Patho Exam 2:Bone Tumors & Tumor-Like Lesions Flashcards Preview

Clinical Medication: HEENT > Patho Exam 2:Bone Tumors & Tumor-Like Lesions > Flashcards

Flashcards in Patho Exam 2:Bone Tumors & Tumor-Like Lesions Deck (55):
1

Bone Forming Tumors

Osteoma
Osteoid Osteoma & Osteoblastoma
Osteosarcoma

2

Cartilage Forming Tumors

Osteochondroma
Chondromas
Chondroblastoma
Chondromyxoid Fibroma
Chondrosarcoma

3

Fibrous & Fibro-Osseous Tumors

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

4

Miscellaneous Tumors

Ewing Sarcoma/Primitive Neuroectodermal Tumor
Giant-Cell Tumor

5

Bone Forming Tumors
Benign:
Malignant

Benign:
Osteoma
Osteoid Osteoma
Osteoblastoma

Malignant
Osteosarcoma

6

Osteoma

Osteoma

7

what is an Osteoma

Benign & slow growing of cortical dense bone

8

what age is most likely to have osteomas?

Middle age

9

where are osteomas commonly fount?

Most common on or inside skull and facial bones

10

what are the tumor characteristic of osteomas?
Consist of

Some variants have

location:

shape

Difficult to distinguished from

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

11

Osteoma
Subtypes
1:
2;
3:
4:

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

12

Osteoid Osteoma

Osteoid Osteoma

13

what are Osteoid Osteoma

Benign bone tumors with more compression than an Osteoma

14

what age is likely to have a Osteoid Osteoma
Sex?

Age: Teens & 20s (75% are < 25 years of age)
Males > Females (2:1 to 3:1)

15

Common location for a Osteoid Osteoma

Diaphyseal Cortex of tubular bones of leg

Appendicular skeleton & posterior elements of spine

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

16

what are the tumor characteristic of Osteoid Osteoma?
describe:

Abnormalities in what xsomes?

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

17

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

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

Treatment
Radioablation or Surgical excision

18

Osteoblastoma

Osteoblastoma

19

Osteoblastoma
age:
sex:
location
Tumor characteristics:

Chromosomal abnormalities
-
-

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

20

Osteoblastoma
Manifestation
Treatment:

Dull achy pain
Not relieved by ASA


Malignant transformation associated when treated with radiation

21

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

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)

22

Osteosarcoma (osteogenic sarcoma)
Location:
-
-elderly:

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

Elderly
Flat bones and long bones equally

23

Osteosarcoma (osteogenic sarcoma)
Pathogenesis

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

24

Treatment of ostosarcoma?

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

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:

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

26

Osteosarcoma (osteogenic sarcoma)
Clinical Course

Painful
Progressively enlarging
sudden fracture might be initial symptom

27

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

Tumor breaks through cortex and lifts periosteum

Reactive periosteal bone

Codman triangle: triangular shadow between cortex and raised ends of periosteum

28

if a osteosarcoma mets where to and how?

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

29

Osteosarcoma (osteogenic sarcoma)
Treatment

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

30

BONE TUMORS: Cartilage Forming Tumors

BONE TUMORS: Cartilage Forming Tumors

31

Benign Cartilage Forming Tumors

Osteochondroma
Chondroma
Chondroblastoma
Chondromyxoid Fibroma

32

Malignant Cartilage Forming Tumors

Chondrosarcoma

33

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

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

34

Osteochondroma (exostosis)
Tumor Characteristics

Cartilage Capped:

Solitary in ___%

Cap composed of :

Cartilage cap undergoes:

what is all contained in the tumor outgrowth?

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

35

Osteochondroma (exostosis)
have multiple hereditary syndromes:

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

36

Osteochondroma (exostosis)
Clinical manifestations

Slow growing
Painful
Impinge nerves
Stalk fractures

37

Osteochondroma (exostosis)
Hereditary exostosis

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

38

Chondroma
define:
age:
location:

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

39

Chondroma: Tumor Characteristics

Most

Cartilaginous tissue well

Well

Peripheral

Center can

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

40

Chondroma types:

En-chondroma: Arise within the medullary cavity

Subperiosteal or juxtacortical chondromas: Arise on surface of bone

41

what is ollier disease:

Multiple Enchondroma

42

what is maffucci syndrome?

multiple enchondromas with Hemangioma present

43

Chondroma
Clinical Features
-
-
-
Treatment:

Maffucci syndrome is at risk for developing

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)

44

Chondroblastoma
Age:
Sex:
Location:

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

45

Chondroblastoma
Tumor characteristics

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

46

Chondroblastoma
Clinical manifestations
Treatment:

Painful
Due to location: joints

Treatment
Curettage
May reoccur

47

Chondromyxoid Fibroma
define:
age:
sex:
location:

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

48

Chondromyxoid Fibroma
Tumor Characteristics?

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

49

Chondromyxoid Fibroma
Clinical Course
Treatment

Clinical Course
Manifest with dull achy pain


Treatment
Treated with Curettage
No threat of malignant transformation

50

Chondrosarcoma: neoplastic cartilage
Define:
age:
sex
location:

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

51

Chondrosarcoma: neoplastic cartilage
Tumor Characteristics
3 anatomic variants

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

52

Chondrosarcoma: neoplastic cartilage-
Tumor Characteristics Pathogenesis:


Central chondrosarcoma:
Secondary peripheral chondrosarcoma:

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

53

Chondrosarcoma: neoplastic cartilage
Tumor Characteristics Histology

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

54

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

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

55

Chondrosarcoma treatment:

Wide excision
Response to radiation and chemotherapy is poor