Principle of bone tumor:

Q. What is tumor?
Answer: according to national cancer institute, USA, tumor is an abnormal mass of tissue that
forms when cells grow and divide more than they should or do not die when they should. Tumors
may be benign (not cancer) or malignant (cancer).

Q. What is neoplasm?
Answer: Willis defined the term, 'neoplasia' as an abnormal mass of tissue, the growth of which
exceeds and is uncoordinated with that of the normal tissues and persists in the same excessive
manner after cessation of the stimuli which evoked the change.

Q. What are the types of bone tumor?

Answer: bone tumors are classified as-
A. Primary

A benign lesion of bone is defined as one that does not invade surrounding tissue or
spread elsewhere in the body.
Intermediate (locally aggressive) lesions of bone can destroy bone and surrounding tissue
(e.g. osteoblastoma).
Intermediate (rarely metastasizing) lesions often behave in a similar way to locally
aggressive lesions but occasionally demonstrate the ability to spread to distant sites.
Malignant tumors are truly aggressive with the potential for both local extension and
metastases to distant sites.

B. Secondary: metastasis from- thyroid, prostate, lungs, breasts, kidneys. (All tumors are
osteolytic except from kidneys are osteosclerotic).
Q. What are classification of spinal tumors?
Answer: spinal tumors classifications:
A. Extradural (55%):
1. Metastatic-lymphoma, from lungs,
Breasts, prostate, kidneys.
2. Primary spinal tumors (rare) -
Chordoma, neurofibroma, osteoid
Osteoma, osteoblastoma, ABC,
Hemangioma.

B. Intradural extramedullary (40%):

1. Meningioma
2. Neurofibroma
3. Lipomas
4. Metastases

C. Intradural intramedullary (05%):

1. Astrocytoma
2. Ependymoma
3. Hemangiblastoma
4. Others- glioblastoma, dermoid, teratoma.

Q. What are the histological parameters for tumor staging?

Answer: Definition of histopathological parameters in the Federation Nationale des Centres de
Lutte Contre le Cancer (FNCLCC) grading system (HPF = high-power field):
Q. What is tumor grading and staging?
Grading: Grading of a cancer is based on the degree of differentiation of the tumor cells and, in
some cancers, the number of mitoses and the presence of certain architectural features. The grade
of the tumour (a measure of the aggressiveness of the tumour – high, intermediate or low), the
size of the tumour, the local extent, and the presence of metastases.
Types: recommended by the American Joint Commission on Cancer and other bodies:
GX Grade cannot be assessed
G1 Well differentiated (Low grade)
G2 Moderately differentiated (Intermediate grade)
G3 poorly differentiated (High grade)
G4 Undifferentiated (High grade

Staging: The staging of solid cancers is based on the size of the primary lesion, its extent of
spread to regional lymph nodes, and the presence or absence of blood borne metastases. Staging
is the process of assessing the extent of a tumour both locally and distantly.
Types: American Joint Committee on Cancer Staging. This system uses a classification called
the TNM system—T for primary tumor, N for regional lymph node involvement, and M for
metastases.
When compared with grading, staging has proved to be of greater clinical value. Higher-grade
lesions have a >25% risk of local recurrence and distant spread, whereas low-grade lesions a
<25% risk of local recurrence and metastases.

Q. What are the staging system of bone tumors?

a. The classic staging system for primary malignant tumours of bone is the Enneking
system:
Stage Grade Compartment Metastases
1A Low Intra-compartment tumor Absent
1B Low Extra-compartment tumor Absent
2A High Intra-compartment tumor Absent
2B High Extra-compartment tumor Absent
3 Any Metastases Local or distant
Importance: extent of surgical resection in primary tumours of bone

b. Conventional tumour– node–metastasis (TNM) staging has been applied to primary

sarcomas of bone, developed by American Joint Committee on Cancer (AJCC), and the
Union for International Cancer Control (UICC):
Stage Grade Size Metastases
1A Low Small (soft tissue sarcoma<5cm, bony sarcoma<8cm). Absent
1B Low Large (soft tissue sarcoma>5cm, bony sarcoma>8cm). Absent
2A Intermediate Small Absent
2B Intermediate Large Absent
3 High Large Absent
4 Any Any Present
 c. Enneking staging for benign bone tumors:
Stages Description Margin control
1. Latent Well defined margin. Intracapsular
Grows slowly and then stops.
Heals spontaneously. E.g. osteoid osteoma.
Negligible recurrence after intracapsular resection.
2. Active Progressive growth limited by natural barriers. Intracapsular/margi
Well defined margins but may expands thinning nal plus effective
cortex. E.g. ABC. adjuvant
Negligible recurrence after marginal resection.
3. Aggressive Growth not limited by natural barriers. E.g. GCT. Wide/marginal plus
Metastases present in 5% case. effective adjuvant
High recurrence after intracapsular or marginal
resection, extended resection preferred.

Q. What are the clinical features of bone tumors?

Answer:
 History:
1. Age: young- GCT, Ewing’s tumor, osteosarcoma, older age- metastases, secondary
osteosarcoma.
2. History of radiation exposure, Paget’s disease.

 Symptoms: Swelling and pain, Benign- swelling first than pain, long history (Years).
Malignant- Pain first than swelling, short history (Months).
 Worrisome features-Night pain, pain not responding to simple analgesia, persistent pain
following injury, as well as prior benign or malignant lesions, family history and previous
radiotherapy.

 Distal neurology- paranesthesia or numbness are suggestive of compression of a nerve by

an expanding mass, progressive neurological dysfunction is far more worrisome and is
suggestive of direct tumour invasion.
 Pathological fracture- has been reported in between 5 and 12% of osteosarcomas and up
to 21% of chondrosarcomas.

Q. What are the pre-cancerous lesion of

bone?
Q. What are the most common sites of bone
metastases?
Most common sites of bone metastases:
1. Spine
2. Proximal femur
3. Proximal humerus
Q. What are the Warning signs – bone tumour?
Answer:
1. Non-mechanical bone pain
2. Especially around the knee in young adolescents
3. Concerning radiographs.

Q. What are the Warning signs – soft tissue tumour?

Answer:
1. Larger than 5 cm
2. Increasing in size
3. Painful
4. Deep to the fascia
5. Recurrence after previous excision

Q. How can you evaluate a case with bone tumors?

Answer: The assessment and investigation of any patient with a bone or soft tissue tumour can be
divided into three phases. The first two phases can be performed at the referring hospital, but the
third phase may be best done in a specialist centre.

Phase 1 (within 24 hours, at referring hospital)

1. History and examination
2. Bloods
 CBC- leukocytosis, pancytopenia
 ESR- high raised
 ALP, LDH –Positive
 CRP- raised
3. Radiograph whole bone- sites and types of lesions, osteolytic or osteosclerotic and skin
metastases.
4. Chest radiograph- pulmonary metastases.

Phase 2 (within first week, at referring hospital)

1. Bone scan- for suspected primary or metastatic bone tumours);
2. Ultrasound scan abdomen- if renal metastasis is a possibility.
3. CT-scan chest- pulmonary metastases.

Phase 3 (at specialist centre)

1. CT-scan lesion- for staging and more sensitive than X-Rays.
2. MRI-scan lesion- staging and soft tissue status.
3. Biopsy- confirm the tumors variety.
Q. What is biopsy? What are the types of biopsy? How can you take bone biopsy?
Answer: Biopsy: it’s a surgical procedure of taking cells or tissues from living human body for
microscopic and other laboratory investigations to establish the diagnosis.

Types:
A. Open biopsy
1. Incisional biopsy
2. Excisional biopsy
3. Frozen section biopsy
B. Closed biopsy
1. FNAC
2. Tru-cut biopsy
3. Brush biopsy
4. Punch biopsy
5. Shaving biopsy
6. Aspiration biopsy
7. Image or endoscopic guidance biopsy

Bone biopsy principles:

1. Only biopsy once staging is completed
2. Biopsy should be performed at the institution undertaking the main surgery
3. Imaging-guided biopsy is more reliable
4. The biopsy track must be excised at definitive surgery
5. Jamshidi needles for bone, Trucut needles for soft tissues

Biopsy technique:
1. A tourniquet can be used; but exsanguination by compression should be avoided as
this may theoretically disseminate the tumour locally or into the circulation.
2. Use longitudinal incisions that are part of an extensile approach.
3. Do not cross anatomical compartments or contaminate critical anatomical structures
(e.g. nerves or blood vessels).
4. Use a biopsy track that can be excised at the time of definitive surgery.
5. Ensure specimens are sent for microbiology as well as histopathology.
6. Some specimens should be sent fresh to the laboratory for cytogenetic studies.
7. Complete hemostasis must be achieved.
8. Samples should always be sent for microbiology as well as histology.
9. The pathologist reporting the biopsy must have an appropriate level of experience.
10. If there is a risk of fracture following biopsy, the
bone must be appropriately splinted.

Figure: Bone marrow biopsy trocar and cannula. Green is 8

gauge, pink is 9, white is 11 and blue is 13 gauge.
Q. What are the treatment for bone tumor?
Answer:
Treatment of benign bone tumours:
 Benign lesions can be simply curetted
 CT-guided thermocoagulation is used for osteoid osteoma
 Large benign tumours may require reconstruction
Treatment of malignant bone tumours:
 Osteosarcomas and Ewing’s sarcoma require neoadjuvant chemotherapy
 Chondrosarcomas are insensitive to radiotherapy or chemotherapy
 Most malignant tumours can be treated with limb salvage
 There is no difference in survival between amputation and limb salvage
Treatment of bone metastases:
 Surgery cannot lengthen life but may shorten it
 The spine may need stabilizing and nerves or the cord decompressing
 Long bones will need stabilizing if a pathological fracture is imminent
 Patients who have a possibility of long-term survival may need a prosthesis
 Radiotherapy relieves pain
Classification of surgical resection margins:
Surgical margins Resections
Intralesional Resection through the tumour
Marginal Resection through the reactive zone of the tumour
Wide resection Resection outside the reactive zone of the tumour
Radical resection Radical Resection of the whole anatomical compartment
The surgical options for malignant primary bone tumours include:
 Amputation or van Ness rotationplasty;
 Excision alone (for dispensable bones or areas where reconstruction is difficult, e.g. in
parts of the pelvis);
 Excision and replacement with graft or massive endoprosthesis.
Preoperative embolization- Renal metastases tend to be very vascular and massive blood loss
can be encountered during surgery. Therefore, embolization should be considered just before
surgery to prevent blood loss.
Chemotherapy- neo-adjuvant or adjuvant. E.g. doxorubicin, methotrexate, cisplatin,
isofosphamide.
Radiotherapy- tumor at in operable sites-pelvis, ribs, sternum, vertebral body. Spinal
compression by tumor, palliative pain remedy, multiple myeloma, lymphoma.
Denosumab- monoclonal antibody for the treatment of osteoporosis, treatment-induced bone loss,
metastases to bone, and giant cell tumor of bone. Denosumab is an inhibitor of RANKL (receptor
activator of nuclear factor kappa-Β ligand), which works by decreasing the development of
osteoclasts, which are cells that break down bone, metastases bone tumors.
Mifamurtide- is an immune macrophage stimulant. This means it works by encouraging the
immune system to produce specialised cells that kill osteosarcoma cancerous cells.
Q. What are the tumor markers?
Answer:
Tumor Immunohistochemical marker
Metastatic osteosarcoma Bone ALP,
Low-grade osteosarcoma CDK4,MDM2
Conventional osteosarcoma Osteocalcin, osteonectin,S100
Ewing Sarcoma CD99,O13,FLI 1
Chordoma Brachyury
Synovial sarcoma TLE 1
Vascular tumor CD 31
Metastasis from prostate PSA

Q. What is Mirel score?