Patho Exam 2:Bone Tumors & Tumor-Like Lesions Flashcards
Bone Forming Tumors
Osteoma
Osteoid Osteoma & Osteoblastoma
Osteosarcoma
Cartilage Forming Tumors
Osteochondroma Chondromas Chondroblastoma Chondromyxoid Fibroma Chondrosarcoma
Fibrous & Fibro-Osseous Tumors
Fibrous Cortical Defect & Non-Ossifying Fibroma
Fibrous Dysplasia
Fibrosarcoma Variants
Miscellaneous Tumors
Ewing Sarcoma/Primitive Neuroectodermal Tumor
Giant-Cell Tumor
Bone Forming Tumors
Benign:
Malignant
Benign:
Osteoma
Osteoid Osteoma
Osteoblastoma
Malignant
Osteosarcoma
Osteoma
Osteoma
what is an Osteoma
Benign & slow growing of cortical dense bone
what age is most likely to have osteomas?
Middle age
where are osteomas commonly fount?
Most common on or inside skull and facial bones
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
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
Osteoid Osteoma
Osteoid Osteoma
what are Osteoid Osteoma
Benign bone tumors with more compression than an Osteoma
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)
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)
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
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
Osteoblastoma
Osteoblastoma
Osteoblastoma age: sex: location Tumor characteristics:
-
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
Osteoblastoma
Manifestation
Treatment:
Dull achy pain
Not relieved by ASA
Malignant transformation associated when treated with radiation
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)
Osteosarcoma (osteogenic sarcoma)
Location:
-
-elderly:
Metaphyseal of long bones of extremities
>50% occur around knee
Elderly
Flat bones and long bones equally
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
Treatment of ostosarcoma?
Differs depending on mutation if tx improperly with the incorrect chemo you can actually make it worse
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
Osteosarcoma (osteogenic sarcoma)
Clinical Course
Painful
Progressively enlarging
sudden fracture might be initial symptom
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
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
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
BONE TUMORS:Cartilage Forming Tumors
BONE TUMORS:Cartilage Forming Tumors
Benign Cartilage Forming Tumors
Osteochondroma
Chondroma
Chondroblastoma
Chondromyxoid Fibroma
Malignant Cartilage Forming Tumors
Chondrosarcoma
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
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
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
Osteochondroma (exostosis)
Clinical manifestations
Slow growing
Painful
Impinge nerves
Stalk fractures
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
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
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
Chondroma types:
En-chondroma: Arise within the medullary cavity
Subperiosteal or juxtacortical chondromas: Arise on surface of bone
what is ollier disease:
Multiple Enchondroma
what is maffucci syndrome?
multiple enchondromas with Hemangioma present
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)
Chondroblastoma
Age:
Sex:
Location:
Benign: rare (>F (2:1)
Location
Proximal femur, tibia and humerus
Pelvis and ribs in older patients
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
Chondroblastoma
Clinical manifestations
Treatment:
Painful
Due to location: joints
Treatment
Curettage
May reoccur
Chondromyxoid Fibroma define: age: sex: location:
Benign: RAREST of cartilage tumors Age: TEENS Male > Females Location: Most common in metaphysis of long tubular bones
Chondromyxoid Fibroma
Tumor Characteristics?
Composed of chondroid, myxoid and fibrous tissue in variable amounts
“ATYPIA”: large hyperchromatic nuclei (a lot of DNA
Chondromyxoid Fibroma
Clinical Course
Treatment
Clinical Course
Manifest with dull achy pain
Treatment
Treated with Curettage
No threat of malignant transformation
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
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
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
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
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%
Chondrosarcoma treatment:
Wide excision
Response to radiation and chemotherapy is poor
