BONE AND SOFT

TISSUE
TUMOURS
   
  Metastatic Tumours  

The most common bony malignancies are

metastatic carcinomas. With improved treatment
regimens, which has prolonged survival, the
deletion is involved in over half of all metastatic
carcinomas through hematogenous spread. Spread
can be arterial or venous (retrograde venous
spread is through the veins of Batson in the spine).
Metastatic lesions represent the most common
cause of pathology fractures due to a neoplasm.
Usually are multiple, but can be solitary. Most
common primaries are breast, prostate, lung,
kidney and thyroid, in that order. Renal mets are
quite vascular and have cold bone scans, and
histology may demonstrate clear cells that are PAS
positive. Prostate and breast mets are typically
osteoplastic. Wilms’s tumour and neuroblastoma
(rosettes) are the most common metastatic lesions
in childhood. Prophylactic fixation of weight-bearing
bones (often with PMMA supplementation) is
recommended for lesions >2.5 cm in diameter or
involving >50% of the cortex, or in patients having
pain. XRT is often helpful for tumours metastatic to
bone (it is administered preop in patients with
thyroid and kidney mets). Work-up should include
labs (increased calcium common but nonspecific),
bone scan (looking for multiple lesions), CT (axial
lesions, and chest CT), angiography (preop for
thyroid and hypernephroma mets), and biopsy. In
general, 50% of patients with bony mets will survive
6 months and 30% will survive 1 year. Shorter
survival periods can be anticipated with lung
cancer, and longer periods with thyroid mets.
 Cortical osteoid osteoma
Calcar femur with florid
cortical reaction

Primary Bone Tumours

Oesteogenic Lesions

Osteoid Osteoma - Usually are intracortical lesions

of any bone, especially the femur, tibia, and
vertebra (posterior elements), with a characteristic
nidus and associated pain. The classic pain is
worse at night, relieved by salicylates (not
narcotics), and exacerbated by alcohol. Bone
scans, tomograms, and CT are often helpful,
Cortical osteoid osteoma
spontaneous resolution. Treatment ordinarily
Calcar femur with florid
consists of surgical removal of the nidus with a
cortical reaction
marginal excision.

Osteoblastoma - Characterised by immature

osteoid production and found commonly in the
cancellous bone of the spine (posterior elements)
and skull.

Osteoma

Paget’s Disease

This disease, which affects about 3% of men and

women, is characterised by increased bony
turnover common in Australia and N.Z. Increased
osteoplastic bone resorption with irregular bone
formation leads to focally deficient bone. Paget’s
disease typically involves the sacrum, spine, femur
and cranium of older patients. Some have
hypothesised a viral etiology. Features of Paget’s
include deformities (bowing), pain, change in size of
bones (increased hat size), kyphosis, and systemic
features like cranial nerve impingement, bruits, and
high-output cardiac failure. Lab studies are notable
for increased alkaline phosphatase (osteoblasts)
and urinary hydroxyproline (osteoclasts).
Radiographic features vary with the stage of the
disease but include cortical thickening, coarse
trabeculae, sclerotic ivory or enlarged picture frame
vertebrae, cotton wool appearance of the femur and
pelvis, and osteoporosis circumscripta of the
calvarium. Late radiographic hallmarks include
enlarged bone, thickened cortices, and prominent
trabeculae. Bone scans are also helpful for
screening. Histologically there is mosaic. Fractures
occur commonly, are usually transverse, and
frequently require internal fixation; total hip
arthroplasty is often required for stabilisation of
proximal femur fractures. Available treatment for
symptomatic patients (with significant deformity or
impending fracture) includes calcitonin (directly
inhibits osteoplastic bone resorption); Malignant
degeneration (usually to osteosarcoma,
chondrosarcoma, or MFH) has been reported in up
to 6% of cases and is usually heralded with an
increase in pain. Malignant degeneration of Paget’s
disease usually carries a poor prognosis. (See
case13 ).
Click to see pot from Case 13

Ossifying Fibroma (Osteofibrous Dysplasia,

Campanacci’s Tumour) - A variant of fibrous
dysplasia.

Osteosarcoma

The most common primary bone malignancy in

children (boys > girls), occurs most commonly in
the second and third decades of life. It is
characterised by the production of osteoid directly
from malignant spindle cell stroma. The knee and
proximal humerus are common sites, and early
diagnosis is difficult. A relatively painless mass is a
frequent presentation. Osteosarcomas are
classified as primary or secondary (following a
preexisting lesion such as Paget’s or a result of
XRT, and usually seen in older patients). Primary
tumours are further classified as central or
juxtacortical. (See case 6 ).

Click to see pot from Case 6

Central Osteosarcomas
Juxtacortical Osteosarcomas
Periosteal Osteosarcoma
High-Grade Surface Osteosarcoma

Chondrogenic Lesions

Chondromas - Benign tumours of mature hyaline

cartilage usually located within bone
(enchondroma), but occasionally on the bony
surface (periosteal chondroma).

Enchondroma - Benign cartilaginous neoplasm

arising in diaphyseal medullary cavities of long
bones, possibly from epiphyseal plate remnants.
Can be solitary or multiple (Ollier’s disease [alone]
and Maffucci’s syndrome [with skin hemangiomata]-
both with higher rates of malignant transformation
[up to 30% in Ollier’s]). Enchondromas are
asymptomatic and can be located in any bone,
especially in the hands and feet.

Periosteal Chondroma

Osteochondroma (Osteocartilaginous Exostosis) -

One of the most common benign bone tumours.
These lesions represent a disorder of normal
enchondral bone growth, and are usually sessile or
pedunculated lesions arising from the cortex of a
long bone adjacent to the epiphyseal plate. Tumour
enlargement is common and ceases after maturity.
Malignant transformation (to low-grade
chondrosarcoma) is possible, and is more common
with multiple osteochondromas and more proximal
lesions and growth with skeletal maturity. Multiple
hereditary exostosis (Ehrenfried’s disease) is an
autosomal dominant disorder with a mild decrease
in stature, normal intelligence, and multiple
osteochondromas. It is commonly accompanied by
leg length discrepancy, knee and elbow angular
deformities, and other skeletal abnormalities.
Osteochondromas develop in multiple metaphyses
of affected children and they continue to increase in
size and number until skeletalmaturity. Problems
include nerve compression (especially peroneal
nerve), ankle diastasis, angular deformities, and
malignant transformation (2%). (See POT).

Chondromyxoid Fibroma

Chondroblastoma

Chondrosarcoma - Second to osteosarcoma in

prevalence of bony sarcoma’s. Chondrosarcomas
can arise from preexisting lesions
(osteochondromas, chondromas) or they can be
primary. They are usually associated with dull, deep
pain. Radiographs may show invasiveness and soft
tissue extension. Chondrosarcomas are commonly
divided into five types. (See case 9 ).
Click to see pot from Case 9

Central Chondrosarcoma

Peripheral or Periosteal Chondrosarcoma

Mesenchymal Chondrosarcoma

Dedifferentiated Chondrosarcoma

Clear Cell Chondrosarcoma

Fibrogenic Lesions

Simple (Unicameral) Bone Cyst - Benign lesions

that occur during growth.

Aneurysmal Bone Cyst (ABC).

Fibrous Dysplasia

The shepherd’s crook. Fibrous dysplasia is also

common the in skull, ribs, and tibia. Radiographs
also show a typical ground glass appearance and
scalloping from within. Cafe-au-lait spots Albright’s
syndrome precocious puberty.

Nonossifying Fibroma (Fibrous Cortical Defect)

Malignant Fibrous Histiocytoma (MFH).

Fibrosarcoma

Haematopoietic Lesions

Eosinophilic granuloma is a solitary lesion that

arises from the reticuloendothelial system in young
children. Vertebra plana.

Myeloma - Uncontrolled proliferation of marrow

plasma cells (highly differentiated B lymphocytes)
leads to the development of this tumour, the most
common primary malignant tumour in bone.
Common presentations include bone pain, anaemia
and raised ESR in a 50-60 year old patient.
Histology demonstrates sheets of plasma cells
(clock faced eccentric nuclei, perinuclear clear
area, and increased RER on electron microscopy).
Of note, the lesion lacks any background stroma. X-
ray shows punched out lesions. Treatment is
chemotherapy, radiotherapy and surgical
stabilisation impending fractures.
Lymphoma of Bone (Histiocytic Lymphoma).

Vascular Lesions

Undetermined Etiology

Giant Cell Tumour - Aggressive, locally recurrent

tumour with a low metastatic potential. Represents
about 20% of primary bone tumours. Most lesions
occur in closed epiphyses around the knee joint
and distal radius of 20 - 40 year old female > male
patients (See case 8 ).

Click to see pot from Case 8

Ewing’s Sarcoma - Second (to osteosarcoma)

most common malignant bone tumour of childhood.
Occurs in young (white >> black) children in the flat
and axial bones (pelvis), and in the diaphyses of
long bones (fibula, femur). Often associated with
systemic signs (fever, weight loss) and local
tenderness with erythema and induration, and can
be confused with osteomyelitis.

CLUES TO DIAGNOSIS
 CATION/SITE TYPICAL PATHOLOGY

Chondroblastoma,
Epiphyseal chondrosarcoma, giant
cell tumour, infection

Metaphyseal Any lesion

Osteoblastoma, Ewing’s
eosinophilic granuloma,
Diaphyseal
lymphoma, adamantinoma,
fibrous dysplasia

Mets, myeloma, Ewing’s,

Pelvis chondrosarcoma, Paget’s
Disease.

Proximal
Chondroid lesions.
humerus

   Osteosarcoma, adamantinoma,  
Knee
chondromyxoid fibroma

Mets, myeloma, Ewing’s

Ribs chondrosarcoma, fibrous
dysplasia

Mets, myeloma, eosinophilic

Spine (Vertebral
granuloma, chondroma,
body)
Paget’s, haemangioma

Spine (Posterior Aneurysmal bone cyst, osteoid

elements) osteoma, osteoblastoma

Myosotis, osteosarcoma,
Periosteal
chondrosarcoma, chondroma

Mets, myeloma, haemangioma,

fibrous dysplasia,
Multiple lesions
osteochondromas,
enchondromas, histiocytosis X

  

Tumours of Muscular Tissue

Leiomyoma
 Rhabdomyoma

Leiomyoscarcoma

Rhabdomyosarcoma

Tumours of Synovial Tissues

Ganglia - Cystic outpouching of a synovium-lined

cavity, frequently about the wrist, and consisting of
myxoid degeneration of synovial fluid. Histologically,
the cyst wall is made up of dense paucicellular
collagenous tissue. (See POT).

Pigmented Villonodular Synovitis.

Treatment Bone Tumours in General

Work-up - History (pain - dull/constant/nocturnal;

fracture).

• Examine bone / spine / thyroid / breast /

abdomen / prostate).

• Blood tests (FBC, ESR, Blood smear, ca/p

levels, urine analysis, liver function tests.

• X-rays spine and limbs, bone scan for

metastases, CXR, MRI for occult lesions,
possibly CT and arteriography.

• Stage (biopsy) lesion. Important principles

involved.

• Surgery either amputation or limb salvage.

• Adjuvant chemotherapy (especially for

micrometastases) and radiotherapy (local
control).