bone tumors Flashcards
bone tumors clinical presentation
nonspecific, presents with pain (osteoid osteoma is sever pain at night), mass, pathologic fracture, asymptomatic
Ages
Children: osteosarcoma, ewings sarcoma
Young adults: giant cell tumor
Elderly: chondrosarcoma
Radiologic patterns
sclerotic margin is generally an indication of benign, slowly growing neoplasm
Ill defined margin is indication of malignant, rapidly growing neoplasm
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 and osteoblastoma
Osteoid osteoma: long bones, femur and tibia, smaller, night pain, responds to aspirin, radiolucent lesion within sclerotic cortex
Osteoblastoma: vertebrae or long bone metaphysis, large, painful, nonresponsive to aspirin, expansile radiolucency with mottling
Bone forming osteogenic tumors
rare in comparison with carcinomas and hematopoietic tumors
Malignant bone tumors (.2% of all cancerss
Osteosarcoma
malignant mesenchymal tumor, cells make osteoid/bone, 2000 new cases per year in US
Most common sarcoma of bone!
Bi modal age distribution
Males more than females
Mean age is 15 then 55-80
Metaphysis of long bones, may be polyostotic (not common)
Hematogenous spread to lungs is common
Pathogenesis: inhereted mutant allele of RB gene, mutation of p53 suppressor gene (liFraumeni–bone and soft tissue sarcomas, early onset breast cancer, brain tumors, leukemia), Over expression of MDM2, sites of bone growth/disease (Pagets disease), prior irradiation
Osteosarcoma diag and treatment
X ray: poorly dilineated, bone destruction, cortical disruption, bone matrix, soft tissue extension, codmans triangle
Pathology: infiltrative tumor, extending into soft tissue, malignant cells producing osteoid
Treat: Neo-adjuvant chemotherapy and surgical resection
Prognosis: mets to lung, pleura, other bones, CNS, post chemo (60-65% 3-5 yr survival for pts with non metastatic disease), en bloc resection following chemotherapy>90% necrosis–> near 90% survival
Osteochondroma
Most common benign tumor of bone
Metaphysis of long bones
Malignant transformation is rare, but increased risk in hereditary multiple exostoses (Autosoma dominant)
Kinda looks like an out pouching of metaphysis
enchondroma
Benign hyaline cartilage lesion
Enchondroma=intramedullary chondroma
Periosteal chondroma = juxtacortical chondroma (located on the cortical surface under the periosteum)
Usually asymptomatic, appendicular skeleton (small bones of hands and feet)
X rays- lytic lobutlated cortical thinning
Micro: lobules of hyaline cartilage, minimal atypia
Treatment: non unless lesion is changing,
multiple chondromatosis
Point mutations on IDH1 and IDH2
Ollier disease: multiple enchondromata, tend to have regional distribution, w/or s/o severe skeletal malformation
Maffucci syndrome: multiple enchondromata and angiomata, severe skeletal malformation, higher incidence of malignant transformation
Chondrosarcoma
Malignant tumor: neoplastic cells produce pure cartilage
Second most common bone sarcoma
Wide range of ages mainly older adults (peaks at seniors)
Central skeleton: pelvis and ribs
Imaging: Medullary location, frequently contain calcifications, tend to be lost in grade 3 tumors, cortical erosion/destruciton, occasional soft tissue extension
Popcorn like radiograph
Chondrosarcoma pathology
generally more cellular and nuclei more pleomorphic than in enchondromas
Bi nucleation is frequent, but does not suffice for malignant diagnosis
Myxoid change of chondroid matrix
non ossifying fibroma
Common developmental cortical defect, most common space occupying lesion of bone 25% of pop, multifocal common
tibia, femur, in 1st 30 years
Eccentric, lytic peripheral
Incidental finding or pathologic fracture
Storiform pattern on histology (stary night)
Fibrous dysplasia
Developmental arrest of bone
Monostotic: most common seen in adolescents, ribs, mandible and femur
Polyostotic: infancy/childhood, crippling deformities, craniofacial involvement common
Mccune-albright syndrome: polyostotic FD w/endocrinopathies and cafe au lait spots, rare (F>M), sexual precocity, acromegaly, Cushings, activating germline mutations of GNAs result in excess cAMP –> endocrine gland hyperfunction
Expansile, circumscribed, thinned cortex, ground glass, may be multiple
Pathology: haphazard curvilear random chinese characters, surrounded fibroblastic stroma, no significant osteoblastic rimmin
Treatment: conservative except polyostotic form
Ewing sarcoma/PNET
second most common malignant bone tumor in childhood
Adolescents, young adults m>f, present as painful, often enlarging mass
Diaphysis of long tubulat bones, ribs, pelvis
X ray: destructive moth eaten, permeative medullary lesion with large soft tissue mass
Onion skin pattern of periosteal reaction in response to rapid growth
Path: sheets of primitive small round blue cells with neural phenotype (CD99) contain abundant glycogen, hemorrhage and necrosis common
EWS in 95%, t (11,22)
treatment: chemotherapy and surgery, radiation therapy may be added, stage 1- 5 year survival 70%
Giant cell tumor of bone
young adults 20-40 yrs older adolescents, skeletally matured (F>M)
Epiphyseal location on knee humerous, radius
Most are benign, locally aggressive, may destroy cortex fo bone and extend into soft tissue
