Bone tumors Flashcards
Global approach to bone lesions
- Determine if the tumor has agressive or non-agressive appearance, then classify it as “I know what it is” or “I don’t know what it is”
Aggresive appearance characteristics (6)
- Indisctinct margins.
- Wide zone of transition.
- Permeative appearance.
- Agressive periosteal reaction.
- Soft tissue component.
- Endosteal scalloping.
Non-Agressive appearance characteristics (6)
- Sclerotic margins.
- Narrow zone of transition.
- Well circumscribed.
- No agressive periosteal reaction.
- No soft tissue component.
- No endosteal scalloping.
Agressive “I know what it is” lesions (2)
- Osteosarcoma.
- Condrosarcoma.
Non-agressive “I know what is is” lesions (7)
- Enchondroma.
- Osteoid osteoma.
- Osteochondroma.
- Non-ossifying fibroma.
- Hemangioma.
- Giant cell tumor.
- Fibrous dysplasia.
Types of benign periosteal reaction (2)
- Normal periosteal anatomy.
- Solid periosteal reaction.
(See examples in the book)
Types of malignant periosteal reaction (3)
- Lamellamed periosteal reaction.
- Sunburst periosteal reaction.
- Codman triangle.
(See examples in the book)
Pattern of bone destruction: Margin analysis
- Agressive and non-agressive appearance.
- A sharply marginated zone of transition denotes as less agressive.
- A wide zone of transition denotes a more agressive lesion.
- Can be classified with the “Lodwick classification”.
Lodwick Classification
- Classification of bone destruction, helps to stratify agressiveness:
- Type 1: Geographic pattern
- Type 1a: Thin schlerotic margins, almost always non-agressive.
- Type 1b: Well-defined margins, usually non-agressive
- Type 1c: Any part of the margin is indistinct.
- Type 2: Moth-eaten pattern: Difficult to define any border at all. Agressive.
- Type 3: Permeative: Multiple tiny holes that infiltrate bone. Very agressive. Seen in Lymphoma, leukemia, and Ewing sarchoma.
Pseudopermeative appearance of margins
The permeative appearance will extend to the cortex (Seen in osteoporosis and osseous hemangioma).
Types of matrix production
- Osteoid lesions: Fluffly cloud-like bone. (Ex. malignant osteosarcoma).
- Chondroid lesions: Ring and arc or pop-corn like appearance (Ex. benign enchondroma or malignant chondrosarcoma).
- Ground glass matrix: Blurring of trabeculae (Ex: Fibrous dysplasia).
Fallen-fragment sign
- The presence of a bone fracture fragment resting dependently in a cystic bone lesion.
Aneurysmal or expansile tumor appearance
Sugest an aneurysmal bone cyst.
Hyperparathiroidism manifestations in the bones
- Subchondral reabsortion (Distal clavicles, symphisis pubis, SI joint).
- Tumoral calcinosis.
- Brown tumors.
- Acro-osteolysis.
- Rugger jersey spine.
Patient age in agresive bone lesions
- Agressive lytic bone lesion >40 years old: Metastasis or myeloma.
- Agressive lytic bone lesion <20 years old: Eosinophilic granuloma, infection or Ewing sarcoma.
Eccentric within bone tumors
- Giant-cell tumor.
- Chondroblastoma.
- Aneurysmal bone cyst.
- Non-ossifying fibroma.
- Chondromyxoid fibroma (rare).
Central bone tumors
- Simple bone cyst.
- Enchondroma.
- Fibrous dysplasia.
Enostosis (bone island)
- Benign.
- Small spiculated osteoblastic focus.
- It may be rarely difficult to differenciate from osteoblastic metastasis, osteoid osteoma or low-grade osteosarcoma.
- Giant variant >2 cm may be most difficut to differenciate from low-grade osteosarcoma.
- Bone scan is normal.
- Associated with mixed schlerosis bone dysplasia: Osteopoikilosis, melorheostosis and osteopathia striata.
Osteopathia striata
- Voorhoeve disease
- Benign, asymptomatic sclerotic dysplasia characterized by linear bands of sclerosis in the long bones and fan-like sclerosis in the flat pelvic bones.
- Bone scan is normal.
Osteopoikilosis
Autosomal dominant disorder with multiple focuses of enostosis and keloid formation.
Osteoma
- Slow growing lesions that may arise from the cortex of the skull or the frontal/etmoidal sinuses.
- Gardner syndrome: Multiple osteomas, intestinal polyposis and desmoid tumors.
Melorheostosis
- Pain, decreased range of motion, leg bowing and leg-length discrepancy.
- Associated with schleroderma-like skin lesions.
- Distribution of a single dermathome.
- Non-neoplasic proliferation of thickened and irregular cortex with typical candle-wax appearance.
- Intense uptake on bone scan.
Osteoid osteoma
- Benign osteoblastic lesion.
- Typically in children.
- Lucent nidus of osteoid tissue (may be with central calcification) surrounded by reactive bone sclerosis.
- Presentation: Nocturnal pain relieved by aspirin in a teenager or young adult.
- Tends to occur in diaphyses of leg long bones.
- 20% in posterior vertebral elements.
- Double density sign at bone scan (hotter spot within hot area sign).
- MRI: nidus is T1 low-signal and reactive marrow edema can obscure de lesion on T2.
- Treatment: Radiofrequency ablation, surgical curettage or resection.
Osteoblastoma
- Benign osteoid-producing tumor.
- Histologically is the same as a osteoid osteoma, only that it is >2 cm in size.
- 4 times less common that osteoid osteoma.
- Presentation: Nocturnal pain not relieved by aspirin in a teenager or young adult.
- Most commonly in posterior elements of the spine, also in diaphyses of leg long bones.
- Lytic apprearance with mineralization, may have a secondary aneurysmal bone cyst.
- Lytic lesion in the posterior elements of the spine in a young person may be osteoblastoma or aneurysmal bone cyst (The last does not have mineralization).
Osteosarcoma primary types
- More than 10 sub-types (most important ones):
- Conventional (intramedullary).
- Talangiectatic.
- Paraosteal.
- Periosteal.
Osteosarcoma generalities
- Heterogeneous group of malignant tumors.
- Cells derived from osteoid linage, most of them produce an osteoid matrix.
- Primary: 10 sub-types.
- The most common primary type is the conventional.
- Secondary: Paget disease or radiation.
- Hallmarks: Bony destruction, production of osteoid matrix, agressive periosteal reaction and soft-tissue mass.
Conventional osteosarcoma
- 75% of osteosarcomas.
- Occurs in adolescent/young adults usually at the knee in the metaphysis of the femur or tibia.
- Features: Intramedullary osteoid matrix, intramedullary and cortical bone destruction, agressive periosteal reaction (sunburst or codman) and soft-tissue mass.
- MRI: T1 low-signal, T2 high-signal (edema) and T2 low-signal (sclerosis) areas.
Talengiectatic osteosarcoma
- Osteolytic destructive sarcoma.
- May mimic a benign aneurysmal bone cyst.
- Vascular, with large cystic spaces filled with blood.
- Does not produce any osteoid matrix components.
- The presence of nodular components at MRI differenciates it from a benign aneurysmal bone cyst.
- MRI: Heterogeneous lesion with T2 high-signal and areas of enhancement at T1-post contrast.
Parosteal osteosarcoma
- Juxtacortical osteosarcoma that arises from the outer periosteum.
- Most commonly occurs at the posterior aspect of the distal femoral metaphysis.
- Cauliflower-like exophytic morphology.
- Least malignant of all osteosarcomas (90% five-year survival).
Periosteal osteosarcoma
- Juxtacortical osteosarcoma that arises from the inner periosteum.
- <20 years old, commonly at the diaphysis of the femur or tibia.
- Cortical thickening, agressive periosteal reaction and soft-tissue mass.
- Histologically may show chondroid differentiation.
Synovial chondromatosis
- Synovial metaplasia with formation of intra-articular lobulated cartilaginous nodules.
- When ossified it is called osteochondromatosis.
- May produce mechanical erosions and secondary OA.
- Most common location is the knee. Other locations: Shoulders, hip and elbows.
- Radiography shows multiple round intra-articular bodies of similar size and variable mineralization.
- MRI appearance is variable: Multiple globular and rounded foci of low signal - DD in MRI: pigmented villonodular synovitis, which do not calcify.
- Very rarely: malignant degeneration to chondrosarcoma.
Enchondroma
- Benign lesion of mature hyaline cartilage rests.
- In the long bones it features the classic popcorn and ring and arc calcifications
- DD: Bone infarct (serpentine sclerosis) and chondrosarcoma.
- MRI: Lobulated T2 high-signal.
- At the hand typically doesn’t produce chondroid matrix and appears as a geographic lytic lesion.
- May be complicated with pathological fracture at the hand.
- Very rarely: malignant degeneration to chondrosarcoma, except in multiple enchondromas of Ollier and Maffucci syndromes.
- Worrisome clinical findings: New pain in abscence of fracture.
- No universal law for following, depends if it has high or low risk of malignant transformation.
Characteristics of low risk malignant transformation enchondromas (4)
- <4 cm
- Knee or shoulder
- No endosteal scalloping.
- Increase of chondroid mineralization.