Documenti di Didattica
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of Bone Tumors:
Beyond Pattern
Recognition
Aunt
Minnie
Aunt Minnies
Aunt
Aunt Minnies
2 KEYS TO DIAGNOSIS
Grade and Tissue Type
GRADE
Local aggressiveness (margins)
Differentiation (maturity of matrix)
Metabolic activity
Size
TISSUE TYPES:
Bone
Cartilage
Round cells
BONE MARGINS
INVOLUTING INFLAMMATORY
Non-ossifying Chondroblastoma
fibroma
Eosinophilic Abscess/Eosinophilic
granuloma granuloma
MATRIX:
BONE
MICROSCOPIC
Cloud-like
Fluffy
MACROSCOPIC
Spicules
Cortex
DENSITY
Does not
exceed cortex
BONE LESION GRADE
LOW :
Small lesion
Densely mineralized
Rare
HIGH:
Sparsely mineralized
Invasive
OSTEOMA
ADULTS
SLOW GROWTH
SYMPTOMS (if any) DUE TO MASS
MEDULLARY- Bone island
PERIOSTEUM-
SKULL Button osteoma, Sinuses >> LONG
BONES Ivory exostosis
Bone Islands
(osteopoikilosis
)
Uniform, mature
bone
Oval, long axis
parallel to stress
lines
Serrated, irregular
outlines
OSTEOMA
medullary
cortica periosteal
OSTEOBLASTOMA
CHILDREN, YOUNG ADULTS
PROGRESSIVE
PAINFUL
MEDULLARY > PERIOSTEAL
SPINE (blastic) > LONG BONES (lytic)
TRANSITION LESION
AGGRESSIVE, PSEUDOMALIGNANT
FORMS
OSTEOBLASTOMA
6 mths later
OSTEOBLASTO
MA
Aggressive
osteoblastoma
Aggressive
osteoblastoma vs OSA
17 male
Osteoblastoma
curettaged,
packed
? recurrence
Osteosarcoma
OSTEOSARCOMA:
a malignant tumor which
forms bone
CHILDREN, YOUNG ADULTS BUT
LONG TAIL
MOST FREQUENT 1
MALIGNANT BONE TUMOR
PAINFUL
MEDULLARY > PERIOSTEAL >
SOFT TISSUE > CORTEX
LONG BONES > SPINE
OSTEOSARCOMA
TYPES:
Location in Bone
MEDULLARY most common
INTRACORTICAL least common
SURFACE conventional, parosteal,
periosteal
SOFT TISSUE
SPECIAL SITES:
MANDIBLE, SPINE
OLDER, SOMEWHAT BETTER
PROGNOSIS
OSTEOSARCOMA
Intramedullary
PAROSTEAL OSA
LOW GRADE,
WELL DIFFERENTIATED BONE
PAROSTEAL
OSTEOSARCOMA:
Marrow invasion
SURFACE OSA:
(conventional)
Intracortical
Osteosarcoma
OSTEOSARCOMA
TYPES:
PRIMARY ETIOLOGY
SECONDARY
Genetic-
Li Fraumeni syndrome
Familial retinoblastoma
Pre-existing lesions
Paget sarcoma
Fibrous dysplasia
Dedifferentiation
Radiation
SECONDARY
SARCOMA
Initial 9 mths
SECONDARY
SARCOMA
Ax Cor T2 T1
postgado
OSTEOSARCOMA TYPES:
HISTOLOGY
OSTEOBLASTIC
CHONDROBLASTIC
FIBROBLASTIC
TELANGECTATIC
mmary #4: Many lesions contain more than one cell type !
OSTEOSARCOMA:
Osteobla Chondroblastic Telangiect
stic atic
OSTEOSARCOMA:
Imaging longitudinal extent
AVASCULAR
CARTILAGE TUMORS:
GRADE
LOW:
NODULES
MINERALIZATION THROUGHOUT
HIGH
UNCOMMON
DEGENERATED vs. DEDIFFERENTIATED
ALL:
INVASIVE IN BONE, PUSHING IN SOFT
TISSUES
CHONDROMA
ADULTS
MEDULLARY (enchondroma) >>
PERIOSTEAL SOFT TISSUE>> CORTICAL
SYMPTOMS
Medullary - none
Periosteal, Cortical - PAIN
SLOW OSSIFICATION
MULTIPLE LESIONS:
Olliers Disease
Maffuccis Disease
ENCHONDROMA
ENCHONDROMA
OSTEOCHONDROMA:
A GROWTH DISTURBANCE
CHILDREN, YOUNG ADULTS
Growth ceases at skeletal maturity
May (rarely) regress
SYMPTOMS (if any) DUE TO MASS OR
COMPLICATION:
Fracture
Bursitis
Malignant degeneration
LONG BONES >> FLAT BONES
SUBTYPES
Radiation
Trauma
Multiple hereditary
OSTEOCHONDROMA
OSTEOCHONDROMA
OSTEOCHONDROMA
Cartilage cap
OSTEOCHONDROMA
Medullary continuity
Cartilage cap
Points
wrong
PERIOSTEAL
CHONDROMA
PERIOSTEAL
CHONDROMA
CHONDROBLASTOMA
CHILDREN, YOUNG ADULTS
LONG BONE EPIPHYSIS (humerus,
femur)>> FLAT BONES
PAINFUL (prostaglandin)
GROWTH USUALLY LIMITED
CHONDROBLASTOMA
CHONDROBLASTOM
A
CHONDR
OMYXOID
FIBROMA
Usually resembles
non-ossifying
fibroma
Not usually
calcified
CHONDROSARCOMA
Third most common primary
malignant bone tumor
Older adults
Some pain
Long bones >> Flat bones
Mostly low, intermediate grade
CHONDROSARCOMA
SUBTYPES BASED ON
LOCATION
Lymphoma
Metastases
EWING TUMOR
Second most common 1 bone tumor
80% < age 20
Males > females
Survival
before chemotherapy 5-10%
current 70%
Subperiosteal
spread
Ewing Tumor
ROUND CELL TUMOR:
RAPID GROWTH AFTER
PATHOLOGICAL FRACTURE
2 mths later
ROUND CELL TUMOR:
RAPID GROWTH AFTER PATHOLOGICAL
FRACTURE
EWING TUMOR:
PROGNOSIS
GOOD PROGNOSTIC FEATURES:
Distal location
Rib
Good response to chemo (>90%
histological necrosis)*
BAD PROGNOSTIC FEATURES:
Large (>8-10 cm or 100 ml)
Central (especially pelvic tumors)
* Wunder, Jay S.; Paulian, Gabe; Huvos, Andrew G.; Heller, Glenn; Meyers, Paul A., and Healey, John
H. The histological response to chemotherapy as a predictor of the oncological outcome of operative
treatment of Ewing Sarcom. Journal of Bone and Joint Surgery. 1998 Jul; 80-A(7):1020-1033
EWING FAMILY LYMPHOMA
AGE 5-30
AGE 20-60
PAINFUL, OFTEN SYSTEMIC
SYMPTOMS OFTEN NOT VERY SICK
T(11;22) TRANSLOCATION
EWING / PNET:
Soft tissue primary
Before Chemotherapy: 4/26/00