ORTHOPEDICS

Tumors of the Musculoskeletal System


• Bone tumors: 0’5-1% of all malignant tumors

• Soft tissue tumors: 1’5-3% of all malignant tumors

Most common primary malignant bone tumors:

1. Osteosarcoma
2. Chondrosarcoma
3. Ewing Sarcoma

X-Ray features of Benign vs Malignant

BENIGN MALIGNANT

Cortical bone thin Codman triangle

Sclerotic rim Onion peel (esp in ewing sarcoma)

Ill-defined border

Destructed cortical bone

Invasion into soft tissue

Spicule formation/spiculated mass

BENIGN BONE TUMORS

Osteoid Osteoma Osteoblastoma

Frequency common (10% of benign bone tumors) less common

Age 15-25 (M > F) < 20

Size < 2cm > 2cm

Histology osteoblastic lesion similar (shows active expansion)

sclerotic margins absent/less prominent

Symptoms deep aching pain at night that responds to aspirin 1. Neurological complications: vertebral arches
(diagonstic feature) 2. Asymptomatic for long time: tubular bones

Location Femur & tibia (diaphysis) 1. Large tubular bones

2. Vertebral arches
3. Ribs
4. Pelvis

X-ray Features Dense sclerotic centre ‘Nidus’ surrounded by reactive 1. Extensive osteolytic destruction
sclerosis 2. Less sclerotic margins

Treatment 1. Total removal of Nidus. If unsuccessful, pain &
recurrence
2. Curettage and resection of nidus. ‘excochleation’
3. Radio-frequency, thermoregulation, CT guided
needle
1. TOC: curettage & filling with cancellous graft
2. En-bloc excision in normal tissue
* wide resection recommended
* benign but expressed tendency for
progression
 MALIGNANT BONE FORMING (OSTEOGENIC) TUMORS

OSTEOSARCOMA (CENTRAL) PAROSTEAL PERIOSTEAL

OSTEOSARCOMA OSTEOSARCOMA

Frequency * Most common primary malignant bone tumor

* 25-30% of all bone sarcomas
* Central osteosarcoma is most common

Age 60% in 1st two decades of life (10-20)

Location 50% occur around the knee: Juxtacortical * Located on outer

* distal femur osteosarcoma’ surface of bone
* proximal tibial metaphysis * located on the surface (periosteum)
May occur in any bone of the bone from * Usually on the
parosteal tissues diaphysis

Symptoms 1. Intermittent pain that becomes persistent

2. Swelling that expands rapidly
3. Warm skin with swollen subQ veins
4. Pathological fractures in 10% (advanced stage)

X Ray 1. Breaking of cortex

2. Infiltrating soft tissues around
3. Osteoid production; spicule creation (only central OS)
4. Fibroblastic, telangiectatic & anaplastic osteosarcomas
don’t show bone production, they show osteolytic
destruction
5. Most common: mixed lytic-sclerotic forms
6. “Moth-eaten/Sunburst” appearance

CT & MRI * To assess soft tissue and intramedullary penetration

* ‘Skip metastasis’ seen a few cm from tumor site

Treatment Central Osteosarcoma * Low grade malignant

* Chemo & surgery (radio-resistant!) osteosarcoma
* Pre and Post operative cytostatic surgery * Better prognosis
* Pre-op (Neo-adjuvant) chemo to decrease chance of * Wide resection
metastasis during biopsy * No chemo
* 5 yr: 60-75% (half are limb saving procedures) * 95% survival
* Bone segment is removed together with joint segment
 BENIGN CARTILAGE FORMING TUMORS

Endochondroma Osteochodroma Chondroblastoma Chondromyxoid Bone

Fibroma

Intramedullary Cartilaginous Exostosis Cartilaginea: a

Neoplasm cartilage bud that migrates
out of bone plate (boney lump
covered by hyaline cap)

Frequency 20% of benign bone tumors 50-60% of all benign lumps RARE RARE
5-10% of malignant
transformation

Age 20-40 (M=F [only one]!) 1st decade of life (<20) Boys: before end of 2nd decade
growth

Location Metaphysis: Metaphysis: Epiphysis next to joint Metaphysis of long

* phalanges * long tubular bones * Tibia tubular bones
* metacarpals * distal femur * Femur
(hands & feet) * proximal tibia and * Humerus
humerus

Symptoms * mostly asymptomatic * Mostly asymptomatic * Swelling Asymptomatic

* stabbing pain/pathological * Accidental discovery * Restricted range of
fracture * As it grows: compression motion
of nerves/vessels, tendons
snapping over it

X-Ray *central destruction in * ‘Hat-rack’ shaped * Eccentric lytic lesions

metadiaphysis * Up to 10 cm * Uneven sclerotic
*no periosteal reaction margin
* May lead to bone
protrusion

Histology Lobular hyaline cartilage

Treatment * Excochleation * Chiseling * Excochleation * Excochleation

* Cavity filling with * 1% malignant * Autologous cancellous * Autologous/
autologous cancellous transformation bone graft Homologous cancellous
bone bone grafting
* 0’5-1% produce
secondary
chondrosarcoma
 MALIGNANT CARTILAGE FORMING TUMORS

CHONDROSARCOMA

Histology * Hyaline cartilage cells in various stages of maturation in a matrix

* many groups: different histology

Frequency 2nd most common malignant bone tumor

Age/Gender M>F; 3rd-6th decade

Location * Most Common: proximal femur, pelvis, humerus, scapula

* Less: ribs, tibia (proximal)
* Rarely: wrist and foot

Symptoms Local swelling and pain

* Well differentiated (hypovascular) limb swelling over many years
* Poorly differentiated: breaks through cortex early, invades soft tissues, metastasizes to lung (these are
mesenchymal & highly malignant chondrosarcomas)

X-Ray 1. Intraosseous central chondrosarcoma: swells bone—> lobular, lytic destruction

2. Calcification may appear—> calcified spots
3. Peripheral chondrosarcoma: initially- doesn’t involve medullary cavity, only later stage. (flat-bones, sacrum,
scapula—> malignant transformation from osteochondroma)

Pathology grey white/bluish white transparent solid tumor ‘mother of peal’ appearance

Treatment 1. Low malignancy chondrosarcomas (Grade I): light cell chondrosarcoma, secondary chondrosarcoma
a. radio & chemo resistant
b. Surgery: radical/wide resection of tumor with its intact muscle envelope (5yr-90%)
2. Grade II & III (mesenchymal dedifferentiated chondrosarcomas)
a. radical amputation
b. regional lymph node radiation (lung metastasis= poor prognosis)
c. 5yr: 30-35/50?%
 BONE MARROW TUMORS

Ewing Sarcoma Primary Bone Multiple Myeloma

Lymphoma
Age early (5-25) middle-age > 40
85% of cases chromosome translocation
(t11:12 & q4:22—> 11:22)

Histology Round cell sarcoma with neuroectodermal

differentiation
* undifferentiated small ‘blue-cell’ tumor
* Glycogen granules shown by PAS on LM

Location Diaphysis, Meta-diaphysis of long tubular Pelvis, Femur, Tibia, Humerus, Vertebra, ribs, skull, pelvis,
bones: femur, tibia, humerus pelvis Ribs sternum
Usually solitary but can be multiple (systemic
disease)

Frequency 3rd most common primary cone sarcoma RARE

Symptoms * Swelling and pain Asymptomatic for a long time * Vague back pain
* Rapid progression Pathological fracture may be
* Accompanied by fever and leukocytosis 1st sign Severe cases:
* Duration of complaints 3-6 months * vertebral compression,
* Lump spreads from intramedullary space to paralysis, parasthesia
sub-periosteal area—> elevates the * Inc ESR, pathologic plasma
periosteum cells, bence jones proteins in
urine

X-ray * Spotty radio frequency on the diaphysis Extensive bone destruction in Rounded lytic bone destruction
* Onion peel shaped periosteal reaction the diametaphysis of long bone
* Codman triangle is common without periosteal reaction

Treatment Radio & Chemo sensitive 1. Local Irradiation Solitary Forms: irradiation,
1. pre-op chemo 2. Surgery (limb saving) resection replacement
2. radiation (40-60Gy) 3. Chemotherapy Most commonly: chemo
3. radical surgery intervention Pathological fractures:
4. Post-op chemo in axial locations osteosynthesis (nails & plates)
* with this algorithm: 5yr 40-50%
* with lung metastasis: poor prognosis

*Codman triangle: triangular area of new

subperiosteal bone that is created when a
lesion, often a tumor, raises the periosteum
away from the bone
 GIANT CELL TUMOR OF BONE (OSTEOCLASTOMA)

Origin Medullary stem cells, osteoblast precursor cells

Age/Gender 2nd-4th decade F > M (ONLY ONE)

Classification SEMIMALIGNANT:
80% benign
10% primary malignant
5% malignant transformation
3-5% lung metastasis

Location Epi-metaphysis of long tubular bones:

* distal femur, proximal tibia, proximal humerus, distal radius
* more than 50%: knee area
* Less common sacrum, pelvis

Symptoms Tumor grows rapidly

Vague local pain and swelling
Pathological fractures

X-ray Exclusively lytic bone destruction-rounded/oval, eccentric, cortex is thinned

No periosteal reaction, no sclerotic margin, frequently invades margin of joint surfaces —> surface may
collapse

Pathology Macro: reddish brown soft tumor with haemorrhage

Micro: connective tissue fusiform cells, mononuclear cells, osteoclast type giant cells

Treatment Excochleation (curettage)- fill with bone chips or bone cement: recurrence common
In aggressive case: resect with intact tissue margin (if we don't need to sacrifice joint surface)
 OTHER TUMORS OF THE BONE

BENIGN RARE BONE TUMORS

Age Location X-Ray Treatment

Hemangioma 20-60 flat bones, skull, spine *oval lytic destruction (tiger stripes) observe
*spicule formation surgery if needed

Desmoplastic bone 20-40 metaphysis of long tubular lytic lesions surgery

fibroma bones cortical destruction

Lipoma 20-60 tubular bones, cuboid bones lytic lesions surgery

+/-sclerotic border

MALIGNANT RARE BONE TUMORS

Age Location X-ray Treatment

Fibrosarcoma 30-50 metaphysis tibia, femur Lytic-sclerotic destruction surgery

blurred border 5yr: 30-70%

Malignant Fibrous 20-50 femur, tibia, humerus Lytic-sclerotic destruction surgery

Histiocytoma blurred border 5yr: 60-80%

Liposarcoma 30-50 femur, tibia, humerus, pelvis Lytic destruction surgery

blurred border 5yr: 40-60%

Adamantinoma 5-30 diaphysis of tibia cystic ‘honeycomb like’ sharply surgery

bordered lesion 5yr: 90%

Chondroma 20-70 Sacrum, cervical spine, skull Lytic destruction surgery

blurred border, calcification 5yr: 60-90%

BONE METASTASIS

Stats Location (dec Symptoms Diagnosis Treatment Prognosis

order)

* 80-100x more common 1. Spine Pain X-ray Surgery Better

than primary (lumbar 1st) * independant of CT Radio * breast
* 20-40% of cases- 2. Ribs mvmt MRI Chemo * prostate
metastasis is found before 3. Proximal * doesn't respond PET-CT * thyroid
primary femur to pain killers Bone Scan Only if expected survival * kidney
* 3rd most common location 4. Skull * lasts months of patient is >3 months
for metastasis (after 5. Sternum Area Poor:
lung&liver) 6. Humerus * Tender Bisphosphonates can be * lung
* 65-85% are from breast, * Warm given in lytic and mixed * melanoma
lung, kidney, prostate * Swollen type * unknown
Pathological origin
Fractures
* Vertebra (origins)
* Femur
* Tibia

Prevention of Pathological Lifelong bisphosphonate therapy

Fractures Stabilisation
Rarely resection

Aim of Surgery To achieve pain relief and a better quality of life

 TUMOR LIKE BONE PROCESSES

• More common than primary malignant bone tumors

• Appearance may mimic malignant bone tumors
• They all appear at an early age

•
Location Symptoms X-Ray Treatment

Juvenile Bone Cyst Metaphysis of humerus, discovered by accident lytic lesion, depot steroids
femur, tibia pathological fracture of sharp border, excochleation
thin cortex thin cortex, sclerotisation
swollen bone (@ first a plaster cast
can be tried)

Aneurysmal bone cyst Meta-epiphysis of long pain, swelling, central/eccentric growth observation
tubular bones, pathological fracture blurred margin (@ first plaster cast can
25%: appears in deformed base be tried)
association with other Incase of fracture:
primary bone tumors excochleation &
cancellous graft
sclerotisation

Non-Ossifying bone Metaphysis of long no symptoms/mild pain/ subcortical, sclerotic excochleation &
Fibroma tubular bones pathological fracture margin lobular cystic cancellous graft
thinning bisphosphonate
supplement

Fibrous Dysplasia Meta-diaphysis of long mild pain blurred margin excochleation &
tubular bones, curvature of bone multiple opaque glass cancellous graft
pelvis pathological fracture lytic defects
‘shepherd’s crook’
shaped femur

Eosinophil Granuloma Vertebrae, pain sharp border excochleation &

Long tubular bones, eosinophilia lytic bone defect cancellous graft
Meta-diaphysis of flat lamellar periosteal
bones, reaction

Myositis Ossificans long tubular bones Initially: fever, Initially: oval lump with Surgical excision
Meta-diaphysis of the leukocytosis, pain ossification of the
muscles, due to trauma Later: solid lump, periphery
in young adults contractures Later: lump with uniform
bone density divided
from the bone by a
radiolucent area