Patho Exam 2:Bone Tumors & Tumor-Like Lesions

Question 1

Bone Forming Tumors

Answer 1

Osteoma
Osteoid Osteoma & Osteoblastoma
Osteosarcoma

Question 2

Cartilage Forming Tumors

Answer 2

Osteochondroma
Chondromas
Chondroblastoma
Chondromyxoid Fibroma
Chondrosarcoma

Question 3

Fibrous & Fibro-Osseous Tumors

Answer 3

Fibrous Cortical Defect & Non-Ossifying Fibroma
Fibrous Dysplasia
Fibrosarcoma Variants

Question 4

Miscellaneous Tumors

Answer 4

Ewing Sarcoma/Primitive Neuroectodermal Tumor

Giant-Cell Tumor

Question 5

Bone Forming Tumors
Benign:
Malignant

Answer 5

Benign:
Osteoma
Osteoid Osteoma
Osteoblastoma

Malignant
Osteosarcoma

Question 6

Osteoma

Answer 6

Osteoma

Question 7

what is an Osteoma

Answer 7

Benign & slow growing of cortical dense bone

Question 8

what age is most likely to have osteomas?

Answer 8

Middle age

Question 9

where are osteomas commonly fount?

Answer 9

Most common on or inside skull and facial bones

Question 10

what are the tumor characteristic of osteomas?
Consist of

Some variants have

location:

shape

Difficult to distinguished from

Answer 10

Consist of woven and lamellar bone deposited in a cortical pattern with haversian like systems

Some variants have trabecular bone

From subperiosteal or endosteal surfaces

Round to oval sessile tumor

Difficult to distinguished from REACTIVE BONE looks a lot like osteoid of new bone growth

Question 11

Osteoma
Subtypes
1:
2;
3:
4:

Answer 11

Calvarial and mandibular osteomas
Osteomas of the sinonasal and orbital bones
Bone islands occurring in medullary bone
Surface osteomas of long bones

Question 12

Osteoid Osteoma

Answer 12

Osteoid Osteoma

Question 13

what are Osteoid Osteoma

Answer 13

Benign bone tumors with more compression than an Osteoma

Question 14

what age is likely to have a Osteoid Osteoma

Sex?

Answer 14

Age: Teens & 20s (75% are < 25 years of age)

Males > Females (2:1 to 3:1)

Question 15

Common location for a Osteoid Osteoma

Answer 15

Diaphyseal Cortex of tubular bones of leg

Appendicular skeleton & posterior elements of spine

Femur and tibia involved in 50% of cases (mostly in cortex)

Question 16

what are the tumor characteristic of Osteoid Osteoma?
describe:

Abnormalities in what xsomes?

Answer 16

Has a NIDUS < 2 cm diameter

Central mineralization: Thin irregular trabeculae of woven bone
Stroma contains Osteoclasts and Osteoblasts
Surrounded by reactive bone

Abnormalities of Chr 22 & deletion of 17

Question 17

what are the manifestations of Osteoid Osteoma?
-
-
- pain is relieved by: 
What is the treatment?

Answer 17

Manifest with pain (nocturnal)
Pain due to osteoblast production of PGE2
Pain relieved with ASA

Treatment
Radioablation or Surgical excision

Question 18

Osteoblastoma

Answer 18

Osteoblastoma

Question 19

Osteoblastoma
age: 
sex: 
location
Tumor characteristics: 

-

Answer 19

Benign
Age: 10-35 years of age
Males = Females
Location: Spine and long bones

Tumor Characteristics
NO Nidus & No bony reaction
> 2cm in diameter

Chromosomal abnormalities
MDM2 gene amplification: receptor of p53
TP53 gene deletion

mdm2 gene which regulates p53 tumor suppressor therefore if increased mdm then decreased p 53

Question 20

Osteoblastoma
Manifestation
Treatment:

Answer 20

Dull achy pain
Not relieved by ASA

Malignant transformation associated when treated with radiation

Question 21

Osteosarcoma (osteogenic sarcoma)
most common:
Age:
sex:

Answer 21

Malignant
Most common Primary malignant tumor of bone (exclusive of myeloma and lymphoma)

Age: bimodal
75% in persons < 20 years
Elderly with predisposing conditions:

Males > females (1.6:1)

Question 22

Osteosarcoma (osteogenic sarcoma)
Location:
-
-elderly:

Answer 22

Metaphyseal of long bones of extremities
>50% occur around knee

Elderly
Flat bones and long bones equally

Question 23

Osteosarcoma (osteogenic sarcoma)

Pathogenesis

Answer 23

Genetic (70%)
Ploidy changes
Chromosomal aberrations: RB & p53
Germline mutations in RB have 1000 fold increased risk
Patients with Li-Fraumeni syndrome (germline p53 mutation) have greatly elevated incidence

INK4a codes for:

p16: cell cycle regulator
p14: aids and abets p53 function

Question 24

Treatment of ostosarcoma?

Answer 24

Differs depending on mutation if tx improperly with the incorrect chemo you can actually make it worse

Question 25

Osteosarcoma (osteogenic sarcoma) Tumor Characteristics Identified by anatomic portion of the bone from which they arise:ex: Classified by: Classified as Solitary Destroy: Spread in medullary canal: In most cases:

Answer 25

Identified by anatomic portion of the bone from which they arise: intramedullary, intracortical, or surface Classified by differentiation: Most are poorly Classified as primary or secondary:secondary is associated with pre existing condition Solitary Destroy surrounding cortices Spread in medullary canal: Infiltrate the marrow Histologic variants (osteoblastic, chondroblastic, fibroblastic, telangiectatic, small cell, and giant cell) In most cases: malignant cells with osteoblastic differentiation producing woven bone

Question 26

Osteosarcoma (osteogenic sarcoma) | Clinical Course

Answer 26

Painful Progressively enlarging sudden fracture might be initial symptom

Question 27

what will the X-ray for Osteosarcoma (osteogenic sarcoma) show?

Answer 27

Tumor breaks through cortex and lifts periosteum Reactive periosteal bone Codman triangle: triangular shadow between cortex and raised ends of periosteum

Question 28

if a osteosarcoma mets where to and how?

Answer 28

Hematogenous spread: 10-20% at time of dx Metastases: 98% who die of osteosarcoma have lung mets

Question 29

Osteosarcoma (osteogenic sarcoma) | Treatment

Answer 29

Multimodality Chemotherapy Due to metastasis at time of diagnosis. Chemo with limb sparing surgery: 60-80% 5-year disease free Surgery Does not increase 5 year survival

Question 30

BONE TUMORS:Cartilage Forming Tumors

Answer 30

BONE TUMORS:Cartilage Forming Tumors

Question 31

Benign Cartilage Forming Tumors

Answer 31

Osteochondroma Chondroma Chondroblastoma Chondromyxoid Fibroma

Question 32

Malignant Cartilage Forming Tumors

Answer 32

Chondrosarcoma

Question 33

``` Osteochondroma (exostosis) age: - - Sex: location: - - - ```

Answer 33

``` Benign: most common benign bone tumor Age: Solitary lesions: late adolescence to early adulthood Multiple lesions: childhood Males > Females (3:1) ``` Location: only in bones of endochondral origin Pelvis Scapulae Ribs

Question 34

Osteochondroma (exostosis) Tumor Characteristics Cartilage Capped: Solitary in ___% Cap composed of : Cartilage cap undergoes: what is all contained in the tumor outgrowth?

Answer 34

Cartilage Capped tumor attached to underlying skeleton by a bony stalk. Sessile or mushroom shaped Solitary in 85% Cap composed of benign hyaline cartilage Cartilage cap undergoes enchondral ossification: newly formed bone forming the inner portion of the head and stalk. Catilage, bone and marrow

Question 35

Osteochondroma (exostosis) | have multiple hereditary syndromes:

Answer 35

Hereditary exostoses: (EXT codes for proteins that function in biosynthesis of heparin sulfate proteoglycans) ( makes up the cartilage) Mutation of gain in function in EXT1 or EXT2 Mutation of inactivation in EXT1 Reduced expression of EXT1 or 2: Defective endochondral ossification leading to abnormal growth

Question 36

Osteochondroma (exostosis) | Clinical manifestations

Answer 36

Slow growing Painful Impinge nerves Stalk fractures

Question 37

Osteochondroma (exostosis) | Hereditary exostosis

Answer 37

Bones may be bowed and shortened: disturbance in epiphyseal growth: can effect one leg and not the other, one shorter than the other. May transform to chondrosarcoma Stop growing at time of growth plate closure

Question 38

Chondroma define: age: location:

Answer 38

``` Benign tumor of hyaline cartilage Age: 20-40s Location Most common intraosseous cartilage tumors Short tubular bones of hands and feet ```

Question 39

Chondroma: Tumor Characteristics Most Cartilaginous tissue well Well Peripheral Center can

Answer 39

Most solitary chondromas occur in metacarpals and phalanges of hands Cartilaginous tissue well differentiated with sparse chondrocytes. Well circumscribed nodules of cytological benign cartilage Peripheral ossification Center can calcify and die

Question 40

Chondroma types:

Answer 40

En-chondroma: Arise within the medullary cavity Subperiosteal or juxtacortical chondromas: Arise on surface of bone

Question 41

what is ollier disease:

Answer 41

Multiple Enchondroma

Question 42

what is maffucci syndrome?

Answer 42

multiple enchondromas with Hemangioma present

Question 43

``` Chondroma Clinical Features - - - Treatment: ``` Maffucci syndrome is at risk for developing

Answer 43

Most are asymptomatic: occasionally painful Occasional pathologic fractures Deformities Treatment depends on clinical situation: usually observation or curettage. Maffucci syndrome at risk of developing other types of malignancies (ovarian carcinomas and brain gliomas)

Question 44

Chondroblastoma Age: Sex: Location:

Answer 44

Benign: rare (>F (2:1) Location Proximal femur, tibia and humerus Pelvis and ribs in older patients

Question 45

Chondroblastoma | Tumor characteristics

Answer 45

Sheets of chondroblasts Mitotic activity & necrosis Tumor cells surrounded by hyaline matrix deposited in lace like configuration When matrix calcified: chicken wire pattern Bone destruction by stimulating osteoclastic resorption: may perforate the cortex

Question 46

Chondroblastoma Clinical manifestations Treatment:

Answer 46

Painful Due to location: joints Treatment Curettage May reoccur

Question 47

``` Chondromyxoid Fibroma define: age: sex: location: ```

Answer 47

``` Benign: RAREST of cartilage tumors Age: TEENS Male > Females Location: Most common in metaphysis of long tubular bones ```

Question 48

Chondromyxoid Fibroma | Tumor Characteristics?

Answer 48

Composed of chondroid, myxoid and fibrous tissue in variable amounts “ATYPIA”: large hyperchromatic nuclei (a lot of DNA

Question 49

Chondromyxoid Fibroma Clinical Course Treatment

Answer 49

Clinical Course Manifest with dull achy pain Treatment Treated with Curettage No threat of malignant transformation

Question 50

``` Chondrosarcoma: neoplastic cartilage Define: age: sex location: ```

Answer 50

Malignant tumor of cartilage From preexisting cartilage rest or enchondroma Age: 40s or older (average age of 45) Males> Females (2:1) 15% Arise from preexisting enchondroma or osteochrondroma Location Central portions of skeleton: pelvis, shoulder, ribs Rarely involves the distal extremities

Question 51

Chondrosarcoma: neoplastic cartilage Tumor Characteristics 3 anatomic variants

Answer 51

Central Chondrosarcoma Medullary cavity of pelvic bones, ribs and long bones May penetrate the cortex: extension beyond the periosteum is uncommon. Peripheral Chondrosarcoma Arises outside the bone, almost always in cartilaginous cap of an osteochondroma Frequent location: pelvis JUXTACORTICAL Metaphysis of long bones

Question 52

Chondrosarcoma: neoplastic cartilage- Tumor Characteristics Pathogenesis: Central chondrosarcoma: Secondary peripheral chondrosarcoma:

Answer 52

Numerous nonrandom chromosomal abnormalities Central chondrosarcoma Abnormalities of chromosome 9: CDKN2A (TSG) Secondary peripheral chondrosarcoma: tumors arising in the cartilaginous cap of an osteochondroma - Upregulation of PTHrP & Bcl-2 - P53 mutations

Question 53

Chondrosarcoma: neoplastic cartilage | Tumor Characteristics Histology

Answer 53

Malignant cartilage cells in various stages of maturity Expands by stimulating osteoclastic resorption of bone Often breaks through the cortex Rearrangement of chromosome 17 associated with high grades

Question 54

``` Chondrosarcoma: neoplastic cartilage Clinical course - - low grade: moderate grade high grade: ```

Answer 54

``` Painful Progressively enlarging masses Low grade: 5 year survival of 80% Moderate grade: 5 year survival 50% High grade: 5 year survival 20% ```

Question 55

Chondrosarcoma treatment:

Answer 55

Wide excision | Response to radiation and chemotherapy is poor