PAGET’S DISEASE OF BONE(OSTEITIS
DEFORMANS)
FIRST DESCRIBED BY SIR JAMES PAGET IN
1877
IS AN STEOLYTIC AND OSTEOSCLEROTIC BOE
DISEASE OF UNCERTAIN ETIOLOGY
INVOLVING ONE (MONOSTOTIC) OR MORE
BONE( POLYOSTOTIC)
PAGET’S DISEASE OF BONE(OSTEITIS
DEFORMANS)
AFFECTS PREDOMINANTLY MALES ABOVE
THE THE AGE OF 50 YEARS
FACTORS IMPLICATED IN THE ETIOLOGY ARE
1 . SLOW VIRUS INFECTION BY
PARAMYXOVIRUS ( RESPIRATORY SYNSITIAL
VIRUS,MEASLES VIRUS) OF OSTEOCLASTS.
BUT VIRUS HAS NOT BEEN CULTURED FROM
THE OSTEOCLASTS OF PAGET’S DISEASE
PAGET’S DISEASE OF BONE(OSTEITIS
DEFORMANS)
2.AUTOSOMAL DOMINANT INHERITANCE AND
GENETIC SUSCEPTIBILITY -7 – 10 FOLD
HIGHER PREVALENCE OF DESEASE IN FIRST
DEGREE RELATIVES. SUSCEPTIBILITY GENE IS
LOCATED CHROMOSOME 18q WHICH
ENCODES FOR A TUMOUR NECROSIS FACTOR
RANK(RECEPTOR ACTIVATOR OF NUCLEAR
FACTOR: kB
PAGET’S DISEASE OF BONE(OSTEITIS
DEFORMANS)
CLINICALLY MONOSTOTIC FORM MAY
REMAIN ASYMPTOMATIC AND MAY BE
DISCOVERED INCIDENTALLY ON RADIOLOGIC
INVESTIGATION
PAGET’S DISEASE OF BONE(OSTEITIS
DEFORMANS)
MORPHOLOGY
MONOSTOTIC FORM AFFECTS MOST
FREQUENTLYTIBIA,PELVIS,FEMUR,SKULL AND
VERTEBRA
POLYOSTOTIC FORM - ORDER OF
INVOLVMENT IS –VERTEBRA,PELVIS,,FEMUR
,SKULL,SACRUM AND TIBIA.
PAGET’S DISEASE OF BONE(OSTEITIS
DEFORMANS)-MORPHOLOGY
SEQENTIAL CHANGES IN PAGET’S DISEASE
1.INITIAL OSTEOLYTIC STAGE-OSTEOCLASTIC
RESORPTION BY INCREASED NUMBER OF LARGE
OSTEOCLASTS
2.MIXED OSTEOLYTIC AND OSTEOBLASTIC STAGETHERE IS IMBALANCE BETWEEN OSTEOCLASTIC
RESORPTION AND OSTEBLASTIC LAYING DOWN
OF NEW BONE(LAGS BEHIND).
PAGET’S DISEASE OF BONE(OSTEITIS
DEFORMANS)-MORPHOLOGY
SEQENTIAL CHANGES IN PAGET’S DISEASE
THIS RESULS IN CHARACTERISTIC MOSAIC
PATTERN OR JIGSAW PUZZLE APPEARANCE
OF OSTEOID SEAMS OR CEMENT LINES.SPACE
BETWEEN CORTEX AND TRABECULAE IS
FILLED WITH COLLAGEN
PAGET’S DISEASE OF BONE(OSTEITIS
DEFORMANS)-MORPHOLOGY
3.QUESCENT OSTEOSCLEROTIC STAGE
AFTER MANY YEARS EXCESSIVE BONE
FORMATION RESULTS ,BONE BECOMES
MORE COMPACT AND DENSE PRODUCING
OSTEOSCLEROSIS
PAGET’S DISEASE OF BONE(OSTEITIS
DEFORMANS)-MORPHOLOGY
BUT NEWLY FORMED BONE IS POORLY
MINERALISED SOFT AND SUSCEPTIBLE TO
FRACTURE
X RAY APPEARANCE OF INVOLVED BONE –
COTTON – WOOL APPEARANCE
SKULL BONE – COTTON WOOL
APPEARANCE
PAGET’S DISEASE OF BONEOSTEOLYTIC STAGE
PAGET’S DISEASE OF BONE OSTEOSCLEROTIC STAGE
TUMOUR LIKE LESIONS OF BONE
NON-NEOPLASTIC CONDITIONS RESEMBLING
TRUE NEOPLASMS
FIBROUS DYSPLASIA
BENIGN CONDITION,POSSIBLY DEVELOPMENTAL IN
ORIGIN
CHARACTERISED BY REPLACEMENT OF BONE BY
FIBROUS CONNECTIVE TISSUE WITH A
CHARACTERISTIC WHORLED PATTERN AND
CONTAINING TRABECULAE OF WOVEN BONE
X RAY – GROUND GLASS APPEARANCE
FIBROUS DYSPLASIA – 3 TYPES
1. MONOSTOTIC FIBROUS DYSPLASIA
2.POLYOSTOTIC FIBROUS DYSPLASIA
3. ALBRIGHT SYNDROME
MONOSTOTIC FIBROUS DYSPLASIA
MOST COMMON TYPE( 70% CASES),
AFFECT SOLITARY BONE
AFFECT EITHER SEX
MOST PATIENTS ARE BETWEEN 20 AND 30
YEARS
MONOSTOTIC FIBROUS DYSPLASIA
THE BONES AFFECTED IN DECREASING ORDER
OF FREQUENCY ARERIBS,CRANIOFACIAL BONE
ESPECIALLY MAXILLA,FEMUR,TIBIA AND
HUMEROUS
REMAINS ASYMPTOMATIC AND DISCOVERED
INCIDENTALLY AND INFREQUENTLY PRODUCE
TUMOUR LIKE ENLARGEMENT OF AFFECTED
BONE
POLYOSTOTIC FIBROUS DYSPLASIA
25% OF ALL CASES OF FIBROUS DYSPLASIA
AFFECT SEVERAL BONES
BOTH SEXES ARE AFFECTED EQUALLY
LESIONS APPEAR AT A EARLIER AGE
COMPARED TO MONOSTOTIC FORM
POLYOSTOTIC FIBROUS DYSPLASIA
MOST FREQUENTLY AFFECTED BONES ARE
CRANIOFACIAL,RIBS,VERTEBRAE AND LONG
BONES OF THE LIMBS
IN 20% OF CASES MORE THAN HALF OF THE
SKELETON INVOLVED BY THE DISEASE
POLYOSTOTIC FIBROUS DYSPLASIA
MAY AFFECT ONE SIDE OF THE BODY
OR MAY BE DISTRIBUTED SEGMENTALLY IN A
LIMB.SPONTANEOUS FRACTURE, SKELETAL
DEFORMITIES OCCUR IN CHILDHOOD
ALBRIGHT SYNDROME(McCUNEALBRIGHT SYNDROME)
IS A FORM OF POLYOSTOTIC FIBROUS
DYSPLASIA ASSOCIATED WITH ENDOCRINE
DYSFUNCTIONS AND ACCOUNTS FOR LESS
THAN 5% OF ALL CASES
MORE COMMON IN FEMALES
ALBRIGHT SYNDROME(McCUNEALBRIGHT SYNDROME)
CLINICALLY CHARACTERISED BY
POLYOSTOTIC BONE LESIONS,SKIN
PIGMENTATION( café-au-lait MACULAR
SPOTS) SEXUAL PRECOCITY AND
ENDOCRINOPATHIES.
MORPHOLOGIC FEATURES OF
FIBROUS DYSPLASIA
ALL FORMS OF FIBROUS DYSPLASIA HAVE AN
IDENTICAL PATHOLOGIC APPEARANCE
GROSSLY -LESIONS ARE SHARPLY DEMARCATED
LOCALISED DEFFECTS MEASURING 2 – 5 cm,
PRESENT WITHIN THE CANCELLOUS BONE
HAVING THIN AND SMOOTH OVERLYING
CORTEX
FIBROUS DYSPLASIA
MORPHOLOGIC FEATURES OF
FIBROUS DYSPLASIA-GROSS FEATURES
EPIPHYSEAL CARTILAGE IS SPARED IN
MONOSTOTIC BUT INVOLVED IN POLYOSTOTIC
FORM
REPLACEMENT OF NORMAL CANCELLOUS BONE
OF THE MARROW CAVITY BY GRITTY ,GREY PINK
RUBBERY SOFT TISSUE WITH AREAS OF
HAEMORRHAGE,MYXOID CHANGE AND CYST
FORMATION
MORPHOLOGIC FEATURES OF FIBROUS
DYSPLASIA- MICROSCOPIC FEATURES
BENIGN LOOKING FIBROBLASTIC TISSUE
ARRANGED IN A LOOSE,WHORLED PATTERN
IN WHICH THEREARE IRREGULAR AND
CURVED TRABECULAE OF WOVEN (NONLAMELLAR )
MORPHOLOGIC FEATURES OF FIBROUS
DYSPLASIA- MICROSCOPIC FEATURES
BONE IN THE FORM OF FISH HOOK
APPEARANCE OR CHINESE LETTER SHAPES
THERE ARE NO OSTEOBLAST RIMMING THE
BONE TRABECULAE.
RARELY MALIGNANT CHANGE MAY
OCCUR(SECONDARY OSTEOSARCOMA)
MORPHOLOGIC FEATURES OF FIBROUS DYSPLASIAMICROSCOPIC FEATURES,NO OSTEOBLASTIC
RIMMING
SOLITARY (SIMPLE, UNICAMERAL)
BONE CYST)
BENIGN CONDITION
CHILDREN AND ADOLESCENTS
MOST FREQUENTLY LOCATED IN THE
METAPHYSIS AT THE UPPER END OF
HUMERUS AND FEMUR
AS THE CYST EXPANDS THINNING OF THE
OVERLYING CORTEX OCCUR
SOLITARY (SIMPLE, UNICAMERAL)
BONE CYST
SOLITARY (SIMPLE, UNICAMERAL)
BONE CYST)
POSSIBLY THE LESION ARISES DUE TO LOCAL
DISORDER OF BONE GROWTH AND
DEVELOPMENT
CLINICALLY IT MAY REMIN SYMPTOMLESS OR
MAY CAUSE PAIN AND FRACTURE
SOLITARY (SIMPLE, UNICAMERAL)
BONE CYST)-MORPHOLOGY- GROSS
UNILOCULAR SIMPLE CYST OF THE BONE
SMOOTH INNER SURFACE
CAVITY IS FILLED WITH CLEAR FLUID
SOLITARY (SIMPLE, UNICAMERAL)
BONE CYST
SOLITARY (SIMPLE, UNICAMERAL) BONE CYSTMORPHOLOGY- HISTOLOGY
CYST WALL IS MADE UP OF THIN
COLLAGENOUS TISSUE WITH FEW
OSTEOCLAST GIANT CELLS AND NEWLY
FORMED BONY TRABECULAE
SOLITARY (SIMPLE, UNICAMERAL)
BONE CYST
SOLITARY (SIMPLE, UNICAMERAL)
BONE CYST
SOLITARY (SIMPLE, UNICAMERAL) BONE CYSTMORPHOLOGY- HISTOLOGY
FRACTURE MAY PRODUCE SANGUINOUS
FLUID IN THE CYST
HAEMORRHAGE,HAEMOSIDERIN DEPOSIT
AND MACROPHAGES ARE SEEN IN THE CYST
WALL
ANEURYSMAL BONE CYST
EXPANDING OSTEOLYTIC LESION FILLED WITH
BLOOD
ANEURYSM MEANING DILATATION
UNDER 30 YEARS OF AGE
ANEURYSMAL BONE CYST
FREQUENTLY INVOLVED BONES ARE SHAFTS
OF METAPHYSIS OF LONG BONES OR THE
VERTEBRAL COLUMN
X RAY – CHARACTERISTIC EXPANSILE LESION
UNDERNEATH THE PERIOTEUM
ANEURYSMAL BONE CYST
ANEURYSMAL BONE CYST
PROBABLY ARISES FROM PERSISTENT LOCAL
ALTERATION IN HAEMODYNAMICS
CLINICALLY –THE LESION MAY ENLARGE
OVER YEARS AND PRODUSE PAIN
,TENDERNESS AND PATHOLOGIC FRACTURE
ANEURYSMAL BONE CYST –
MORPHOLOGY -GROSS
LARGE HAEMORRHAGIC MASS COVERED BY
THINNED OUT REACTIVE BONE
ANEURYSMAL BONE CYST –
MORPHOLOGY -MICROSCOPY
LESION CONSIST OF BLOOD- FILLED
ANEURYSMAL SPACES OF VARIABLE
SIZE,SOME OF WHICH ARE ENDOTHELIUM
LINED
ANEURYSMAL BONE CYST
ANEURYSMAL BONE CYST –
MORPHOLOGY -MICROSCOPY
THE SPACES ARE SEPERATED BY CONNECTIVE
TISSUE SEPTAE CONTAINING OSTEOID
TISSUE,NUMEROUS OSTEOCLAST LIKE GIANT
CELLS AND TRABECULAE OF BONE
ANEURYSMAL BONE CYST –
MORPHOLOGY -MICROSCOPY
DIFFERENTIAL DIAGNOSIS –OSTEOCLASTOMA
ANDTELANGIECTATIC OSTEOSARCOMA