7- Bone Tumors Flashcards
Relatively rare group of tumors in comparison with carcinomas and hematopoietic tumors • Malignant tumors make up ~0.2% of all types of cancer
Bone tumors
What are some clinical presentations we would expect to see in bone tumors?
Usually nonspecific, may present: • Pain (any tumor;) • Mass (parosteal OS: painless, hard growing mass in popliteal fossa) • Pathologic fracture • Asymptomatic
tumors causes severe pain, worse at night, relieved by aspirin
osteoid osteoma:
What do we need to consider when Dx for bone tumors?
Age • Sex • Skeletal localization • Specific bone • Specific area of bone 1. Medullary cavity, cortex, juxtacortical 2. Epiphysis, metaphysis, diaphysis • Radiographic appearance
Osteosarcoma, Ewing’s sarcoma seen commonly in:
childhood, adolescence
Giant cell tumor seen in
young adults
Chondrosarcoma seen in
elderly
Pattern on radiology:
Sclerotic vs Ill-defined
Sclerotic = well defined margins, slow growing, usually benign
Ill-defined = fuzzy border, fast growing and usually metestatic
Review for location of bone tumors
Review location of bone tumors
Solid, ivory-like pattern is
generally seen in
malignant
bone matrix-forming tumors
Rings and arcs are generally seen
in
chondroid matrix-forming
tumors
OSTEOID OSTEOMA
where
size
X-ray apperance
special about it
•Long bones, femur &
tibia
•< 2 cm
•Night pain
•Responds to aspirin
•Radiolucent lesion within
sclerotic cortex
Osteoblastoma
Where
size
x-ray apperance
special about
Vertebrae or long bone
metaphysis
•> 2 cm
•Painful
•Not responsive to aspirin
•Expansile radio-lucency
with mottling
Pt come in with really bad night pain in her leg, the only thing that helps is taking aspirin. What do you expect to see on xray
indicitave of osteoid osteoma; will see radiolucent lesion within the Sclerotic cortex
benign tumor of long bones, usually more then 2 cm
Mottled appearance larger then 2 cm. Painful and aspirin doesn’t help
Osteoblastoma; seen in long bone metaphysis and more then 2 cm
What do we expect to see in histology of osteoid osteoma
All immaure osteoids
Malignant mesenchymal tumor in which cells
produce osteoid or bone.
Osteosarcoma
Most common bone sarcoma
Osteosarcoma
• ~2000 new cases per year in US.
Age distribution of osteosarcoma
sex preference?
Peak incidence?
- Bimodal age distribution, M>F
- Mean age: 15 (60% 10-20)
- 2nd peak: 55-80
Location of osteosarcoma
Metaphysis of long bones
• Femur, tibia, humerus (56%); flat
bones, spine (older patients)
• May be polyostotic (not common)
• Hematogenous spread to lungs is
common
Most common spread of osteosarcoma is:
Hematogenous spread to lungs is
common
Pathogenesis of Osteosarcoma:
3 genetic components
1 disease you see it in
Inherited mutant allele of RB gene
• Hereditary RB: marked increase (1000X) in OS
• Mutation of p53 suppressor gene
• Li-Fraumeni: bone and soft tissue sarcomas, early
onset breast cancer, brain tumors, leukemia
• Overexpression of MDM2 (5-10%); INK4 and
p16
• Sites of bone growth/disease (i.e. Paget dz.)
• Prior irradiation
Describe what to expect in Xray of osteosarcoma
- Poorly delineated
- Bone destruction
- Cortical disruption
- Bone matrix
- Soft tissue extension
- Codman’s triangle
Infiltrative tumor… extends to soft tissues. Has malignant cells producing osteoid
Osteosarcoma
Tx of osteosarcoma
• TREATMENT
• Neo-adjuvant chemotherapy and surgical
resection
Histology of osteosarcoma
Bone formation and in higher power you see nuclei and convoluted and variable size, see tri polar mitosis
Prognosis of osteosarcoma post chemo
65% survial for pts with non-metastatic disease
Prognosis of osteosarcoma with en-bloc and chemo follow up
En-bloc resection following chemotherapy: >90%
necrosis near 90% survival
Most common benign tumor of bone
Osteochondroma
Location of osteochondroma
metaphysis of long bone
PT has multiple osteochondromas. Do we worry about maligant transformation? Whats the linked etiology
Yes, increased to 40%
Autosomal dominant most commly secondary to EXT-1 or a 8q24 muation
What does a osteochondroma look like
The cartilage, bone and marrow all grow out together in a little outpouching
Benign hyaline cartilage lesion
Located intramedullary
– Enchondroma
juxtacortical chondroma is located on cortical surface under periosteum. Called:
– Periosteal chondroma
Descibe the lesion and what it is
Lytic, lobulated, cortical thinning.
Endochondroma
Pt comes in to get a Bone density scan… you notice some irregularites in her hand so you take an xray and see this:
Recommended tx
Endochondroma, present in hand, feet, appendicular skeleton… IG incidental finding
TX
none, unless lesion shows changes:
1. Symptomatic (onset of acute pain is felt to be
circumstantial evidence that lesion is malignant)
2. Evidence of recent growth after skeletal maturity
Expected HE for endochondroma:
lobules of hyaline cartilage, minimal
atypia.
Pt comes in with multilple chondromatosis: suspected genetic alteration:
Frequent point mutations in IDH1 or IDH2
– Multiple enchondromata
– Tend to have regional distribution
– ± severe skeletal malformation
Olliers disease
– Multiple enchondromata + angiomata
– Severe skeletal malformation
– Higher incidence of malignant transformation
Maffucci’s syndrome
Malignant tumor in which neoplastic cells
produce a purely cartilaginous matrix
Chondrosarcoma
Age range and location of chondrosarcomas
Wide range of ages, mainly older adults
• Mostly above 40-50 years
• Peak during 6th- 7th decades
• Central skeleton: pelvis and ribs (45%); humerus,
femur (metaphysis, diaphysis)
Most common and second most common bone sarcoma
Most common is osteosarcoma
chondrosarcoma second
Describe imaging and Dx
Location
Cortical invovlement
soft tissue invovlement
Chondrosarcoma = popcorn like
Medullary location
• Frequently present calcifications, which tend
to be lost in grade 3 tumors
• Cortical erosion or destruction
• Occasional soft tissue extension
Histology of chondrosarcoma
Nuclei:
Any changes?
Generally more cellular and nuclei more
pleomorphic than in enchondromas.
• Binucleation is frequent, but does not suffice
for malignant diagnosis.
• Myxoid change of chondroid matrix.
What two things correlate with behavior of chondrosarcoma
Size (< or > 10 cm) and grade correlate
with behavior
Grading (1-3) of chondrosarcoma based on
degree of
cellularity and atypia
5 year survival outcomes for chondrosarcoma
• Grades 1,2:
• Grade 3:
80-90%
29% (pulmonary metastasis)
Variants of chondrosarcoma depend on:
de-differntiaton, myxoid, clear cells, mesenchymal, juxtacortical
Common developmental cortical defect
• Most common space-occupying lesion of bone: 1
of every 4 individuals
• Multifocal in 25% of cases
Non-ossifying fibrosarcoma
Location of non-ossifying fibroma and age to get them
Tibia, femur (metaphysis); 1st – 3rd decades.
• Eccentric, lytic, peripheral sclerosis
• Incidental finding or pathologic fracture
Descibe this and expected Dx
lots of spindle cells, no ryhyme or reason. Looks like Starry night
Non-ossifying fibroma
• Developmental arrest of bone
FIBROUS DYSPLASIA
•
Monostotic fibrous dysplasia seen most in what age group and where
- Most common, seen in adolescents
- Ribs, mandible and femur
Ployostotic fibrous dysplasia most common in which age and what location
• Infancy/childhood
• Crippling deformities, craniofacial involvement
common
• Polyostotic FD with endocrinopathies and café-
au-lait spots
McCune-Albright syndrome:
McCune Albright more common M, F
Other findings assocated with it
More females
sexual precocity, Cushings, acromegaly,
CAuse of McCune albright
Germline mutation of GNAS that causes excess cAMP leading to endocrine gland hyperfunction
X ray findigs in Fibrous dysplasia
- Expansile
- Circumscribed
- Thinned cortex
- “Ground glass”
- May be multiple
Histology of Fibrous Dysplasia
PATHOLOGY:
• Haphazard, curvilinear, randomly oriented
woven bone trabeculae (“Chinese
characters”), surrounded by fibroblastic
stroma
• No significant osteoblastic rimming
Tx for fibrous dysplasia
- TREATMENT:
- Conservative, except polyostotic form
Second most common malignant bone tumor
in childhood
• 16% of primary bone malignancies
• Adolescents, young adults; M>F
• Present as painful, often enlarging mass
Ewing Sarcoma
Location of Ewing Sarcoma
Diaphysis of long tubular bones, ribs and pelvis
Xray apperance of Ewings Sarcoma
X-RAY:
• Destructive moth-eaten, permeative
medullary lesion with large soft
tissue mass
• “Onion-skin” pattern of periosteal
reaction in response to rapid growth
Sheets of primitive small round blue
cells with neural phenotype
• Membranous CD99 staining
• Contain abundant glycogen
• Hemorrhage and necrosis common
EWing Sarcoma
Histologic findings with Ewing sarcoma
• Sheets of primitive small round blue
cells with neural phenotype
• Membranous CD99
• Contain abundant glycogen
• Hemorrhage and necrosis common
85% of EWS tumors involve this gene mutation
EWS involved in >95% of ES/PNET
• t(11;22) present in 85% of the tumors
• EWS on 22q fused with FLI-1 transcription factor on 11q
on 22q fused with FLI-1 transcription factor on 11q
EWS translocation
Tx for EWS
Chemotherapy and surgery
• Radiation therapy may be added
• Stage I- 5 year survival 70% with chemo/ RT
Age group targeted in Giant Cell tumor
Young adults (20-40 years), older adolescents (skeletally matured); F\>M
Location of Giant Cell Tumor
• Epiphyseal location
• Knee, proximal humerus, radius
• Most are benign, locally aggressive
• May destroy cortex of bone and extend
into soft tissue
Histology of Giant Cell Tumor
Multinucleated cells
Most common malignant bone tumor, especially
in adults (20X more frequent than primary bone
tumors)
• Mostly multiple
Metestatic bone tumors
Solitary lesions may mimic a primary bone tumor
and precede discovery of its source
70% go to;
– 70% go to axial skeleton (skull, ribs, vertebral column, sacrum)
– Mostly lytic
– May be blastic (bone-forming): breast, prostate
Most bone lesions are not primary but come from:
80%- breast, lung, thyroid, prostate and kidney
(BLT-KP)
