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TUMORS AROUND KNEE IN

PAEDIATRIC PATIENTS
• BY VINEETH
What is to be discussed?
• BONE TUMORS – BASICS
• OSTEOCHONDROMA
• OSTEOSARCOMA
• EWING’S TUMOR
Bone tumors
• The term ‘bone tumor’ is a broad term used for
benign and malignant neoplasms, as well as
‘tumor like conditions’ of the bone
• Osteochondroma is the most commonest benign
tumor of the bone. Of the primary bone
malignancies multiple myeloma is the
commonest but metastatic deposits in the bone
are commoner than primary bone tumors .
• The primary malignant bone tumors occur in
children and young adults of which osteosarcoma
and Ewing’s sarcoma are common.
Classification of bone tumors modified after
Revised WHO Classification – Schajowicz (1994)
Clinical presentation
• Pain
• Lumpy swelling
• Local tenderness
• Limitation of movements
• Loss of weight
Investigations
• X rays
• Radionuclide scanning
• Computed tomography
• MRI
• Laboratory investigations
• Biopsy
Differential diagnosis
• Soft tissue hematoma
• Myositis ossificans
• Stress fracture
• Tendon avulsion injury
• Bone infection
• Gout
Staging of tumors
Staging of benign bone tumors by Enneking
• Latent: Well-defined margin. Grows slowly and then
stops. Remains static/heals spontaneously [E.g. Osteoid
osteoma ]
• Active: Progressive growth limited by natural barriers Not
self-limiting. Tendency to recur [E.g. Aneurysmal bone cyst]
• Aggressive: Growth not limited by natural barriers [e.g.
giant cell tumour]
Principles of management
• Benign, asymptomatic lesions: If the diagnosis is beyond doubt,one
can afford to temporize; treatment may never be needed
• Benign, symptomatic or enlarging tumors: Painful lesions, or
tumors , require biopsy and confirmation of the diagnosis. Unless
they are unusually aggressive, they can generally be removed by
local (marginal) excision or (in the case of benign cysts) by
curettage.
• Suspected malignant tumors If the lesion is thought to be a
primary malignant tumor, the patient is admitted for more detailed
examination, blood tests, chest x-ray, further imaging. A choice
needs to be made between amputation, limb-sparing operations
and different types of adjuvant therapy, and the patient must be
fully informed about the pros and cons of each.
Methods of treatment
• Tumor excision (intra capsular excision and curettage, marginal
excision, wide excision and radical resection)
• Limb salvage
• Amputation
• Multi- agent chemotherapy
• Radiotherapy
Osteochondroma
• This is most commonest benign tumors of bone
• It is a developmental lesion which starts as a small overgrowth
of cartilage at the edge of the physeal plate and develops by
endochondral ossification into a bony protuberance still
covered by the cap of cartilage.
• The patient is usually a teenager or young adult
• Commonest sites are the fast-growing ends of long bones and
the crest of the ilium.
• Here it may go on growing but at the end of the normal
growth period for that bone it stops enlarging. Any further
enlargement after the end of the growth period is suggestive
of malignant transformation
X-ray
• The x-ray appearance is pathognomonic. There is a well-defined exostosis.
It looks smaller than it feels because the cartilage cap is usually invisible
on x-ray; however, large lesions undergo cartilage degeneration and
calcification and shows the bony exostosis surrounded by clouds of
calcified material.
Malignant change
Features suggestive of malignant change are:
(1) enlargement of the cartilage cap in
successive examinations;
(2) a bulky cartilage cap (more than 1 cm in
thickness);
(3) irregularly scattered flecks of calcification
within the cartilage cap; and
(4) spread into the surrounding soft tissues.
MRI may be needed to reveal these changes
Treatment
• If the tumour causes symptoms it should be excised; if,
in an adult, it has recently become bigger or painful
then operation is urgent, for these features suggest
malignancy. This is seen most often with pelvic
exostoses – not because they are inherently different
but because considerable enlargement may, for long
periods, pass unnoticed. If there are suspicious
features, further imaging and staging should be carried
out before doing a biopsy. If the histology is that of
‘benign’ cartilage but the tumour is known for certain
to be enlarging after the end of the growth period, it
should be treated as a chondrosarcoma.
Osteosarcoma
• Malignant mesenchymal tumor in which there is formation of bone by tumor cells

• Most common non-hematopoietic bone tumor

• 75% occur before the age of 20 years. Second peak in elderly

• Commoner in males (1.6:1)

• Arises from metaphysis of long bones. 60% arise around the knee

• Patients with hereditary retinoblastomas have a 1000 fold risk

• Metastasizes through hematogenous route (90% to lungs)

• Advances in treatment (chemotherapy and limb salvage therapy) has improved long

term survival to 60-70%


Osteosarcoma - subcategorization
Based on Subtypes

Location Intramedullary / intracortical / periosteal etc

Differentiation Well / poorly

Osteoblastic / chondroblastic / fibroblastic,


Histology
telangiectatic / giant cell / small cell

Solitary / multicentric
Others
Primary / secondary
Gross:
Bulky, gritty, hemorrhage and necrosis,bone destructive and spreading in
different directions
Microscopy:
- Bizarre tumors cells with pleomorphic nuclei
- Multinucleated giant cells
- Formation of “lacy osteoid”
- Formation of other types of matrix
- Conspicuous vascular invasion - metastasize to lungs, brain
Lab findings:
Increased level of ESR and serum alkaline phosphatase
X-ray
• Irregular destruction
• Periosteal reaction
• Codman’s triangle
• Sun-ray appearance
Surgical staging system by Enneking
Ewing’s sarcoma
• Highly malignant tumor occurring between 10-20 years
• Primary malignant small round cell tumors of bone and soft tissue
• Second commonest malignant non-hematopoietic bone tumor in
children
• Diaphysis of long bones is affected and tumor ruptures through the
cortex early and extends into the soft tissues
• Bones affected: 2/3rd in femur and tibia and 1/3rd in flat bones.
Occasionally it is known to have a multi centric origin.
• 85% show t(11;22)(q24,q12)
• Tumors arise from medullary cavity, invade cortex, spread to soft
tissue
• Metastasis at the time of diagnosis – lungs(35%)
• Combination chemotherapy has improved 5 year survival to 75%
Histopathology
• Comprised of small round cells slightly larger than
lymphocytes
• Cells are rich in glycogen.
• May show Homer-Wright rosettes. Necrosis is seen
X-ray
Treatment
• The prognosis is always poor and surgery alone does little to
improve it. Radiotherapy has a dramatic effect on the tumour
but overall survival is not much enhanced. Chemotherapy is
much more effective, offering a 5-year survival rate of about
50 per cent
• Control of local tumor by radiotherapy of 6000rads and
control of metastasis by chemotherapy of vincristine,
cyclophosphamide and adriamycin in cycles, repeated every
3-4 weeks for about 12-18 cycles.