Bone tumors

Irfanullah
Final year MBBS

Bone tumors
Tumor
It is an abnormal growth of tissue resulting
from uncontrolled,progressive multiplication
of cells and serving no physiological function.
May be benign or malignant.

Epidemiology
The American Cancer Societys estimates for cancer of the bones and joints for
2016 are:
About 3,300 new cases will be diagnosed
About 1,490 deaths from these cancers are expected.
Primary cancers of bones account for less than 0.2% of all cancers.
In adults, over 40% of primary bone cancers are chondrosarcomas. This is followed
by osteosarcomas (28%), chordomas (10%), Ewing tumors (8%), and malignant
fibrous histiocytoma/fibrosarcomas (4%). The remainder of cases are several rare
types of bone cancers.
In children and teenagers (those younger than 20 years), osteosarcoma (56%) and
Ewing tumors (34%) are much more common than chondrosarcoma (6%).
Chondrosarcomas develop most often in adults, with an average age at diagnosis
of 51. Less than 5% of cases occur in patients younger than 20.
Chordomas are also more common in adults. Less than 5% of cases occur in
patients younger than 20.
Both osteosarcomas and Ewing tumors occur most often in children and teens.

Classification of bone
tumors

Bone tumors are classified into:

Primary bone tumors
Secondary bone tumors ( Metastasis)

Most are classified according to the normal

cell of origin and apparent pattern of
differentiation

Classification
Based

on tissue of origin

Bone
Cartilage
Fibrous tissue
Bone marrow
Blood vessels
Mixed
Uncertain origin

Primary Bone Tumors

Bone-Forming tumors

Osteoma

Osteoid osteoma
and osteoblastoma

Osteosarcoma
Cartilage-Forming
tumors

Chondroma
(Enchondroma)

Osteochondroma

Chondrosarcoma

Miscellaneous tumors

Ewings sarcoma

Giant cell tumor of

bone

Bone-Forming

Tumors

Osteoma

Osteoma are benign lesions of bone that in

many cases represent developmental aberrations
or reactive growths rather than true neoplasms.

Site;
Age;
Gross:
Histology:

Osteosarcoma
Osteosarcoma is a bone-producing
malignant
mesenchymal tumor
Incidence
Age
Sex
site
.

Osteosarcoma
Distribution

Bone-Forming tumors;

BENIGN

MALIGNANT

Tumor Type

Locations

Age

Morphology

Osteoma

Facial bones, skull

40-50

Exophytic growths
attached to bone
surface; histologically
resemble normal bon

Osteoid osteoma

Metaphysis of femur
and tibia

10-20

Cortical tumors,
characterized by
pain; histologically
interlacing trabeculae
of woven bone

Osteoblastoma

Vertebral column

10-20

vertebral processes;
histologically similar
to osteoid osteoma

Primary

Metaphysis of distal
femur, proximal tibia,
and humerus

10-20

Grow outward, lifting

periosteum, and
inward to the
medullary cavity;
microscopically
malignant cells form
osteoid.

Femur, humerus,
pelvis

>40

Complications of
polyostotic Paget
disease;
histologically similar
to primary
osteosarcoma

osteosarcoma

Secondary
osteosarcoma

Cartilage -Forming
Tumors

Osteochondroma
Morphology

Osteochondromas are mushroom shaped and range in size from

1 to 20 cm.
The outer layer of the head of the osteochondroma is composed
of benign hyaline cartilage varying in thickness
Newly formed bone forms the inner portion of the head and
stalk, with the stalk cortex merging with the cortex of the host

Chondrosarcoma

Chondrosarcomas

comprise a
variety of tumors sharing the
ability to produce neoplastic
cartilage

Chondrosarcoma
Gross features

SITE;
pelvis,
shoulder,
ribs.
rarely involve the distal extremities.

Cartilage-forming Tumors;
Tumor Type

BENIGN

MALIGNANT

Locations

Age

Morphology

Osteochondroma

Metaphysis of long
tubular bones

10-30

Bony excrescences
with a cartilaginous
cap; may be solitary
or multiple and
hereditary

Chondroma

Small bones of hands

and feet

30-50

Well-circumscribed single
tumors resembling
normal cartilage; arise
with medullary cavity of
bone; uncommonly
multiple and hereditary

Chondrosarcoma

Bones of shoulder,
pelvis, proximal
femur, and ribs

40-60

Arise within
medullary cavity and
erode cortex;
microscopically well
differentiated
cartilage-like or
anaplastic

Giant Cell Tumor

This is a neoplasm that contains large

numbers of osteoclast like giant cells admixed
with mononuclear cells.

These tumors are slightly more common in

females.

SECONDARY TUMORS

Secondary tumors further subdivided

Metastatic tumors
Tumors resulting from contiguous spread of adjacent soft
tissue neoplasms
Tumors representing malignant transformation of the preexisting benign lesions

METASTATIC TUMORS
Features of MT.
-More common than primary bone tumors
-Predominant occurrence in two age groups: adults over 40 years
of age and children in the first decade of life.
Multifocality
Predilection for the hematopoietic marrow sites in the axial
skeleton (vertebrae, pelvis, ribs and cranium) and proximal long
bones.

METASTATIC TUMORS

Features of MT.

Metastases to long bones distal to the elbows and knees are

unusual.
Metastases to the small bones of the hands and feet are even
rarer.
Occasionally, metastases may appear as solitary lesions
(particularly true for the lung, kidney and thyroid cancer).

METASTATIC TUMORS
Common malignancies producing sk
metastases

Adults

Prostate, breast, kidney,and lung.(>75%)

Thyroid and colon cancers & melanoma.

Children

Neuroblastoma
Rhabdomyosarcoma
Retinoblastoma

METASTATIC TUMORS
Radiographic appearance of the metastatic tumors

Purely lytic (kidney, lung, colon, and melanoma)

Purely blastic (prostate and breast carcinoma)
Mixed lytic and blastic (most common appearance)

CLINICAL PRESENTATION

Pain (May be a presenting feature in certain tumors)

May indicate
Rapid expansion of the lesion with stretching of surrounding soft tissue
Central hemorrhage or degeneration in the tumor
Incipient pathological fracture
A tiny lesion may be very painful if it is encapsulated in a dense bone
(e.g. an osteoid osteoma)

CLINICAL PRESENTATION

Swelling /Lump (May be alarming often a patient seek

advice when a mass becomes painful
or continuous to grow )

Examine the lump

Discrete or ill-defined
Soft ,Hard, or Pulsatile

Overlying skin is warm or inflamed

Tender / Non tender

CLINICAL PRESENTATION

Examine the lump

Is there any effusion or limitation in movement in the joint
adjacent to the lump
Examine the limb distal to the lump for any oedema,muscle
wasting, and N/V status
Examine for draining lymph nodes

INVESTIGATIONS
Lab Investigations
HB
ESR
Serum Alkaline phosphatase
Serum protein electrophoresis
Bence-jones protein
Serum Acid phosphatase

INVESTIGATIONS
Biopsy:

(essential for accurate diagnosis)

(delay biopsy untill the imaging
studies have bee completed)

TYPES: (1) Large bore needle biopsy

(2) Open biopsy
(a) excisional biopsy
(b) incisional biopsy

INVESTIGATIONS
Guide-lines for incisional biopsy
Longitudinal incision
Planned so that biopsy incision could be removed completely during
definitive procedure
Violate only one compartment
Split muscles
Plug bone defects
Meticulous homeostasis

INVESTIGATIONS
Guide-lines for incisional biopsy (cont)
Experienced surgeon
Ideally in same institution where definitive procedure is
planned
No important N/V structure should be exposed
Collect sample from periphery of the tumor

INVESTIGATIONS
Biopsy Complications:
Hemorrhage
Wound breakdown
Infection
Pathological fracture

INVESTIGATIONS
Imaging studies
Plain X-rays
Bone scintigraphy
CT
MRI

INVESTIGATIONS
Plain x-rays: (should answer the following questions)
Precise location of the lesion
Any evidence of underlying bone abnormality (eg., bone
infarct,pathological fracture)
Is the lesion multifocal?
Does the tumor have a well-defined margin? Is there a rim of
sclerotic bone? (usually benign tumors)

Site of the lesion.

Distribution of various
lesions in a long
tubular bone in a
growing skeleton

Distribution of various
lesions in a long tubular
bone after skeletal
maturity

tribution of various lesions in a vertebra

Malignant lesions are
seen predominantly in
its anterior part (body)

Benign lesions
predominate in its
posterior elements.

Patterns of bone destruction.

geographic

moth-eaten

a uniformly affected
area within sharply
defined borders

rapidly growing
infiltrating lesions

giant cell tumor.

myeloma

permeative type
characteristic of
round cell tumors

Ewing sarcoma

INVESTIGATIONS
Plain x-rays (should answer the following questions)
Is there evidence of significant cortical expansion or
destruction? (locally aggressive or malignant tumors)
Is there an associated periosteal reaction
Does the lesion produce mineralized matrix (osteoid or
cartilage)?
Is there a soft tissue mass?

INVESTIGATIONS
BONE SCINTIGRAPHY
Highly sensitive
Relatively non-specific
main role is in detecting of skip lesions or silent
secondary deposits in the whole skeleton.
Helpful in the detection of a small tumor (e.g. osteoid
osteoma )

INVESTIGATIONS
COMPUTER TOMOGRAPHY (CT)

When plain film assessment is difficult due to the nature of

the lesion (e.g. permeative pattern of destruction)

anatomic site (e.g. sacrum)

CT is the best technique in assessment of

matrix mineralization
cortical detail
detection of the cystic and fatty lesions.

INVESTIGATIONS
MRI

is a method of choice for local staging.

It is superior to CT in the definition of

medullary and extra cortical spread
relationship of the tumor to critical neurovascular
structures.

STAGING
Staging of Malignant bone & soft tissue tumors
(Enneking system)

Grade
In the Enneking system, bone tumors are
graded as follows:
G0 - Benign lesion
G1 - Low-grade malignant lesion
G2 - High-grade malignant lesion

STAGING
Staging of Malignant bone & soft tissue tumor
(Enneking system)

Site
In the Enneking system, the site and local extent of bone
tumors are classified as follows:

T0- A benign tumor that isconfined within a true capsule and the lesion's anatomic
compartment of origin ( i.e., a benign intracapsular, intracompartmental lesion

T1-An aggressive benign or malignant Tumor that is still confined within its anatomic
compartment (i.e., an intracompartmental lesion)

T2 - A lesion that has spread beyond its anatomic compartment of origin ( i.e., an extra
compartmental lesion)

STAGING
Staging of Malignant bone & soft tissue tumor
(Enneking system)

Metastasis
Metastatic classification in the
Enneking system is as follows:
M0 - No regional or distant metastasis
M1 - Regional or distant metastasis

STAGING
Staging of Malignant bone & soft tissue tumor
(Enneking system)
Stage

Grade

Site

Metastasis

IA

G1

T1

M0

IB

G1

T2

M0

IIA

G2

T1

M0

IIB

G2

T2

M0

III

G1 / G2

T1/T2

M1

TREATMENT
RADIATION THERAPY.
Radiation causes cell death by inducing the formation of
intracellular free radicals that subsequently cause DNA
damage.

Most primary bone malignancies are relatively radioresistant.

( Exceptions are the marrow cell tumors, including multiple myeloma,
lymphoma, and Ewing sarcoma, each one is radiosensitive).
Carcinomas Metastatic to bone, with the exception of renal cell carcinoma,
also frequently are sensitive to radiation treatment.

TREATMENT

RADIATION THERAPY.
Radiotherapy can be used to reduce the incidence of local
recurrence of malignant soft-tissue tumors treated
with marginal resection.

Radiation also can be used for preoperative treatment

of soft-tissue sarcomas in the hopes of reducing the
tumor volume and making the resection easier.

TREATMENT
CHEMOTHERAPY

Neoadjuvant chemotherapy refers to chemotherapy administered

before
surgical resection of the primary tumor.

Adjuvant chemotherapy refers to chemotherapy administered

postoperatively to
treat presumed micro metastases.

TREATMENT
CHEMOTHERAPY

Currently most orthopedic oncologists favor preoperative

chemotherapy with the definitive procedure performed 3 to 4
weeks after the last dose has been administered.
Chemotherapy is restarted 2 weeks postoperatively if the wound
has healed.

TREATMENT

Chemotherapeutic Agents Commonly Used for Treatment of Bone

and Soft-Tissue Tumor

Cyclophosphamide
Cisplatin
Methotrexate
Doxorubicin
Dactinomycin
Etoposide
Vincristine

TREATMENT

Surgery

AMPUTATION vs LIMB SALVAGE.

Advances in diagnostic imaging, chemotherapy,
radiation therapy, and surgical technique for
resection and reconstruction now allow limb
salvage to be a reasonable option for most
patients with bone or soft-tissue sarcomas.

TREATMENT
Limb Salvage Surgery

Indications
The tumor is situated in the extremities and/or the axial skeleton.
The tumor margins are amenable to surgery.
Only moderate soft-tissue extension is present.
The neurovascular bundles are intact.
Metastases are absent or amenable to curative treatment.
The patient is in good general health

TREATMENT
Limb Salvage Surgery

Complications
Infection
Wound dehiscence
Flap necrosis
Blood loss

TREATMENT
Limb Salvage Surgery

Complications
Deep venous thrombosis
Periprosthetic fractures
Prosthetic loosening or dislocation
Allograft fracture
Leg-length discrepancy

TREATMENT

( MARGINS)

An intralesional margin is one in which the plane

of surgical dissection is within the tumor,
described as debulking because it leaves
behind gross residual tumor, appropriate as a
palliative procedure in the setting of Metastatic
disease.

A marginal margin is achieved when the closest

plane of dissection passes through the pseudo
capsule. This type of margin usually is adequate
to treat most benign lesions and some low-grade
malignancies. (High recurrence)

TREATMENT

( MARGINS)

Wide margins are achieved when the plane of

dissection is in normal tissue, the entire tumor
remains completely surrounded by a cuff of
normal tissue. optimal surgical margins are 6 cm
of healthy bone around the bone margins and 2
cm of healthy soft tissue around the soft-tissue
extent of the tumor

TREATMENT

( MARGINS)

Radical margins are achieved when all the

compartments that contain tumor are
removed en bloc.
For deep soft-tissue tumors, this involves
removing the entire compartment (or multiple
compartments) of any involved muscles.
For bone tumors, this involves removing the entire
bone and the compartments of any involved
muscles.

TREATMENT

( MARGINS)

TREATMENT ( Amputation Levels)

TREATMENT ( Surgical Resection)

Benign tumors
Stage 1 tumors -Intracapsular excision (or curettage)
is adequate.
Stage 2 tumors -Extracapsular excision passing
through the reactive zone is needed.
Stage 3 tumors -Wide margins of resection are required in stage
3 lesions (aggressive benign tumors). In areas
that are not amenable to wide
excision,marginal
excision together with adjuvant treatment (eg,
radiation therapy) may be acceptable.

TREATMENT ( Surgical Resection)

Malignant tumors
Stage IA-Thesetumors are treated with wide
excision and are usually amenable
to limb salvage procedures
Stage IB-Suchtumors may be treated with wide
excision, but the choice between
amputation and limb salvage depends on
the estimated amount of residual tumor left
behind after a limb salvage procedure.

TREATMENT ( Surgical Resection)

Malignant tumors
Stage II - Require radical amputation or
disarticulation in most patients.
However, bone tumors responsive to
chemotherapy may be treated
successfully using wide excision and
adjuvant therapy.

TREATMENT ( Surgical Resection)

Malignant tumors
Stage III -Tumors at this stage that are
responsive to chemotherapy and
may be treated with aggressive
resection.Those that arenot
responsive to adjuvant therapy
should be treated with palliative
resection.