Sarcoma

Definition
• Mesenchymal connective tissue cancer
originated from mesoderm on supporting
tissue including cartilage, muscle, fat, nerve,
vessels and another connective tissue
including bone.

Spieler P, Rössle M. Soft Tissue and Bone. 2012. 1053–1105 p.

 Epidemiology
• Bone sarcomas account for only 0.2% of all new cancer
diagnoses in the UK, an incidence of only 8.2 cases per
million of the population.
• Soft-tissue sarcomas occur comparatively more frequently,
with an incidence of 45 cases per million of the
population. 
• The incidence of bone and soft-tissue sarcomas is
marginally higher in males than females.
• Soft-tissue sarcomas, the incidence increases with age,
with the highest incidence occurring in males over 85
years. 
 Classification and
Distribution

Fig.1 Distribution of new sarcoma cases by

Burningham Z, Hashibe M, Spector L, histology
Schiffman JD. The Epidemiology of
Sarcoma. Clin Sarcoma Res. 2012;2(1):14.
 Classification
Table 1. The classification of tumours of bone (based on the World Health Organization (WHO)
histological classification of tumours)

Apley, A. G., & Solomon, L. (2018). Apley's system of orthopaedics and trauma. 10th Ed London: Arnold.
 Classification
Table 2. Definition of histopathological parameters in the Federation Nationale des Centres de Lutte Contre le
Cancer (FNCLCC) grading system (HPF = high-power field)

Apley, A. G., & Solomon, L. (2018). Apley's system of orthopaedics and trauma. 10th Ed London: Arnold.
 Risk Factor
• Commonly studied birth anomaly
that has been found to be
Inguinal and
umbilical Hernia associated with Ewing’s sarcoma

• The risk of rhabdomyosarcoma

increasing between cases with
Maternal Age older mother

Burningham Z, Hashibe M, Spector L, Schiffman JD. The Epidemiology of Sarcoma. Clin Sarcoma Res. 2012;2(1):14.
 Risk Factor
• Farmers, farm managers, and market gardeners have a
significant increase in risk for developing soft tissue
sarcomas.
Job

• Fluoride exposure in drinking water has been studied as a

potential risk factor in the development of osteosarcomas
• Fluoride is known to act as a mitogen, increasing the
Drinking proliferation of osteoblasts and the uptake of fluor- ide in
Water the bone during periods of growth.

Burningham Z, Hashibe M, Spector L, Schiffman JD. The Epidemiology of Sarcoma. Clin Sarcoma Res. 2012;2(1):14.
 Clinical Findings
• Patients may be asymptomatic until
the lesion is discovered incidentally
on radiographs, common for benign
lesions, or for malignant lesions
arising in areas where there is room
for innocuous expansion, such as
the pelvis, where tumours can
achieve a very large size prior to
presentation.

A soft tissue swelling of the leg

Johnson CJD, Pynsent PB, Grimer RJ. Clinical features of soft tissue sarcomas. Ann R Coll Surg Engl. 2001;83(3):203–5.
 Clinical Findings
• Age is often a consistent feature for primary bone
tumours; osteosarcoma and Ewing’s sarcoma have
preponderance for children, adolescents and
young adults.
• Chondrosarcoma typically occurs in older patients
• The most common symptom experienced by a
patient with a bone tumour is pain. The pain is
initially mild but gets worse as the tumour
increases in size.
Apley, A. G., & Solomon, L. (2018). Apley's system of orthopaedics and trauma. 10th Ed London: Arnold.
 • Pathological fracture has been reported in between 5 and
12% of osteosarcomas and up to 21% of
chondrosarcomas, and in the case of benign lesions is
suggestive of a locally aggressive lesion.
• Certain fracture patterns should raise concern for an
underlying lesion, such as supracondylar femoral fractures
in children, and avulsion fractures of the lesser trochanter
in adults. 
•  In elderly patients with diaphyseal long-bone fractures,
the possibility of a pathological fracture should be
considered
Apley, A. G., & Solomon, L. (2018). Apley's system of orthopaedics and trauma. 10th Ed London: Arnold.
 Clinical Findings
• Swelling and tenderness over the affected
bone are the most common findings but there
will be limitation of joint movement if there
has been irritation of the joint by the tumour,
or the tumour has grown into the joint

Apley, A. G., & Solomon, L. (2018). Apley's system of orthopaedics and trauma. 10th Ed London: Arnold.
  Fig 3. Potential diagnoses The location of the lesion within the bone is
significant.

Apley, A. G., & Solomon, L. (2018). Apley's system of orthopaedics and trauma. 10th Ed London: Arnold.
 Investigations
• X-rays. All patients with the suspicion of a lesion of
bone should be investigated with plain X-rays. There
may be an obvious abnormality in the bone, such as
cortical thickening, a ‘cyst’, or ill-defined destruction.
• Radionuclide scanning. Scanning with 99mTc-methyl
diphosphonate (99mTc- MDP) shows non-specific
reactive changes in bone which can be helpful in
revealing the site of a small tumour (e.g. an osteoid
osteoma)

Apley, A. G., & Solomon, L. (2018). Apley's system of orthopaedics and trauma. 10th Ed London: Arnold.
 Investigations
• Computed tomography. CT extends the range
of X-ray diagnosis; it shows more accurately
both intraosseous and extraosseous extension
of the tumour and the relationship to
surrounding structures.
• CT is essential to complete systemic staging
and re-staging in bone and soft- tissue
sarcomas and metastatic disease to identify
pulmonary metastases.
Apley, A. G., & Solomon, L. (2018). Apley's system of orthopaedics and trauma. 10th Ed London: Arnold.
 Investigations
• Magnetic Resonance Imaging. MRI allows
further characterization of lesions and defines
the local extent of the lesion.
• Its greatest value is in the assessment of
tumour spread: (a) within the bone, (b) into a
nearby joint, and (c) into the soft tissues.
• Useful in assessing soft-tissue tumours and
cartilaginous lesion.

Apley, A. G., & Solomon, L. (2018). Apley's system of orthopaedics and trauma. 10th Ed London: Arnold.
 Investigations
• Questions to ask when assessing an x-ray

Apley, A. G., & Solomon, L. (2018). Apley's system of orthopaedics and trauma. 10th Ed London: Arnold.
 Table 3. Possible diagnosis based on the radiographic appearances,
divided by age group

Apley, A. G., & Solomon, L. (2018). Apley's system of orthopaedics and trauma. 10th Ed London: Arnold.
 Investigations
• Biopsy.  The gold standard for obtaining a
diagnosis in an abnormal lesion of bone. 

Fig. The majority of biopsies are now performed with a core needle, such as a Jamshidi needle.
The procedure is often performed with radiographic guidance, either as fluoroscopy (b), or
under CT guidance (c).

Apley, A. G., & Solomon, L. (2018). Apley's system of orthopaedics and trauma. 10th Ed London: Arnold.
 Osteosarcoma The characteristic
features of a distal femoral
osteosarcoma as seen on plain
radiographs

Apley, A. G., & Solomon, L. (2018). Apley's system of orthopaedics and trauma. 10th Ed London: Arnold.
 The lesion, located in the metadiaphysis, demonstrates a mixed lytic blastic
appearance. There is elevation of the periosteum resulting in a Codman’s triangle.
The soft-tissue extension of the tumour results in bone formation within the
surrounding soft tissues and the appearance of sunray spiculations.
On MRI

Apley, A. G., & Solomon, L. (2018). Apley's system of orthopaedics and trauma. 10th Ed London: Arnold.
 Chondrosarcoma–central chondrosarcoma The
challenge of the central chondroid lesion. Plain
X-rays; demonstrate an 8.5 cm chondroid
lesion in the distal femur which remained
unchanged on radio- graphs over a period of
12 months

Apley, A. G., & Solomon, L. (2018). Apley's system of orthopaedics and trauma. 10th Ed London: Arnold.
Minor endosteal scalloping of the anterior femoral cortex
can be seen which, combined with the advent of pain in
the thigh, warranted resection of the tumour. Histology
post resection confirmed a low-grade chondrosarcoma.
 Differential Diagnosis
• There are a variety of bone and soft-tissue
lesions which can cause great concern
clinically, radiologically and sometimes
pathologically.
• If in doubt, the case should be discussed with
a specialist centre where expertise will be
available to obtain the correct diagnosis and
advice on treatment.

Apley, A. G., & Solomon, L. (2018). Apley's system of orthopaedics and trauma. 10th Ed London: Arnold.
 • Bone and soft tissue lesions that mimicking the bone tumors
- Soft tissue haematoma
- Myositis ossificans
- Stress fracture
- Tendon avulsion injuries
- Infection
- Gout
- Osteopetrosis
- Osteopoikilosis
- Melorheostosis
Apley, A. G., & Solomon, L. (2018). Apley's system of orthopaedics and trauma. 10th Ed London: Arnold.
 Management
• The management of patients with bone
sarcomas must be carried out by a
multidisciplinary team

Apley, A. G., & Solomon, L. (2018). Apley's system of orthopaedics and trauma. 10th Ed London: Arnold.
 Principles of Surgical Treatment
• The resection of all detectable tumours,
including metastases, is the cornerstone of
treatment of the majority of sarcomas of
bone. 
• For primary operable tumours, the options are
tumour excision with limb salvage, or
amputation.

Apley, A. G., & Solomon, L. (2018). Apley's system of orthopaedics and trauma. 10th Ed London: Arnold.
 Fig. Margins of excision The margins of tumour excision defined by Enneking
(soft-tissue sarcoma). When considering tumours of bone, the bone can be
considered as a compartment.

Apley, A. G., & Solomon, L. (2018). Apley's system of orthopaedics and trauma. 10th Ed London: Arnold.
 • Many benign lesions can be managed by
intralesional excision. This would apply to
curettage of a benign lesion but would also
apply if an attempt was made to excise a
malignant lesion and the tumour was actually
cut across during the operation, allowing
tumour spill.

Apley, A. G., & Solomon, L. (2018). Apley's system of orthopaedics and trauma. 10th Ed London: Arnold.
 • A marginal excision, going around the edge of
the lesion, will usually be sufficient to
completely remove and control a benign or
intermediate lesion but will usually be
insufficient for a malignant tumour as there
are likely to be tumour cells in the tissues left
behind.

Apley, A. G., & Solomon, L. (2018). Apley's system of orthopaedics and trauma. 10th Ed London: Arnold.
 • A wide excision implies that the surgery has
been carried out through completely normal
tissue, well away from the tumour.
• The decision between carrying out limb
salvage or amputation is governed by the
expected postoperative limb function,
potential for complications, psychological
acceptance and oncological outcomes.

Apley, A. G., & Solomon, L. (2018). Apley's system of orthopaedics and trauma. 10th Ed London: Arnold.
 Neoadjuvant and Adjuvant Therapies
• Neoadjuvant chemotherapy has been used for most
patients with osteosarcoma and Ewing’s sarcoma for
well over 35 years.
• In osteosarcoma the principle drugs used are
doxorubicin, cisplatin, ifosfamide and high-dose
methotrexate, in various combinations to avoid
chemo-resistance and increase the rate of tumour
necrosis.
• The aim is to try to shrink the tumour while also
treating the micrometastatic disease.
 Neoadjuvant and Adjuvant Therapies

• Neoadjuvant chemotherapy allows the

assessment of the tumour’s sensitivity to
chemotherapy following definitive surgical
resection, itself a predictor of local recurrence
and disease-free survival.
• As Ewing’s sarcoma is often sensitive to
chemotherapy, most patients will receive
neoadjuvant chemotherapy, regardless of the
extent of the local disease.
 Neoadjuvant and Adjuvant Therapies

• High-energy irradiation has long been used to

destroy radiosensitive tumours or as adjuvant
therapy before operation.
• Nowadays the indications are more restricted.
For highly sensitive tumours (such as Ewing’s
sarcoma), it offers an alternative to
amputation or as an adjunct to surgery and
chemotherapy for tumour locations where
achieving an adequate margin may be difficult
 Complication

• The complication of sarcoma varies, start from

infection, necrosis and hematoma
• The complication significantly lower in upper
extremity (17%) compared to trunk and lower
extremity (45%)

Saddegh, M. K, Bauer H. C. Wound complication in surgery of soft tissue sarcoma. Analysis of 103 consecutive patients managed without
adjuvant therapy . Clin Orthop Relat Res
. 1993 Apr;(289):247-53.
 Prognosis
• It’s going to have better prognosis in soft-
tissue sarcoma, size < 5 cm, low histology
grading, and located superficial from fascia.
• Majority of patient with recurrency after
therapy in first two years and more than 85% -
90% recurrent in five years

Johnson CJD, Pynsent PB, Grimer RJ. Clinical features of soft tissue sarcomas. Ann R Coll Surg Engl. 2001;83(3):203–5.
Nakamura T, Grimer RJ, Carter SR, Tillman RM, Abudu A, Jeys L, et al. Outcome of soft-tissue sarcoma patients who were alive and event-free more than five years after initial
treatment. Bone Jt J. 2013;95 B(8):1139–43.