Bone Tumors Flashcards
How do bone tumors usually present?
nonspecific!
pain (worse at ngiht)
mass
pathologi fracture
aymptomatic
What ae the diagnostic factors from bone tumors?
- age
- sex
- skeletal location
- specific bone
- specific area of bone
- medullary cavity, cortex, juxtacortical
- epiphysis, metaphysis, diaphysis
What bone tumors are common in
children?
young adults?
elderly?
Children, adolecents: osteosarcoma, Ewing’s sarcoma
Young adults: Giant cell tumor
Elderly: Chondrosarcoma
What is a sclerotic (well-defined) margin in a radiologic pattern typically an indication of?
a benign, slowly growing neoplasm
what is an illdefined margin radiologically typically an indication of?
malignant, rapidly growing neoplasm
hat is a solid ivory pattern typically and indicator of?
malignant bone-matrix forming tumors
what are rings and arcs typically an indicator of
chondroid matric fomring tumors
what are 2 benign bone forming tumors and what are the differences in type of bone, size, pain and response to aspirin
- Osteoid Osteoma
- long bones, femur and tibia
- less than 2cm
- night pain
- responds to aspirin
- radiolucent lesion within sclerotic cortex
- Osteoblastoma
- vertebrae or long bone metaphysis
- more than 2 cm
- painful
- not responsive to aspirin
- expansile radio-lucency with mottling
what is this?
Osteoid osteoma
central area of immature bone formation. they are all osteoblasts that are producing osteoid that is becoming poorly formed bone
What is this?
osteoid osteoma- note the osteoblasts look normal!!
WHat is this?
osteoblastoma
- histologically siilar to osteoid osteoma
- circumscribed benign lesion in bone
- look like osteoid/osteoma
- area of immature bone being formed by osteoblats
What are bone-forming (osteogenic) tumors?
- rare group of tumors in comparison with carcinomas and hematopoietic tumors bc malignant bone tumor comprise 0.2% of all types of cancers
- represent an important percentage of potentially curable cancers following a multimodal therapy
Define the pathogenesis of an osteosarcoma:
- inherited mutant allele of Rb gene (hereditary Rb: marked increase (1000x) in OS
- mutation of p53 supressor gene
- Li-FraumeniL bone and soft tissue sarcomas, easryl onset breast cncer, brain tumors, leukemia
- Over expression of MDM2 (5-10%) INK4 and p16
- site of bone growht/disease (is pagez)
- prior irradiation
What is an osteosarcoma and what is the incdence and age distribution
- malignant mesenchymal tumor in which cells produce osteoid or bone
- 2000 new a year
- mos tcommon sarcoma of bone
- bimodal age distribution: M>F mean age is 15 2nd peak is 55-80
Where does an osteosarcomea usually ocur? where is spread common
- metaphysis of long bones
- femur, tibia, humerus, flat bones, spine (sometime polyostotic but usually not)
- Hematogenous spread to lungs is common (if not treated will happen for sure)
what are some characteristic of an osteosarcoma?
- poorly delineated
- bone destruction
- cortical disruption
- bone matrix
- soft tissue extension
- codman’s triangle (bone trying to make new cortex around the tumor)
What is the pathology of an osetosarcoma and what is the treatment?
- pathology: infiltrative tumor, extending into soft tissue, malignant cells producing osteoid
- treatment: neo-adjuvant chemotherapy and surgical resection
- prominent irregularly shaped nuclei a lot of mitotic activity that are often atypical, pleomorphism seen histologically
what is the prognosis of an osteosarcoma?
- post chemo: 60-65% 3-5 yr survivl for pts with non-metastatic disease
- En bloc resection following chemotherapy: >90% necrosis which leads to near 90% survival
What is an osteochondroma?
- the most common benign tumor of bone
- metaphysis of long bones
- malignancy is rare but increased risk in herditary multiple extoses
- autosomal dominant, usualyl EXT-1 (8q24)
- small growth that comes off the cortex with a cartilagenous cap
What is this?
where the cartilage cap is forming just looks like normal cartilage!
What is an enchondroma?
benign hyaline cartialge lesion
- enchondroma: intramedullary chondroma
- periosteal chondroma: juxtachortical chondroma (ie located on the cortica surface under the periosteum)
How do you diagnose and what is the treatment for enchondroma?
- usually aymptomatic, incidental, appendicular skeleton; small bones of hands and feet
- X-rays: lytic, lobulated, cortical thinning
- micro: lobules of hyaline cartilage, minimal typia
- treatmentL none, unless lesion shows change:
- symptomatic (onset of acute pain is felt to be circumstantial evidence that lesion is malignant)
- evidence of recent growth after skeletal maturity
what does and enchondroma look lik ehistologically?
typical chondrocytes in the lacunae, looks bland and benign
What point mutations adn diseases are frequently associated wth mutliple chondromatosis?
frequent point mutations in IDH1 or IDH2
- Ollier disease
- Maffucci syndrome
What is ollier disease
point mutation in IDH1 or IDH2, multiple chondromatosis
- multiple enchondromata
- tend to have regional distribution
- +/- severe skeletal malformation
What is Maffucci Syndrome
frequent poitn mutations in IDH1 or IDH2
- multiple echondromata and agiomata
- svere skeletal malformation
- higher incidednce of malignant transformation
what is a chondrosarcoma?
malignant tumor in which neoplastic cells produce a purely catilagenous matrix
second most common bone sarcoma (26% of bone sarcomas)
mainly older adults, but wide age range
central skeleton: pelvis and ribs, jumerus, femur
What is seen with a chondrosarcoma on imagin?
- medullary location
- frequent ly ontain calcifications, which tend to be lost in grade 3 tumors
- cortical erosions or destruction (pop-corn like) rings and arcs bc cartilage. look swhite.blusih bc cartilage
- occasional soft tissue extension
what is the pathology of chrondrosarcoma?
- generally more cellular and nuclei more pleomorphic than in enchondromas
- binucleation is frequent, but does not siffice for malignant diagnosis
- myxoid chaneg of chondroid matrix
**so remember endochondromas the chondrocytes looked pretty normal and there was a normal amount, now ther are way more and they look atypical and tehre are mitosis
in a chondrosarcoma ______ and _______correlate with behavior? also what is grading based on?
in chondrsarcoma size and grade correlate with behavior, also grade is based on degreee of cellularity and atypia
Chondrosarcoma grade 1
more cellular than enchondroma but more matrix than cells, still some atypia, but not much cells look fairly normal
Chondrosarcoma grade 2
more atypia with some focal areas that are dense with cellularity. areas of clear cut cartilage expression
chondrosarcoma grade 3
more severe atypia with mitoses more aggressive and fairly rare
how does 5 year survival relate to grade for a chondrsarcoma? what are the variants?
- higher grade=lower 5 yerr survival
- grade 1,2: 80-90%
- grade 3: 29% (pulmonary metastasis)
- Variants: de-differentiated, myxoid, clear cell, mesenchymal, juxtacortical
What is a non-ossifying fibroma? location and age? How is ti usually identitied
- common developmental cortical defect
- most comm space-occupying lesion of bone: 1/4, often multifocal
- tibia femur (metahysis) 1-3 decades
- eccentric lytic, peripheral sclerosis
- incidental finding or pathologic fracture
what pattern does a non-ossifying fibroma have?
storiform pattern
What is fibrous dysplasia?
- developmental arrest of bone
- monostotic: most common, seen in adolecents, ribs manidble femur
- polyostotic: infancy/childhood, crippling deformities, craniofacial involvement common
What is McCune-Albright Syndrome?
a fibrous dysplasia
- polyostotic FD with endocrinopathies and cafe-au-lait spots
- rare-form; F.M
- sexual precocity, acromegaliy, cushing syndrome
- activating germline mutations of GNAS (GTP-binding protein) results in excess cAMP leading to endocrine gland hyperfunction
what are 5 features of a fibrous dysplasi?
expansible
circuscribed
thinned cortes
“ground glass”
may be multiple
what is the pathology of fibrous dysplasia? What s the treatment?
- haphazard, curvilinear, randomly oriented woven bone trabeculae (“chinees characters”) surrounded by fibroblastic stroma
- no significant osteoblastic rimming
- (bone present in fibrous tissue)
- treatment: conservative except for is polyostotic then you scrape it out!
What is the incidence of Ewing Sarcoma/PNET? How does it present and where does it occur?
2nd most common malignant bone tumor in childhood
- adolecents, young adults M>F
- present as painful, often enlargin mass
- diaphysis of long tubular bones, ribs and pelvis
What is seen on X-ray with an Ewing-Sarcoma
destructive moth-eaten, premeative lesion with large soft tissue mass
onion skin pattern of periosteal reaction in response to rapid growth
What is the pathology of Ewing Sarcoma?
- sheets of primitve small roung blue cells with neural phenotype
- membranous CD99
- Contain abundant glycogen
- hemorrhage and necrosis common
What is the pathogenesis of Ewing Sarcome/PNET?
- EWS involve in over 95% of ES/PNET
- translocation: t(11;22) present in 85% of tumors
- EWS on 22q fused with FLI-1 transcription factor on 11q
Treatment of Ewing Sarcome/PNET? and 5 year survival for stage 1?
chemotherapy and surgery
radiation may be added
stage I: 5 year survival 70% with chemo/RT
Who gets Giant Cell Tumor of Bone?
young adults (20-4-yo), older adolesecents (skeletally matured)
females>males
Where do Giant cell tumors of the bone occur?
epiphyseal location: knee, proximal humerus, radius
most are benign, locally aggressive but may destroy cortex of bone and extend into soft tissue
What is seen histologically with a giant cell tumor of bone?
multinucleate giant cells, that look like osteoclasts
what is the most common malignant bone tumor especially in adults (20x more frequent that primary bone tumors)
metastatic bone tumors
What are metastatic bone tumors like?
- mostly multiple
- solitary lesions may mimic a primary bone tumor and precede discovery of its source
- 70% go to axial skeleton (skull, ribs, vertebral colum, sacrum)
- mostly lytic
- may be blastic (bone-forming): breast, prostate
- 80% breast, lung, thryoid, prostate, and kidney (BLT-KP)
what is the most common sarcoma in the bone?
osteosarcoma
what age raneg doe sosteosarcoma typically occur in?
children/adolescents
what is osteoid osteoma
painful bone lesion that is releived by aspirin
Li-Fraumeni syndrome, Ollier disease, Mafucci syndorme, and McCune-Albright syndrome are all associted with ________
bone lesions
