Bone Tumors Flashcards by Mallory Koula

Are bone tumors common?

Not in comparison to carcinomas and hematopoietic tumors

Malignant bone tumors comprise (0.2%) of all types of cancer

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How do bone tumors commonly present?

Usually non-specific:

Pain

Mass

Pathologic fracture

Asymptomatic

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What are some diagnostic factors to think about with bone tumors?

Age

Sex

Skeletal localization: specific bone, specific area of bone

Radiographic appearance

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What age do Osteosarcoma, Ewing’s sarcoma generally affect?

Childhood, adolescence

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What ages do giant cell tumors generally affect?

Young adults

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What ages do Chondrosarcomas generally affect?

Elderly

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What is a slcerotic margin an indication of?

Benign, slowly-growing neoplasm

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What is an ill-defined margin indicative of?

Malignant, rapidly-growing neoplasm

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What does it mean to see solid, ivory-like patterns on radiographs of bone?

Generally seen in malignant bone matrix-forming tumors

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What does it mean to see rings and arcs on radiographs of bone?

Generally seen in chondroid matrix-forming tumors

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What is the most common primary bone tumor?

Osteochondroma

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Benign bone-forming tumors

Osteoid Osteoma & Osteoblastoma

Histologically very similar

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Located in long bones, femur & tibia

<2cm

Night pain

Responds to aspirin

Radiolucent lesion within sclerotic cortex

Osteoid Osteoma

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Located in vertebrae or long bone metaphysis

>2cm

Painful

Not responsive to aspirin

Expansile radio-lucency with mottling

Osteoblastoma

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Malignant mesenchymal tumor in which cells produce osteoid or bone

35% of bone sarcomas

Osteosarcoma

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What population does osteosarcoma generally affect?

M>F

Mean age: 15 (60% 10-20)

2nd peak: 55-80

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Where does osteosarcoma usually reside?

Metaphysis of long bones (femur, tibia, humerous; flat bones, spine - older pts)

May be polyostotic – multiple bone sites (not common)

Hematogenous spread to lungs is common

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Where does osteosarcoma commonly spread hematogenously?

To the lungs

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What are some characteristics that may predispose someone to osteosarcoma?

Inherited mutant allele of RB gene: marked increased (1000X) in OS
Mutation of p53 suppressor gene: Li-Fraumeni
Overexpression of MDM2 (5-10%); INK4 and p16
Sites of bone growth/disease (Paget’s)
Prior irradiation

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What are some radiographic findings that are commonly indicative of osteosarcoma?

Poorly delineated
Bone destruction
Cortical disruption
Bone matrix
Soft tissue extention
Codman’s triange: radiographic sign, rapidly growing mass with periosteum trying to grow around it

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What is found pathologically with osteosarcomas?

Infiltrative tumor, extending into soft tissue

Malignant cells producing osteoid

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What is the treatment for osteosarcoma?

Neo-adjuvant chemotherapy and surgical resection (amputation)

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What was the prognosis for Osteosarcoma prior to 1970? What is it now, after the advent of chemotherapy?

Prior to 1970: 5 year survival 20%; most relapsed in lung within 6 months after primary amputation; mets to lung pleura, other bones, CNS

Post chemotherapy: 60-65% 3-5 year survival for patients with non-metastatic disease; En-bloc resection following chemotherapy: >90% necrosis – near 90% survival

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Most common benign tumor of bone

Osteochondroma

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Where are osteochondromas commonly located in bones?

Metaphysis of long bones

Is transformation of osteochondroma to malignancy common?

No -- it's rare (\<1%) but increased risk in hereditary multiple exostoses --\> Autosomal dominant, commonly secondary to mutations in EXT-1 (8q24)

Benign hyaline cartilage lesion (2 kinds)

Enchondroma = intramedullary chondroma Periosteal chondroma = juxtacortical chondroma (located on the cortical surface under the periosteum)

How do enchondromas usually present?

Usually asymptomatic, incidental finding. Appendicular skeleton; small bones of hands and feet.

How do enchondromas appear on x-ray? In microanalysis?

X-ray: lytic, lobulated, cortical thinning Micro: lobules of hyaline cartilage, minimal atypia

How are enchondromas treated?

Nothing! Unless lesion shows changes: 1. become symptomatic 2. evidence of recent growth after skeletal maturity

What can lead to Multiple Chondromatosis?

Frequent point mutations in IDH1 or IDH2 Ollier's disease: multiple enchondromata, tend to have regional distribution, could have severe skeletal malformation Maffucci's syndrom: multiple enchondromata + angiomata, severe skeletal malformation, higher incidence of malignant transformation

Malignant tumor in which neoplastic cells produce a purely carilaginous matrix 2nd most common bone sarcoma (~26%)

Chondrosarcoma

What population do Chondrosarcomas commonly affect?

Wide range of ages, mainly older adults (mostly 40-50 years) Peak at 6-7th decades

Where do chondrosarcomas generally occur?

Pelvis and ribs (45%); humerus, femur (metaphysis, diaphysis)

What do chondrosarcomas look like on radiographs?

Medullary location Frequently present calcifications, which tend to be lost in grade 3 tumors Cortical erosion or destruction Occasional soft tissue extension "Popcorn-like appearance"

How do chondrosarcomas appear pathologically?

More cellular and nuclei more pleomorphic than enchondromas Binucleation is frequent but does not suffice for malignant diagnosis Myxoid change of chondroid matrix

How is grading dictated in chondrosarcomas?

Size (\< or \> 10 cm) and grade correlate with behavior Grading (1-3) based on degree of cellularity and atypia

What is the prognosis for chondrosarcoma?

Grades 1,2: 80-90% Grade 3: 29% (pulmonary metastasis) Variants: de-differentiated, myxoid, clear cell, mesenchymal, juxtacortical

Common developmental cortical defect Most common space-occupying lesion of bone: 1 of every 4 individuals

Non-ossifying fibroma

What are some common locations for non-ossifying fibromas?

Tibia, femur (metaphysis)

When do non-ossifying fibromas occur in life?

1st-3rd decade

What is the histological appearance called of non-ossifying fibromas?

"Starry-sky"

Developmental arrest of bone Can be monostotic (most common, in adolescents, ribs, mandible and femur) Or Polyostotic (in infancy/childhood, crippling deformities, cranio facial involvement common)

Fibrous dysplasia

What is McCune-Albright syndrome? More common in F or M?

Polyostotic fibrous dysplasia with endocrinopathies and cafe-au-lait spots F\>M Sexual precocity, acromegaly, Cushing syndrome

Genetically, how does McCune-Albright occur?

Activating germline mutations of GNAS (GTP-binding protein) results in excess cAMP leading to endocrine gland hyperfunction

How does fibrous dysplasia appear on x-ray?

* Expansile * Circumscribed * Thinned cortex * "ground glass" * May be multiple

What does the pathology of fibrous dysplasia look like?

Haphazard, curvilinear, randomly oriented woven bone trabeculae ("chinese characters"), surrounded by fibroblastic stroma No significant osteoblastic rimming

How do you treat fibrous dysplasia?

Conservative, except polyostotic form

Second most common malignant bone tumor in childhood Adolescents, young adults; M\>F Present as painful, often enlarging mass

Ewing Sarcoma/ PNET

What part of bone does Ewing Sarcoma affect?

Diaphysis of long tumular bones, ribs, pelvis

How does Ewing Sarcoma appear on X-ray?

Destructive moth-eaten, permeative medullary lesion with large soft tissue mass "Onion-skin" pattern of periosteal reaction in response to rapid growth

How does Ewing Sarcoma present pathologically?

* Sheets of primitive small round blue cells with neural phenotype * Membranous CD99 * Contain abundant glycogen * Hemorrhage and necrosis common

What genetic translocation is associated with Ewing Sarcoma?

t(11;22): EWS on 22q fused wtih FLI-1 transcription factor on 11 Present in 85% of tumors

What is the treatment for Ewing Sarcoma?

Chemotherapy and surgery Radiation therapy may be added Stage 1: 5 year survival 70% with chemo/RT

What population do giant cell tumors of bone commonly affect?

Young adults (20-40 years) Older adolescents (skeletally matured) F\>M

Where do giant cell tumors generally presents?

Epiphyseal location Knee, proximal humerus, radius

Are giant cell tumors of bone generally malignant or benign?

Most are benign, locally aggressive May destroy cortex of bone and extend into soft tissue

Are primary or metastatic bone tumors more likely?

Malignant bone tumors are 20x more frequent than primary bone tumors -- especially in adults

What are the main types of cancers that are known to cause metastatic bone tumors?

BLT-KP Breast, Lung, Thyroid, Kidney, Prostate