, • CEMENTOBLASTOMA
  31. 31. TREATMENT AND PROGNOSIS • SMALLER LESIONS → COMPLETE LOCAL EXCISION OR THOROUGH CURETTAGE. • RAPIDLY GROWING LESIONS → WIDER RESECTION MAY BE REQUIRED. • RECURRENCE RATES → 30% TO 58% • MALIGNANT TRANSFORMATION HAS NOT BEEN DOCUMENTED.
  32. 32. FIBROUS DYSPLASIA • A BENIGN, SELF-LIMITING, BUT NONENCAPSULATED LESION OCCURRING MAINLY IN YOUNG SUBJECTS, USUALLY IN THE MAXILLA, AND SHOWING REPLACEMENT OF THE NORMAL BONE BY A CELLULAR FIBROUS TISSUE CONTAINING ISLANDS OR TRABECULAE OF METAPLASTIC BONE. • FIRST REPORTED BY VON RECKLINGHAUSEN IN 1891 • THE TERM FIBROUS DYSPLASIA WAS FIRST MENTIONED BY LICHTENSTEIN IN 1938.
  33. 33. ETIOPATHOGENESIS IDIOPATHIC NON HEREDITARY CAUSED BY MUTATION IN GNAS1 GENE
  34. 34. • THE ETIOLOGY HAS BEEN LINKED WITH A MUTATION IN THE GNAS1 GENE LOCATED AT CHROMOSOME 20q13.2 GNAS1 (GUANINE NUCLEOTIDE BINDING PROTEIN) GENE ENCODES A G - PROTEIN MUTATION RESULTS IN THE CONTINUOUS ACTIVATION OF THE G - PROTEIN OVERPRODUCTION OF cAMP IN AFFECTED TISSUES. HYPERFUNCTION OF CELLS AND ORGANS
  35. 35. • OSTEOBLASTS, MELANOCYTES, AND ENDOCRINE CELLS THAT REPRESENT THE PROGENY OF THAT MUTATED CELL CARRIES THAT MUTATION AND EXPRESS THE MUTATED GENE. • THE CLINICAL PRESENTATION OF MULTIPLE BONE LESIONS, CUTANEOUS PIGMENTATION, AND ENDOCRINE DISTURBANCES WOULD RESULT. UNDIFFERENTIATED STEM CELLS → EARLY EMBRYOLOGIC LIFE • PROGENY OF THE MUTATED CELL WILL DISPERSE • MULTIPLE BONE LESIONS OF FIBROUS DYSPLASIA. MUTATION OCCURS DURING THIS LATER PERIOD • PROGENY OF MUTATED CELL ARE CONFINED TO ONE SITE, • MONOSTOTIC FIBROUS DYSPLASIA MUTATION OCCURS DURING POSTNATAL LIFE
  36. 36. ENDOCRINE ORGANS • PRECOCIOUS PUBERTY • HYPERTHYROIDISM • GROWTH HORMONE PRODUCTION • CORTISOL OVERPRODUCTION MELANOCYTES • CAFÉ – AU – LAIT SPOTS OSTEOBLASTS • FIBROUS DYSPLASIA HYPERFUNCTIONS
  37. 37. CLASSIFICATION 1. MONOSTOTIC FIBROUS DYSPLASIA 2. POLYOSTOTIC FIBROUS DYSPLASIA  JAFFE'S LICHTENSTEIN SYNDROME  McCUNE- ALBRIGHT SYNDROME. 3. CRANIOFACIAL FORM 4. CHERUBISM
  38. 38. MONOSTOTIC FIBROUS DYSPLASIA • WHEN THE DISEASE IS LIMITED TO A SINGLE BONE, IT IS TERMED MONOSTOTIC FIBROUS DYSPLASIA. • ACCOUNTS FOR ABOUT 80% TO 85% OF ALL CASES • THE CLINICAL TERM "LEONTIASIS OSSEA" → A LEONINE APPEARANCE
  39. 39. CLINICAL FEATURES • M:F= 1:1 • SWELLING INVOLVES BUCCAL AND LABIAL PLATE AND SELDOM THE LINGUAL PLATE • PAINLESS AND SLOW GROWTH • OCCURS IN RIB (28%) > FEMUR (23%) > TIBIA > CRANIOFACIAL BONES (10-25%) > HUMERUS.
  40. 40. RADIOLOGICAL FEATURES
  41. 41. POLYOSTOTIC FIBROUS DYSPLASIA • 1ST RECOGNIZED BY WEIL IN 1922 • INVOLVEMENT OF TWO OR MORE BONES. • A RELATIVELY UNCOMMON CONDITION. • THE NUMBER OF INVOLVED BONES VARIES FROM A FEW TO 75% OF THE ENTIRE SKELETON. • AFFECTS MULTIPLE BONES LIKE JAWS, FEMUR, TIBIA, PELVIS, RIBS, SKULL, CLAVICLE AND FACIAL BONES 2 TYPES: 1. JAFFE'S LICHTENSTEIN SYNDROME 2. McCUNE ALBRIGHT'S SYNDROME
  42. 42. JAFFE'S LICHTENSTEIN SYNDROME • FD INVOLVING A VARIABLE NUMBER OF BONES, ACCOMPANIED BY PIGMENTED LESIONS OF THE SKIN OR "CAFE-AU-LAIT" SPOTS OF THIN LIGHT BROWN COLOR. • IT IS MILD AND NON- PROGRESSIVE FORM. • OCCURS IN ABOUT 50% OF THE CASES. CAFÉ AU LAIT SPOTS: • FLAT, PIGMENTED BIRTHMARKS • CAUSED BY A COLLECTION OF PIGMENT-PRODUCING MELANOCYTES IN THE EPIDERMIS OF THE SKIN.
  43. 43. McCUNE-ALBRIGHT SYNDROME • FULLER ALBRIGHT FIRST DESCRIBED THIS SYNDROME IN 1937. • MCCUNE ALBRIGHT SYNDROME IS DEFINED AS THE ASSOCIATION OF POLYOSTOTIC FIBROUS DYSPLASIA, PRECOCIOUS PUBERTY, CAFЀ-AU-LAIT SPOTS, AND OTHER ENDOCRINOPATHIES DUE TO HYPERACTIVITY OF VARIOUS ENDOCRINE GLANDS.
  44. 44. Mc CUNE ALBRIGHT SYNDROME NEUROFIBROMATOSIS NEVER CROSS MIDLINE CROSS THE MIDLINE IRREGULAR BORDERS SMOOTH BORDERS COAST OF MAINE COAST OF CALIFORNIA
  45. 45. CLINICAL FEATURES • 20-30 % CASES OF FD. • IT MOST COMMONLY OCCURS IN CHILDHOOD. • AGE: MEDIAN AGE OF ONSET OF SYMPTOMS IS 8-10 YEARS, • STRUCTURAL INTEGRITY OF THE BONE IS WEAKENED • WEIGHT BEARING BONES BECOME BOWED • THE CURVATURE OF THE FEMORAL NECK AND PROXIMAL SHAFT OF THE FEMUR MARKEDLY INCREASE CAUSING A 'SHEPHERD CROOK DEFORMITY', WHICH IS A CHARACTERISTIC SIGN OF THE DISEASE. • COMPLICATION: SPONTANEOUS FRACTURES
  46. 46. ORAL MANIFESTATIONS OF FIBROUS DYSPLASIA MALALIGNMENT TIPPING DISPLACEMENT DELAYED ERUPTION INTACT OVER LESION
  47. 47. MAZABRAUD'S SYNDROME • RARE DISEASE CAUSED DUE TO ASSOCIATION OF FD AND INTRAMUSCULAR MYXOMA. • PATIENTS WITH SOFT TISSUE MYXOMAS SHOULD BE THOROUGHLY EXAMINED FOR FD AS GREATER RISK OF SARCOMATOUS TRANSFORMATION IN FD WITH MAZABRAUD'S SYNDROME. • THERAPEUTIC IRRADIATION EXPOSURE. • FEMALES MAY HAVE A GREATER RISK FOR BREAST CANCER, PROBABLY DUE TO THEIR PROLONGED EXPOSURE TO ELEVATED ESTROGEN LEVELS. • ETIOLOGY: UNDERLYING GS ALPHA GENE MUTATION
  48. 48. classic shepherd’s crook deformity ‘RIND SIGN’. RADIOAGRAPHIC FEATURES
  49. 49. CRANIOFACIAL FIBROUS DYSPLASIA • NOT RESTRICTED TO A SINGLE BONE, BUT MAY BE CONFINED TO A SINGLE ANATOMICAL SITE. • PRIMARILY THE MAXILLA • MAY ALSO CROSS SUTURES INTO THE SPHENOID, ZYGOMA, FRONTONASAL BONES AND BASE OF THE SKULL. • DOES NOT MEET THE PRECISE CRITERIA FOR THE MONOSTOTIC OR POLYOSTOTIC FORMS AND HAS BEEN TERMED CRANIOFACIAL FIBROUS DYSPLASIA.
  50. 50. CLINICAL FEATURES • 10-25% OF PATIENTS WITH THE MONOSTOTIC FORM • 50% WITH THE POLYOSTOTIC FORM. • OCCURS DURING 1ST AND 2ND DECADES. • COMMON SITES OF INVOLVEMENT ARE FRONTAL, SPHENOID, MAXILLARY AND ETHMOID BONES.
  51. 51. INVOLVEMENT OF ORBITAL AND PERIORBITAL BONES  HEADACHE  HYPERTELORISM,  CRANIAL ASYMMETRY,  FACIAL DEFORMITY,  VISUAL IMPAIRMENT,  EXOPTHALMOS AND BLINDNESS. INVOLVEMENTOF ETHMOID BONE OR FRONTAL BONE :  A NARROWING AND DISPLACEMENT OF THE ORBITAL CAVITY. INVOLVEMENTOF NASAL AND PARANASAL CAVITIES:
  52. 52. RADIOGRAPHIC FEATURES • OFTEN MORE RADIODENSE → HIGHER PROPORTIONS OF BONE. • MARGINS OF EXTRA-GNATHIC FD APPEAR WELL DEFINED WHEREAS THEY ARE POORLY-DEFINED IN THE JAWS. • ‘ORANGE PEEL PATTERN’ WHICH CONSISTS OF AREAS OF ALTERNATING GRANULAR DENSITY AND RADIOLUCENCY. • TOOTH DISPLACEMENT AND LOSS OF LAMINA DURA IS NOTED IN PATIENTS WITH LESIONS INVOLVING THE TEETH.
  53. 53. GROSS PATHOLOGY/ MACROSCOPY • CONSISTENCY →VARIABLE, SOFT TO VERY HARD. • COLOR→ GRAYISH WHITE TISSUE • GRITTY TEXTURE WHEN CUT WITH A SCALPEL. • DEFORMITY OF THE AFFECTED BONE IS OBSERVED
  54. 54. HISTOPATHOLOGIC FEATURES OF FIBROUS DYSPLASIA • NO DISTINGUISHING HISTOLOGICAL FEATURES BETWEEN THE THREE TYPES OF FIBROUS DYSPLASIA. • VARY WITH THE DURATION OF DISEASE AND STAGE OF DEVELOPMENT. • FD REPLACES NORMAL BONE WITH A CELLULAR, FIBROUS TISSUE CONTAINING IRREGULARLY SHAPED BONY TRABECULAE. • THE TRABECULAE CONSIST OF IMMATURE, NON LAMELLAR (WOVEN) BONE WITHOUT OSTEOID RIMS OR OSTEOBLASTS. • EARLY OF FD → CHARACTERIZED BY A FIBROBLASTIC TISSUE WHICH IS RICHLY CELLULAR, OFTEN REVEALING A WHORLED PATTERN WITH LITTLE BONE. • "CHINESE CHARACTER TRABECULAE". • AFFECTED BONE USUALLY FUSES WITH THE ADJACENT NONAFFECTED BONE, WHETHER CORTICAL OR CANCELLOUS
  55. 55. • AS FD PROGRESSES, THE AMOUNT OF LAMELLAR TRABECULAE INCREASES. THESE TRABECULAE ARE SLENDER AND TEND TO RUN PARALLEL TO EACH OTHER. THEY LIE VERY CIOSE TOGETHER IN A MODERATELY CELLULAR FIBROUS STROMA. THE TERM OSSEOUS KELOID HAS SOMETIMES BEEN USED FOR THIS TYPE OF LESION. • MONOSTOTIC FD OF THE JAWS MAY EXHIBIT VARYING AMOUNTS OF SPHERICAL, AMORPHOUS CALCIFICATIONS AND CURVED/ LINEAR, ROUND, CALCIFIED TRABECULAE WHICH TEND TO FORM CONGLOMERATE STRUCTURES. THESE ARE CONSIDERED BY SOME RESEARCHERS TO BE MORE REPRESENTATIVE OF CEMENTUM THAN BONE. • A CHARACTERISTIC FEATURE OF FIBROUS DYSPLASIA THAT MAY HELP DISTINGUISH IT FROM OSSIFYING FIBROMA IS THAT THE LESIONAL BONE MERGES IMPERCEPTIBLY WITH ADJACENT CANCELLOUS BONE OR WITH THE OVERLYING CORTEX.
  56. 56. Early of FD → characterized by a fibroblastic tissue which Is richly cellular, often revealing a whorled pattern with little bone. "Chinese character trabeculae".
  57. 57. • LAMELLAR BONE IS ARRANGED IN PARALLEL ARRAYS. • STROMA IS TYPICALLY MODERATELY CELLULAR WITH SPARSE COLLAGEN LESIONAL BONE MERGES IMPERCEPTIBLY WITH ADJACENT CANCELLOUS BONE OR WITH THE OVERLYING CORTEX
  58. 58. LABORATORY FINDINGS • ↑ SERUM ALKALINE PHOSPHATASE LEVEL • PREMATURE SECRETION OF PITUITARY FOLLICLE STIMULATING HORMONE HAS BEEN REPORTED • ↑ BASAL METABOLIC RATE.
  59. 59. HISTOPATHOLOGICAL D/D : • OSSIFYING FIBROMA • PAGET’S DISEASE • OSTEOFIBROUS DYSPLASIA
  60. 60. MALIGNANT TRANSFORMATION • RARE • RANGES FROM 0.5% (IN MONOSTOTIC DISEASE) TO 4% IN ALBRIGHT'S SYNDROME. • THE FIRST DOCUMENTED CASE WAS REPORTED BY COLEY AND STEWART IN 1945. • MOST COMMON OF THE MALIGNANCIES → OSTEOSARCOMA, FOLLOWED BY FIBROSARCOMA AND CHONDROSARCOMA. • MOST MALIGNANT NEOPLASMS DEVELOP IN PATIENTS WHO PREVIOUSLY HAVE UNDERGONE RADIATION THERAPY TO THE AFFECTED AREA.
  61. 61. TREATMENT AND PROGNOSIS • SMALLER LESIONS → SURGICALLY RESECTED IN THEIR ENTIRETY WITHOUT TOO MUCH DIFFICULTY, • LARGE LESIONS → COSMETIC DEFORMITY → SURGICAL RECONTOURING • IN MANY CASES, THE DISEASE TENDS TO STABILIZE AND ESSENTIALLY STOPS ENLARGING WHEN SKELETAL MATURATION IS REACHED. • 25% AND 50% OF PATIENTS SHOW SOME REGROWTH AFTER SURGICAL SHAVE- DOWN. • SURGICAL INTERVENTION SHOULD BE DELAYED FOR AS LONG AS POSSIBLE. • RADIATION THERAPY IS CONTRAINDICATED IN FD BECAUSE IT CARRIES THE RISK FOR DEVELOPMENT OF POST IRRADIATION BONE SARCOMA
  62. 62. CEMENTO- OSSEOUS DYSPLASIA • CEMENTO-OSSEOUS DYSPLASIA (COD) ARE BFOLS OF THE JAWS CLOSELY ASSOCIATED WITH THE APICES OF TEETH AND CONTAINING AMORPHOUS SPHERICAL CALCIFICATIONS WHICH MAKES THEM RESEMBLE AN ABERRANT FORM OF CEMENTUM. • IN COD, THE TERM DYSPLASIA REFERS TO THE ABNORMAL DEVELOPMENT AND DISORDERED PRODUCTION OF BONE AND CEMENTUM-LIKE TISSUE. • MOST COMMON FIBRO-OSSEOUS LESION ENCOUNTERED IN CLINICAL PRACTICE.
  63. 63. CEMENTO-OSSEOUS DYSPLASIA CAN BE CLASSIFIED INTO 2 GROUPS: NON HEREDITARY PERIAPICAL FOCAL FLORID HEREDITARY FAMILIAL GIGANTIFORM CEMENTOMA.
  64. 64. ETIOLOGY THE ETIOLOGY AND PATHOGENESIS OF COD ARE UNKNOWN. PERIODONTAL LIGAMENT ORIGIN. EXTRALIGAMENTARY BONE REMODELING MAY BE TRIGGERED BY LOCAL FACTORS AND POSSIBLY CORRELATED TO AN UNDERLYING HORMONAL IMBALANCE
  65. 65. PERIAPICAL CEMENTO-OSSEOUS DYSPLASIA (SYNONYMS: OSSEOUS DYSPLASIA, CEMENTAL DYSPLASIA, CEMENTOMAS) • PREDOMINANTLY INVOLVES THE PERIAPICAL REGION OF THE ANTERIOR MANDIBLE. • SOLITARY LESIONS OR MULTIPLE FOCI ARE PRESENT MORE FREQUENTLY. • MARKED PREDILECTION FOR FEMALE PATIENTS (RANGING FROM 10:1 TO 14:1) • APPROXIMATELY 70% OF CASES AFFECT BLACKS. • AGE: 30 AND 50 YEARS. • SITE: MANDIBULAR PERIAPICAL AREA IS THE MOST COMMON SITE OF APPEARANCE.
  66. 66. THE KEY POINTS FOR THIS DISEASE DIAGNOSIS, ACCORDING TO BRANNON & FOWLER ARE: PREDILECTION FOR MID-AGE BLACK WOMEN; ONE OR MORE CIRCUMSCRIBED LESIONS → PERIAPICAL AREA OF VITAL TEETH; PAINLESS NON-EXPANSIVE LESION LOCATED USUALLY AT MANDIBLE’S ANTERIOR AREA; RADIOGRAPHIC CHARACTERISTICS CAN BE RADIOLUCENCY OF MIXED DENSITY (RADIOLUCENT WITH OPACITIES), OR OPAQUE WITH A NARROW RADIOLUCENT MARGIN; CELLULAR FIBROUS STROMA WITH LAMELLAR OSSEOUS TISSUE AND/OR OVAL CALCIFICATIONS.
  67. 67. RADIOGRAPHIC FEATURES
  68. 68. • CLASSICALLY, THE HISTOLOGIC FEATURES HAVE THREE STAGES AND ARE CORRELATED WITH THE RADIOGRAPIC FINDINGS  STAGE1: RADIOLUCENT (OSTEOLYTIC STAGE) - UNENCAPSULATED, CELLULAR, FIBROUS CONNECTIVE TISSUE WITH NUMEROUS SMALL-CALIBER BLOOD VESSELS.  STAGE2: RADIOLUCENT/ RADIOPAQUE (CEMENTOBLASTIC STAGE) – VARIABLE AMOUNTS OF WOVEN TRABECULAR BONE AND/OR SPHERULES OF CEMENTUM LIKE TISSUE.  STAGE3: RADIOPAQUE (MATURE STAGE) - COALESCENCE OF THE BONE AND/OR CEMENTUM LIKE TISSUE
  69. 69. FOCAL CEMENTO - OSSEOUS DYSPLASIA • EXHIBITS SINGLE SITE OF INVOLVEMENT. • ACCORDING TO WALDRON "LOCALIZED FIBRO-OSSEOUS CEMENTAL LESIONS PRESUMABLY REACTIVE IN NATURE.” • SEX: 90% IN FEMALES HAVING MALE : FEMALE OF 1: 8, WHITES > BLACKS • AGE: THIRD TO SIXTH DECADES WITH AN APPROXIMATE MEAN AGE OF 38 YEARS • SITE: TOOTH-BEARING AREAS OF THE POSTERIOR JAWS PREVIOUS EXTRACTIONS • ASYMPTOMATIC, PAINLESS AND FREQUENTLY NONEXPANSILE.
  70. 70. FLORID CEMENTO-OSSEOUS DYSPLASIA (FCOD) • MULTIFOCAL INVOLVEMENT NOT LIMITED TO THE ANTERIOR MANDIBLE. • PREDOMINANTLY INVOLVES BLACK WOMEN • MARKED PREDILECTION FOR MIDDLE AGED TO THE ELDERLY. • MARKED TENDENCY FOR BILATERAL AND OFTEN QUITE SYMMETRIC INVOLVEMENT. • NOT UNUSUAL TO ENCOUNTER EXTENSIVE LESIONS IN ALL FOUR POSTERIOR QUADRANTS. • USUALLY ASYMPTOMATIC, • SOMETIMES ALVEOLAR SINUS MAY BE PRESENT.
  71. 71. HISTOPATHOLOGIC FEATURES • ALL THREE PATTERNS DEMONSTRATE SIMILAR HISTOPATHOLOGIC FEATURES. • FRAGMENTS OF CELLULAR MESENCHYMAL TISSUE COMPOSED OF SPINDLE-SHAPED FIBROBLASTS AND COLLAGEN FIBERS WITH NUMEROUS SMALL BLOOD VESSELS. • FREE HEMORRHAGE IS TYPICALLY NOTED INTERSPERSED THROUGH OUT THE LESION. • WITHIN THIS FIBROUS CONNECTIVE TISSUE BACKGROUND IS A MIXTURE OF WOVEN BONE, LAMELLAR BONE, AND CEMENTUM LIKE PARTICLES. • AS THE LESIONS MATURE AND BECOME MORE SCLEROTIC. THE RATIO OF FIBROUS CONNECTIVE TISSUE TO MINERALIZED MATERIAL DECREASES. • WITH MATURATION, THE BONE TRABECULAE BECOME THICK CURVILINEAR STRUCTURES THAT HAVE BEEN SAID TO RESEMBLE THE SHAPE OF GINGER ROOTS. • WITH PROGRESSION TO THE FINAL RADIOPAQUE STAGE, INDIVIDUAL TRABECULAE FUSE AND FORM LOBULAR MASSES COMPOSED OF SHEETS OR FUSED GLOBULES OF RELATIVELY ACELLULAR AND DISORGANIZED CEMENTOOSSEOUS MATERIAL
  72. 72. ct stroma containing spindle-shaped fibroblasts and collagen fibers with numerous small blood vessels, free hemorrhage . curvilinear structures
  73. 73. DIFFERENTIAL DIAGNOSOS • FOCAL SCLEROSING OSTEOMYELITIS • OSSIFYING FIBROMA
  74. 74. TREATMENT AND PROGNOSIS • SCLEROSIS → HYPOVASCULAR → PRONE TO NECROSIS • SEQUESTRATION → OCCURS SLOWLY → HEALING. • ASYMPTOMATIC PATIENT → REGULAR RECALL EXAMINATIONS WITH PROPHYLAXIS. • BIOPSY OR ELECTIVE EXTRACTION OF TEETH SHOULD BE AVOIDED. • SAUCERIZATION OF DEAD BONE MAY SPEED HEALING.
  75. 75. FAMILIAL GIGANTIFORM CEMENTOMA • FIRST DESCRIBED BY AGAZZI AND BELLONI IN 1953 • WHO DEFINES IT AS “A MASS OF DENSE, HIGHLY CALCIFIED PARTLY OR ALMOST CELLULAR CEMENTUM OFTEN OCCURRING SIMULTANEOUSLY IN A NUMBER OF DIFFERENT LOCALISATIONS IN THE JAW.” • RARE AUTOSOMAL BENIGN DENTAL TUMOR • UNKNOWN ETIOPATHOGENESIS
  76. 76. CLINICAL FEATURES • NO SEXUAL PREDILECTION • COMMON IN AFRICAN BLACKS • PREVALENCE: FIRST DECADE; USUALLY CEASES DURING 5TH DECADE • MULTI FOCAL INVOLVEMENT OF BOTH THE MAXILLA AND MANDIBLE → FACIAL DEFORMITY • IMPACTION, MALPOSITION AND MALOCCLUSION.
  77. 77. RADIOGRAPHIC FEATURES (A) (B)
  78. 78. “GINGER ROOT APPEARANCE”
  79. 79. TREATMENT • EXTENSIVE RESECTION OF ALTERED BONE • FACIAL RECONSTRUCTIVE PROCEDURES • RECURRENCE IS RARE
  80. 80. CENTRAL GIANT CELL GRANULOMA • WHO DEFINES IT AS "AN INTRAOSSEOUS LESION CONSISTING OF MORE OR LESS FIBROUS TISSUE CONTAINING MULTIPLE FOCI OF HEMORRHAGE, AGGREGATIONS OF MULTINUCLEATED GIANT CELLS, AND SOMETIMES TRABECULAE OF WOVEN BONE FORMING WITHIN THE SEPTA OF MORE MATURE FIBROUS TISSUE THAT MAY TRAVERSE THE LESION.“ • FIRST INTRODUCED BY JAFFE IN 1953 • LESS THAN 7% OF ALL JAW LESIONS
  81. 81. PATHOGENESIS • GIANT CELL REMAINS THE MOST PROMINENT FEATURE • SPINDLE CELL FIBROBLAST RECRUITS MONOCYTES FROM THE VASCULAR SYSTEM AND INDUCES THEM TO DIFFERENTIATE INTO OSTEOCLASTIC GIANT CELLS THROUGH RELEASE OF CYTOKINES. • ORIGIN- MESENCHYME OF MARROW AND AN EPIGENETIC EVENT
  82. 82. OTHER THEORIES……… CGCG IS A VASCULAR PROLIFERATIVE LESION, → ANGIOGENESIS UNDER THE INFLUENCE OF THE TUMOR CELLS IS REQUIRED FOR TUMOUR GROWTH, INVASION, AND DESTRUCTION OF LOCAL TISSUE. MUTATIONS IN THE GENE SH3BP2 LOCAL FACTOR : TRAUMAS AND VASCULAR DAMAGE, WHICH PRODUCE INTRAMEDULLARY HEMORRHAGE AND INTRAOSSEOUS REPLACEMENT FIBROSIS.
  83. 83. CLINICAL FEATURES • AGE: YOUNG PATIENTS LESS THAN 30 YEARS • SEX: FEMALE˃ MALE • SITE: MANDIBLE ˃ MAXILLA, ANTERIOR PORTION OF THE JAW NOT COMMONLY CROSS THE MIDLINE. • PAINFUL OR PAINLESS RED TO PURPLISH BLUE NODULE LOCATED ON THE GUMS OR EDENTULOUS ALVEOLAR REGION
  84. 84. 2 TYPES: NONAGGRESSIVE SLOW GROWING ASYMPTOMATIC NO CORTICAL PERFORATION OR ROOT RESORPTION AGGRESSIVE RAPIDLY ENLARGING PAINFUL CORTICAL PERFORATION ROOT RESORPTION HIGH RATE OF RECURRENCE
  85. 85. RADIOGRAPHIC FEATURES DESTRUCTIVE LESION → RADIOLUCENT AREAS → SMOOTH OR RAGGED BORDERS SHOWING FAINT TRABACULAE
  86. 86. HISTOPATHOLOGIC FEATURES • LOOSE FIBRILLAR CONNECTIVE TISSUE STROMA WITH MANY INTERSPERSED PROLIFERATING FIBROBLASTS ANS SMALL CAPILLARIES. • PRESENCE OF FEW TO MANY MULTINUCLEATED GIANT CELLS IN A BACKGROUND OF OVOID TO SPINDLE SHAPED MESENCHYMAL CELLS. • THERE IS EVIDENCE THAT THESE GIANT CELLS REPRESENT OSTCOCLASTS, ALTHOUGH OTHERS SUGGEST THE CELLS MAY BE ALIGNED MORE CLOSELY WITH MACROPHAGES. • GIANT CELLS VARY CONSIDERABLY IN SIZE AND SHAPE AND MAY CONTAIN ONLY A FEW OR SEVERAL DOZEN NUCLEI. • AREAS OF ERYTHROCYTE EXTRAVASATION AND HEMOSIDERIN DEPOSITION ARE PROMINENT • FOCI OF OSTEOID OR NEWLY FORMED BONE ARE PRESENT
  87. 87. Numerous multinucleated giant cells within a background of plump proliferating mesenchymal cells. Note extensive red blood cell extravasation spindle-shaped fibroblast-like Aggregations of multinuclear giant cells are distributed between the stromal cells and often found near or evensituated inside (arrow) thin- walled vascular channels. osteoid trabeculum can be seen
  88. 88. DIFFERENTIAL DIAGNOSIS • CHERUBISM • GIANT CELL TUMOUR • BROWN TUMOURS
  89. 89. TREATMENT AND PROGNOSIS • CURETTAGE OR SURGICAL EXCISION • IN PATIENTS WITH AGGRESSIVE TUMORS. THREE ALTERNATIVES TO SURGERY- (I) CORTICOSTEROIDS, (2) CALCITONIN, AND (3) INTERFERON ALFA-2A • RECURRENCE RATE- 11%- 50%
  90. 90. CHERUBISM • WHO IN 1992 DEFINED IT AS “A BENIGN, SELF-LIMITING CONDITION IN WHICH THE LESLONAL TISSUE CONSISTS OF VASCULAR FIBROUS TISSUE CONTAINING VARYING NUMBERS OF MULTINUCLEATED GIANT CELLS ARRANGED DIFFUSELY OR FOCALLY.” • FIRST DESCRIBED BY JONES IN 1933 • AUTOSOMAL DOMINANT • 100% PENETRANCE IN MALES, ONLY 50-70% PENETRANCE IN FEMALES • NON NEOPLASTIC BONE LESIONS AFFECTING ONLY THE JAWS.
  91. 91. PATHOGENESIS MOST ACCEPTED THEORY:  ASSOCIATION WITH AUTOSOMAL DOMINANT GENE.  MUTATION IN GENE SH3BP2 ON CHROMOSOME 4P16 OTHER POSSIBLE HYPOTHESIS (CABALLERO AND VINALS)  MESENCHYMAL ALTERATION DURING JAW DEVELOPMENT  HORMONAL FACTORS  TRAUMA
  92. 92. SH3BP2 GENE MUTATION INFLAMMATION IN THE JAW BONE TRIGGERS PRODUCTION OF OSTEOCLAST BREAKAGE OF BONE TISSUE WHILE REMODELLING
  93. 93. • SEX PREDILECTION:100% MALE PENETRANCE AND 50-70% FEMALE PENETRANCE • PREVALENCE: BETWEEN THE AGES OF 2 AND 5 YEARS. • LOCATION: BILATERAL INVOLVEMENT OF THE POSTERIOR MANDIBLE • PAINLESS, BILATERAL, SYMMETRIC JAW ENLARGEMENT RESULTING IN MARKED FACIAL EXPANSION • CHARACTERISTIC “EYE TO HEAVEN” APPEARANCE. • TOOTH DISPLACEMENT OR FAILURE OF ERUPTION, IMPAIRED MASTICATION, SPEECH DIFFICULTIES, LOSS OF NORMAL VISION OR HEARING CLINICAL FEATURES
  94. 94. SEX STEROID AND THE INCREASE IN PLASMA CONCENTRATION OF ESTRADIOL AND TESTOSTERONE AT PUBERTY REDUCTION IN OSTEOCLAST FORMATION
  95. 95. A GRADING SYSTEM HAS BEEN PROPOSED": GRADE 1: INVOLVEMENT OF BOTH MANDIBULAR ASCENDING RAMI GRADE 2: INVOLVEMENT OF BOTH MANDIBULAR ASCENDING RAMI AND BOTH MAXILLARY TUBEROSITIES GRADE 3: MASSIVE INVOLVEMENT OF THE ENTIRE MAXILLA AND MANDIBLE EXCEPT THE CONDYLAR PROCESSES GRADE 4: SAME AS GRADE 3 WITH INVOLVEMENT OF THE ORBITS, CAUSING ORBITAL COMPRESSION.
  96. 96. ORAL MANIFESTATION • AGENESIS OF THE 2ND AND 3RD MOLAR • DISPLACEMENT OF TEETH • PREMATURE EXFOLIATION OF TEETH • DELAYED ERUPTION OF PERMANENT TEETH • TRANSPOSITION OR ROTATION OF TEETH • NOONAN’S SYNDROME: A LESION IN THE HUMERUS, GINGIVAL FIBROMATOSIS, PSYCHOMOTOR RETARDATION, ORBITAL INVOLVEMENT AND OBSTRUCTIVE SLEEP APNEA
  97. 97. RADIOGRAPHIC FEATURE • FLOATING TOOTH SYNDROME • GROUND GLASS APPEARANCE
  98. 98. HISTOLOGICAL FEATURE
  99. 99. DIFFERENTIAL DIAGNOSIS • GIANT CELL GRANULOMA • GIANT CELL TUMOR • HYPERPARATHYROIDISM • ANEURYSMAL BONE CYST
  100. 100. TREATMENT • SELF LIMITING CONDITION, TREATMENT IS MAINLY FOR THE ESTHETIC NEEDS AND FOR UNERUPTED TEETH. • CURETTAGE IS THE SURGERY OF CHOICE. • LIPOSUCTION • RADIOTHERAPY IS CONTRAINDICATED BECAUSE OF FEAR OF RETARDATION OF JAW GROWTH , OSTEORADIONECROSIS AND CHANCES OF MALIGNANT DEGENERATION. • MEDICAL THERAPY LIKE CALCITONIN IS THEORETICALLY APPROPRIATE • RECENT ADVANCEMENT → GENETIC THERAPY. RECURRENCE RATE OF 15-20%
  101. 101. ANEURYSMAL BONE CAVITY (ANEURYSMAL BONE CYST) • WHO DEFINED IT AS "A BENIGN INTRAOSSEOUS LESION, CHARACTERIZED BY BLOOD-FILLED SPACES OF VARYING SIZE ASSOCIATED WITH A FIBROBLASTIC TISSUE CONTAINING MULTINUCLEATED GIANT CELLS, OSTEOID, AND WOVEN BONE.“ • 1ST RECOGNIZED BY JAFFE AND LICHTENSTEIN IN 1942. • ETIOLOGY: CHROMOSOMAL INSTABILITY INVOLVING THE BAND 16q22 (PANOUTSAKOPOULOS ET AL)
  102. 102. ETIOPATHOGENESIS 1. LICHTENSTEIN IN 1950 - DUE TO ALTERED HAEMODYNAMICS. CYST ↑ VENOUS PRESSURE ENGORGEMENT OF VASCULAR BED RESORPTION CT REPLACEMENT AND OSTEOID FORMATION
  103. 103. 2.BERNIER AND BHASKAR • RESEMBLES CENTRAL GIANT CELL REPARATIVE GRANULOMA OF JAWS • BOTH LESIONS REPRESENT OVERZEALOUS ATTEMPTS OF CT TO REPLACE A HAEMATOMA IN THE BONE MARROW HAEMATOMA MAINTAINS CIRCULATORY CONNECTION WITH DAMAGED B.V ANEURYSMAL BONE CYST OBLITERATION OF CIRCULATION GIANT CELL REPARATIVE GRANULOMA
  104. 104. 3. BIESECKER ET AL PRIMARY BONE LESION OSSEOUS AV MALFORMATION SECONDARY REACTIVE LESION AV FISTULA RESORPTION OF ADJACENT BONE VASCULAR CHANNELS BOUND BY PERIOSTEAL BONE GIANT CELLS AND STROMAL CELLS SEEN FINALLY RESULTS IN THE FORMATION OF CYST CAVITY
  105. 105. 4. STRUTHERS AND SHEAR LOOSE FIBRILLAR CT STROMA OF CGCG INTERCELLULAR OEDEMA AND MICROCYST FORMATION ENLARGE AND COALESCE… PRESSURE RESORPTION OF MEDULLARY BONE ENDOSTEAL RESORPTION AND BLOWOUT OF LESION CYSTIC CAVITY IS FORMED
  106. 106. PHASES OF PATHOGENESIS 1. OSTEOLYTIC INITIAL PHASE 2. ACTIVE GROWTH PHASE 3. MATURE PHASE OR PHASE OF STABILIZATION 4. HEALING PHASE
  107. 107. CLASSIFICATION 1. PRIMARY FORM OF ABC  VASCULAR,  SOLID, OR  MIXED 2. SECONDARY FORM
  108. 108. MALOCCLUSION, MOBILITY, MIGRATION, BLOOD WELLING UP FROM THE TISSUE BLOOD SOAKED SPONGE CAVERNOUS SPACE OF THE LESION
  109. 109. GROSS PATHOLOGY Solid areas are interspersed with multiple cysts or locules
  110. 110. HISTOPATHOLOGIC FEATURES • TWO TYPES ARE: 1.CONVENTIONAL(95%)  OSTEOLYTIC LESION WITH MULTINUCLEATED GIANT CELLS  VASCULAR AREAS OF VARIABLE SIZE SEPARATED BY CONNECTIVE TISSUE  BONE TRABECULAE, OSTEOID TISSUE AND HEMOSIDERIN PIGMENT 2.SOLID(5%):  SOLID MASS WITHOUT CYSTIC COMPONENT  MULTIPLE HEMORRHAGIC FOCI  PLENTY OF FIBROBLASTS, OSTEOBLASTS AND OSTEOCLASTS
  111. 111. DIFFERENTIAL DIAGNOSIS • HAEMANGIOMA • GIANT CELL TUMOUR • SOLITARY BONE CYST
  112. 112. TREATMENT AND PROGNOSIS • CURETTAGE OR ENUCLEATION • CRYOSURGERY • SURGICAL DEFECT HEALS WITH 6 MONTHS – 1 YEAR. • RECURRENCE- 8%- 60%
  113. 113. TRAUMATIC BONE CYST • FIRST DESCRIBED BY LUCAS AND BLUM IN 1929 • LATER DESCRIBED BY RUSHTON AS “A SINGLE CYST THAT HAS NO EPITHELIAL LINING, HAS AN INTACT BONY WALL, IS FLUID FILLED, AND HAS NO EVIDENCE OF ACUTE OR CHRONIC INFLAMMATION.” • IT COMPRISES OF A SINGLE LESION WITHOUT AN EPITHELIAL LINING, SURROUNDED BY BONY WALLS AND EITHER LACKING CONTENTS OR CONTAINING LIQUID AND/OR CONNECTIVE TISSUE.
  114. 114. PATHOGENESIS TRAUMA HEMORRHAGE THEORY TRAUMA TO THE BONE INTRAOSSEOUS HEMATOMA (NO ORGANIZATION AND REPAIR), LIQUEFY CYSTIC DEFECT
  115. 115. OTHER THEORIES MIRRA ET AL PROPOSED THAT “A SMALL NEST OF SYNOVIUM BECOMES TRAPPED INTRAOSSEOUSLY DURING FETAL OR EARLY INFANT DEVELOPMENT AND THAT THIS TISSUE MAY RETAIN SOME SECRETORY FUNCTION, RESULTING IN THE DEVELOPMENT OF A CYST.” LOW-GRADE INFECTION , CYSTIC DEGENERATION OF BONE TUMORS, LOCAL ALTERATION OF BONE METABOLISM RESULTING IN OSTEOLYSIS ISCHEMIC MARROW NECROSIS,
  116. 116. CLINICAL FEATRURES • VAST MAJORITY INVOLVES THE LONG BONES. • AGE: 10-20 YEARS • SEX: MALE ˃ FEMALE • SITE: MANDIBLE ˃ MAXILLA • OCCASIONALLY BILATERAL • ASYMPTOMATIC • SOMETIMES PAIN AND PARESTHESIA PRESENT
  117. 117. RADIOGRAPHIC FEATURES WELL- DELINEATED RADIOLUCENT LESION MARGINS – SHARPLY DEFINED
  118. 118. HISTOLOGIC FEATURES • THE WALLS OF THE DEFECT MAY BE LINED BY A THIN BAND OF VASCULAR FIBROUS CONNECTIVE TISSUE OR DEMONSTRATE A THICKENED MYXOFIBROMATOUS PROLIFERATION THAT OFTEN IS INTERMIXED WITH TRABECULAE OF CELLULAR AND REACTIVE BONE. • THIS LINING MAY EXHIBIT AREAS OF VASCULARITY, FIBRIN, ERYTHROCYTES, AND OCCASIONAL GIANT CELLS ADJACENT TO THE BONE SURFACE. • NO EPITHELIAL LINING. • THE BONY SURFACE NEXT TO THE CAVITY OFTEN SHOWS RESORPTIVE AREAS (HOWSHIP'S LACUNAE) INDICATIVE OF PAST OSTEOCLASTIC ACTIVITY. Bone covered by a layer of loose fibrous connective tissue
  119. 119. DIFFERENTIAL DIAGNOSIS • PERIAPICAL CYST • ANEURYSMAL BONE CYST
  120. 120. TREATMENT AND PROGNOSIS • LONG BONES OFTEN IS MORE AGGRESSIVE AND INCLUDES INTRALESIONAL STEROID INJECTIONS OR THOROUGH SURGICAL CURETTAGE. • ENUCLEATION OF LINING IN THE COURSE OF MANIPULATION • RE-ESTABLISH BLEEDING • IF THE CAVITY IS THEN CLOSED→ HEALING IN 6-12 MONTHS • IN LARGE CAVITY → BONE CHIPS TO FILL THE CAVITIES
  121. 121. CONCLUSION • FIBRO-OSSEOUS LESIONS ARE A POORLY DEFINED GROUP OF LESIONS AFFECTING THE JAWS AND CRANIOFACIAL BONES. • CLASSIFICATION AND, THEREFORE, DIAGNOSIS OF THESE LESIONS IS DIFFICULT BECAUSE THERE IS SIGNIFICANT OVERLAP OF CLINICAL AND HISTOLOGICAL FEATURES. • NEW TERMINOLOGY HAS EMERGED THAT HAS CULMINATED IN THE LATEST WHO CLASSIFICATION. • DEFINITIVE DIAGNOSIS REQUIRES CORRELATION OF THE HISTOPATHOLOGIC FEATURES WITH THE PATIENT’S HISTORY, CLINICAL FINDINGS, RADIOGRAPHIC/IMAGING ANALYSIS, AND OPERATIVE FINDINGS BECAUSE OF THE HISTOLOGIC SIMILARITIES AMONG THIS DIVERSE GROUP OF LESIONS
  122. 122. REFERENCES 1. RAJENDRAN R, SIVAPATHASUNDHARAM B. SHAFER’S TEXTBOOK OF ORAL PATHOLOGY. 7TH EDITION. ELSEVIER PUBLICATION;941-1038 2. NEVILLE ET AL. ORAL & MAXILLOFACIAL PATHOLOGY. 2ND EDITION. ELSEVIER PUBLICATION. 3. GNEPP. DIAGNOSTIC SURGICAL PATHOLY OF THE HEAD AND NECK. 2ND EDITION. 4. MACDONALD-JANKOWSKI D.S. FIBRO-OSSEOUS LESIONS OF THE FACE AND JAWS. CLINICAL RADIOLOGY. 2004;59:11- 25 5. SPEIGHT P.M, CARLOS R. MAXILLOFACIAL FIBRO-OSSEOUS LESIONS. CURRENT DIAGNOSTIC PATHOLOGY. 2006;12:1-10 7. BRANNON RB, FOWLER CB. BENIGN FIBRO-OSSEOUS LESIONS: A REVIEW OF CURRENT CONCEPTS. ADV ANAT PATHOL. 2001; 8:126–43BAHL R, SANDHU S, GUPTA M. BENIGN FIBRO-OSSEOUS LESIONS OF JAWS – A REVIEW. INT DENT J STUDE RES. 2012;1(2):56-68 8. AGARWAL M ET AL. FIBROUS DYSPLASIA: A REVIEW. TMU J. DENT VOL. 1; ISSUE 1 JAN
  123. 123. 9. REGEZI JA, POGREL MA. COMMENTS ON THE PATHOGENESIS AND MEDICAL TREATMENT OF CENTRAL GIANT CELL GRANULOMAS (LETTER). J ORAL MAXILLOFAC SURG 2004; 62: 116-8 10. REGEZI JA, SCIUBBA JJ, JORDAN RCK. ORAL PATHOLOGY CLINICAL PATHOLOGIC CORRELATIONS. 4TH EDITION, ELSEVIER NEW DELHI (2003) 11. MAKEK M. CLINICAL PATHOLOGY OF FIBRO-OSTEO-CEMENTAL LESIONS IN THE CRANIO-FACIAL AND JAW BONES: A NEW APPROACH TO DIFFERENTIAL DIAGNOSIS. 1983 12. REICHENBERGER ET AL.: THE ROLE OF SH3BP2 IN THE PATHOPHYSIOLOGY OF CHERUBISM. ORPHANET JOURNAL OF RARE DISEASES 2012 7 (SUPPL 1):S5. 13. MERVYN SHEAR. CYSTS OF THE ORAL AND MAXILLOFACIAL REGIONS. 4TH EDITION. 14. DEVI P, THIMMARASA VB, MEHROTRA V, AGARWAL M. ANEURYSMAL BONE CYST OF THE MANDIBLE: A CASE REPORT AND REVIEW OF LITERATURE. J ORAL MAXILLOFAC PATHOL 2011;15:105-8. 15. HARNET JC. SOLITARY BONE CYST OF THE JAWS: A REVIEW OF THE ETIOPATHOGENIC HYPOTHESES. J ORAL MAXILLOFAC SURG 66:2345-2348, 2008.
  124. 124. THANK YOU

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