7- Bone Tumors Flashcards by Meghan May

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

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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

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tumors causes severe pain, worse at night, relieved by aspirin

osteoid osteoma:

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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

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Osteosarcoma, Ewing’s sarcoma seen commonly in:

childhood, adolescence

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Giant cell tumor seen in

 young adults

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Chondrosarcoma seen in

 elderly

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Pattern on radiology:

Sclerotic vs Ill-defined

Sclerotic = well defined margins, slow growing, usually benign

Ill-defined = fuzzy border, fast growing and usually metestatic

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Review for location of bone tumors

Review location of bone tumors

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Solid, ivory-like pattern is
generally seen in

malignant
bone matrix-forming tumors

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Rings and arcs are generally seen
in

chondroid matrix-forming
tumors

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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

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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

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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

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Mottled appearance larger then 2 cm. Painful and aspirin doesn’t help

Osteoblastoma; seen in long bone metaphysis and more then 2 cm

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What do we expect to see in histology of osteoid osteoma

All immaure osteoids

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Malignant mesenchymal tumor in which cells
produce osteoid or bone.

Osteosarcoma

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Most common bone sarcoma

Osteosarcoma

• ~2000 new cases per year in US.

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Age distribution of osteosarcoma

sex preference?

Peak incidence?

Bimodal age distribution, M>F
Mean age: 15 (60% 10-20)
2nd peak: 55-80

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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

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Most common spread of osteosarcoma is:

Hematogenous spread to lungs is
common

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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

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Describe what to expect in Xray of osteosarcoma

Poorly delineated
Bone destruction
Cortical disruption
Bone matrix
Soft tissue extension
Codman’s triangle

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Infiltrative tumor… extends to soft tissues. Has malignant cells producing osteoid

Osteosarcoma

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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)