Professional Documents
Culture Documents
Dr Deepak K Singh
PMRD, NIRTAR
INCIDENCE
Age
- multiple enchondromatosis - childhood
- solitary enchondromas - after the second
decade of life
- peak age of presentation - 35 years
PATHOLOGY - MACRO
Color - glistening white, grayish-white, or pearly
tissues
Feel - gritty feel on palpation owing to the
intrinsic calcification
- easily cut with a knife, as if it were soft
chalk.
PATHOLOGY - MICRO
Proliferating nests of cartilage cells ,lack obvious
atypia
Foci of calcification
Plates of lamellar bone surround the lobules of
cartilage in a partial to complete circumferential
manner
PATHOLOGY SARCOMATOUS
CHANGE
Growing children
- with dysplastic cartilaginous lesions
likelihood of malignancy low
Adults
- difficult to differentiate an enchondroma
from a low-grade sarcoma
Site
- small peripheral cartilage tumors - benign
- large axial tumors likely to be malignant
CLINICAL FEATURE
Site
- 50% occur in the hand ( phalanges and carpal
bones )
- long tubular bones ( femur and humerus )
- occasionally the ribs, sternum, innominate
bones, and vertebral columns
Presentation
- usually diagnosed after local trauma
- some with a firm, local swelling in
affected phalanx or metacarpal without a
fracture or local pain.
- femur / humerus usually quiescent, with no
clinical signs evident until adulthood
RADIOLOGY SOLITARY
ENCHONDROMA
well-delineated, lucent defects
JUXTACORTICAL CHONDROMA
DIFFERENTIAL DIAGNOSIS
Bone infarct peripheral located calcification
Epithelial inclusion cysts in phalanges of hand
and feet
Solitary bone cyst / nonossifying fibroma/ fibrous
dysplasia in the metacarpal
TREATMENT
MULTIPLE ENCHONDROMATOSIS /
OLLIERS DISEASE
Rare condition
Many cartilaginous tumors in large and small
tubular bones and flat bones.
Cause - failure of normal endochondral
ossification - dyschondroplasia
Individual lesions similar to solitary chondroma
Most commonly - phalanges, femur, and tibia
PATHOLOGY
Numerous islands of glistening cartilage
Diaphyseal and metaphyseal regions.
Infrequently, in the epiphyseal region
Close proximity to the physis - severe limb length
discrepancies or angular deformities.
abnormal enchondroma cartilage near the
epiphyseal plate - pathognomonic fanlike
metaphyseal septation radiographs
PATHOLOGY SARCOMATOUS
CHANGE
highly cellular lesion with ominous nuclei mimick
a low-grade chondrosarcoma
Eventual malignant transformation 20% to 33%
Biopsy site in hand benign lesion
Biopsy site in pelvis or larger bones - suspicion
of low-grade sarcomas is higher.
high incidence of chondrosarcomatous
transformation in older individuals
CLINICAL FEATURE
ASSOCIATION
When associated with hemangiomas of the
overlying soft tissues Maffucci syndrome.
Sarcoma - 25% of patients with Ollier disease
by 40 years of age.
MALIGNANT CHANGE
25% to 30% by 40 years of age with Olliers disease
Maffucci's syndrome
- higher likelihood of development of malignant
degeneration, nearly 100% expectation
- periodic surveillance of the brain and abdomen
for occult malignant lesions
Hallmark of possible malignancy
- Increased localized growth of a lesion in an
extremity + pain
Hematopoietic malignancies (ALL and CML)
sometimes associates with both Ollier's disease and
Maffucci's syndrome
RADIOLOGY
More extensive bone abnormality
Long bones
- radiolucent longitudinal streaks involve the
metaphysis and extend down into the
diaphysis
- epiphyses usually not affected
Cortex overlying thin
Calcification within the lesion
Shortening and angular deformity
ASSOCIATION WITH
CHONDROSARCOMA
More proximal locations - pelvis, proximal
humerus, proximal femur
Deep endosteal erosion - 2/3rd thickness of the
cortex
- CT best evaluate
endosteal erosion
TREATMENT
Multiple enchondromatosis
- more difficult to treat
- no treatment for individual lesions
- obvious deformities - corrective
osteotomy, limb lengthening ,
epiphysiodesis
- monitor indefinitely for malignant change