EDUCATION UPDATE: April 2012

Ewings Sarcoma

Posted by Prajwal Rijal

Synonym- Endothelial sarcoma of bone

Swings sarcoma is an uncommon but highly malignant sarcoma arising from endothelial cells in the bone marrow.

Age incidence- usually between 10 and 20 years.

Common sites- shaft of femur, tibia, fibula, humerus or clavicle.

Pathology:

The tumour tissue is soft and vascular. As it expands it gradually destroys the bone substance. There is striking reaction beneath the periosteum, where abundant new bone is formed in successive layers. Macroscopically the tumour is lobuloted and often fairly large. It may look Grey (like brain) or red (like red current jelly) if hemorrhage occur into it. Microscopically, sheets of small dark polyhedral cell with no regular arrangement and no ground substance are seen.

Clinical Feature:

1. Pain- Often throbbing is character with rapidly increasing swelling over the shaft of a long bone.

2. Pyrexia, warm, tender swelling which is fusiform in shape and firm in consistency.

Investigations:

1. X-ray a. Onion-peel appearance

b. Sunray appearance and codman’s traingles are also present.

2. CT & MRI - Reveal both osseous & extra osseous component of tumour.

3. Chest X-ray P/A view - Show pulmonary metastesis.

4. Radioisotope scan - Markedly in creased uptake of the isotope in the region of the tumour.

5. FNAC.

Differential Diagnosis

1. Subacute osteomyelitis

2. Syphilitic osteoperiostitis

3. Reticulur cell sarcoma

4. Metastetic neuroblastoma.

Treatment:

The best results are achieved by a combination of all three methods a course of preoperative chemotherapy; then wide excision (or amputation) if the tumour is in a favorable site or radiotherapy followed by local excision if it is less accessible and then a further course of chemotherapy for 1 year.

Multiple Myeloma

Posted by Prajwal Rijal

Multiple myeloma is the commonest of all primary malignant bone tumour arises from the plasma cells of the bone marrow.

Clinical Features:

i) Aged 45-65 years Women > Men.

ii) Weakness

iii) Backache

iv) Bone pain

v) Pathological fracture

vi) Anaemia due to red marrow function

vii) Thrust, polyuria, abdominal pain due to hyper calcaemia

viii) Infection due to decreased antibody

ix) S/S or renal dysfunction, spinal cord or root compression.

Investigations:

1. Blood- decreased Hb%, increased ESR, increased Serum creatinine, increased scrum calcium.

2. Urine- Bencejones protein-present

3. X-ray- i) Generalized ostecoparosis

ii) Vertebral compression fracture

iii) Multiple punched out-defects with soft margin in the skull, pelvis, proximal femur and vartebra.

4. Sternal puncture- show plasma cytosis with typical myeloma cells.

Pathology:

At operation, the affected bone is soft and crumbly. The typical microscopic picture is of sheels of plasmacytes with a large ecentric mucleus containing a spoke like arrangement of chromatin.

Prognosis

The tumour is uniformly fatal, through its progress can often be checked for several years.

Treatment:

i. Pain control

ii. Treatment of pathological fracture

iii. Correction of fluid balance and hypercalcaemia

iv. Perioperative antibiotic

· The tumour foci respond to radiotherapy for while and pain is well relived.

· Chemotherapy- Malphan together with prednisolone can be given. Malphan in conjunction with doxorubicin, curmastine and cyclophosphamide also can be given.

· Fractures are best treated with internal fixation and packing of cavitics with methylmethacrylate cement. Spinal fractures need immedicate stabilization either by effective bracing or by internal fixation.

Osteclastoma

Posted by Prajwal Rijal

· A lesion of uncertain origin.

· Usually benign but may recur after local removal or curettage.

· In about 10% cases if behaves as frankly malignant tumour. Metastasising through the blood stream to the lung.

· Common sites- Epiphyseal region

o The lower end of the femur

o The upper end of the tibia

o The lower end of the radius

o The upper end of the humerus

Those ends of the long bones at which most growth occurs (growing ends)

Pathology

Characteristically it occurs in the end of the bone, occupying the epiphysical regions and often extending almost to the joint surface. It destroy the bone surface, but new bone forms beneath the periosteum, so tumour, giving a faintly loculated appearance (soap bubble appearance). Pathological fracture is common.

The tumour has a reddish fleshy appearance; it comes away in pieces quite easily when curetted, but is difficult to remove completely from surrounding bone. Aggressive lesions have a poorlydefined edge and extend well into surrounding bone.

Histologically, the striking feature is an abundance of multinucleated giant cells scattered on a background of stromal cells (spindle calls) which little or no visible inter cellular tissue.

Clinical Features:

The patient is usually a young adult.

i. Gradually increasing local swelling in the end of a long bone.

ii. Pain the end of a long bone

iii. There may be a history of trauma

iv. Pathological fracture occurs in 10-15% of cases

v. May be tender on firm palpation.

Differential Diagnosis

i) Subacute osteomyelitis

ii) Syphilitic osteo-periostitis

iii) Other bone tumours

iv) Metastatic suprarenal neuroblastoma (Histologically)

Investigations

X-rays

i) A radiolucent area situated eccentrically at the end of along bone and bounded by subchondral bone plate.

ii) The centre has a soap-bubble appearance due to ridding of the surrounding bone.

iii) The cortex is thin and sometimes ballooned; aggressive lesions extend into the soft tissue.

CT & MRI - Will reveal the extent of the tumour, both within the bone and beyond.

Arthoscopy - To establish whether the articular surface has been broached or not.

FNAC or Open biopsy - To confirm the diagnosis.

Treatment

· Well-confined, slow-growing lesions benign histology-Thorough curettage and stripping of the cavity with burrs and gouges, followed by swabbing with hydrogen peroxide or by the application of liquid nitrogen; the cavity is then packed with bone chips.

· More aggressive tumours- Excision followed, if necessary, by bone grafting or prosthetic replacement.

· If the affected bone is one that can resonably be dispensed with- such as the clavicle or fibula- Excision of part of the bone.

· Tumours in awkward sites (e.g. spine) may be difficult to eradicate; supplementary radiotherapy is sometimes recommended but it carries a significant risk of causing malignant transformation.

Osteocarcoma

Posted by Prajwal Rijal

Site : Metaphysis of the lower end of the femur, the upper end of the tibia and humors.

Age incidence

- 10-25 years (prior to epiphyseal fusion)

- Old age (Secondary to paget’s disease)

Sex- Boys > Girls

The tumour extends within the medulla and across the physeal plate and extending into the soft tissue. Grey white invasive and destructive masses having focal haemorrhase & nerosis. There are areas of bone loss and cavitation alternate with dense patches of abnormal new bone.

Clinical Features

Ø Pain, swelling, near a joint with history of trauma

Ø Overlying skin stretched & shiny

Ø Local rise of temp due to increased vascularily

Ø Firm in consistency

Ø Sign & symptoms of bronchitis, pnenmonia if there is lung metastasis

Ø Pathological fracture

Spread- haematogenous spread to lung, usually sometimes to other bone.

Types:

a) Primary or Secondary

b) Dahlin’s prognostic classification

Ø Osteoblastic

Ø Chondroblastic

Ø Fibroblastic

c) Geschickter and copeland classification

Ø Selerosing type

Ø Osteolytic type

Ø Both osteoblastic and osteolytic (Mixed)

Ø Telangicetatic.

X-ray

1. Both osteolytic (Radiolucent) & osteolbastic (Dense) areas

2. Sunray appearance – due to deposition of tumour osteoid along the vessels.

3. Codman’s triangle- due to raised periosteum

CT & MRI: To see extend of the tumour

X-ray Chest: To see pulmonary metastasis.

Biopsy

1. FNAC

2. Open biopsy

Varients of osteosarcoma

Parosteal : A thin gap between cortex & tumour, cortex is not corded.

Periosteal : A superficial defect of the cortex

Paget’s sarcoma : Feature’s of paget’s disease + Bone destruction with soft tissue invasion.

Treatment

· Multi-agent chemotherapy for 8-12 weeks

· Resection of the tumour or amputation

· The pathological specimen is examined to assess the response to preoperative chemotherapy. It tumour necrosis is marked, chemotherapy is continued for another 6-12 months.

· Bone gap is filled by a large bone graft and custom-made implant.

Bone Tumor

Posted by Prajwal Rijal

CLASSIFICATION OF BONE TUMOUR

A. Primary bone tumour

	Origin	Benign	Malignant
1.	Cartilage	Osteochondroma Chondroma Chondreoblastoma Chondromyxoid fibroma	Chondrosarcoma
2.	Bone	Osteoid osteoma Osteoblastoma * Osteochondroma (Commonest benign bone tumour)	* Osteosarcoma (Commonest in adolescent)
3.	Bone marrow	Parosteal osteosarlomia	*Multiple Myeloma (Commonest primary bone tumour in old age) Malignant hymphoma
4.	Fibrous tissue	Fibroma	Fibrosarcoma
5.	Vascular tissue	Aneurysmal bone cyst Haimagioma Angioima	Malignant haemangioma
6.	Unknown origin	Giant cell tumour Fibros histocystoma	Malignant giant cell tumour Ewing’s tumour adamantinoma
7.	Synovium	Synovioma	Synovial sarcoma

B. Secondary bone tumour

Metastatic tumour usually comes from

1. Thyroid

2. Breast

3. Lungs

4. Kidney

5. Prostate

The Principle of management of bone tumour:

1. Diagnosis of bone tumour either benign asymptomatic, benign symptomatic or malignant

2. Treatment of the tumour

3. Follow-up.

Diagnosis depends on:

1. History- Age incidence

Young adult (osteosarcoma)

Children (Ewing sarcoma)

Old age (Multiple myloma, secondary bone tumour)

Slowly growing growth- Benign

Rapidly growing growth- Malignant

History of Trauma (though trauma is not the cause) osteosarcoma

2. Clinical Feature

ü Size & shape regular- Benign; irregular- malignant

ü Margin- well defined- Benign; ill-defined- malignant

ü Over lying skin normal- Benign; fixed- malignant

ü Local temperature normal- Benign; Raised- malignant (Osteosarcoma)

ü Over lying structure free- Benign; fixed- malignant

ü Regional lymphode-not enlarged- Benign; May be enlarged- malignant

ü Usually no pain & tenderness-Benign; Pain & tenderness- malignant

ü No systemic features- Benign; Systemic features- malignant

Investigations:

I. X-ray- small radiolucent nidus- osteoid osteoma

well defined exostosis emerging from the meaphysis- osteochondroma

Sun ray appearance + Codman’s triangle- Osteosarcoma

Onion peel appearance- Ewing sarcoma

Soap Bubble appearance- Giant cell tumour.

1. CT * Show cortical crosion or fracture

* Show intraosseons and extraosseons extension of tumour and the relation ship to surrounding structure.

* Can detect pulmonary metastasis.

2. MRI- The best method for evaluation soft tissue tumour

3. Radionuclide scanning with 99m Tc- HDP

4. Blood for i) Hb%- decreased ii) ESR- raised

5. Unire for Bence- jonces protein (i.e. multiple myeloma)

6. Scrum alkaline phosphatase- increased

7. Scrum acid phosphatase (in case of ca. prostate which cause secondary bone tumour)

8. Biopsy - FNAC

- Open biopsy- tissue is taken from boundary zone.

Histopathological findings will confirm the diagnosis either benign or malignant. If malignant what type of the lesion is.

Treatment:

a) Benign, asymptomatic tumour-treatment in not required; excision or curettage of the lesion can be done.

b) Benign, symptomatic or enlarging tumour- They can generally be removed either by local (marginal) excisionor (in case of benign cysts) by curettage.

c) Suspected malignant tumours- Treatment regions amputation, limb sparing operations and different types of adjuvant therapy.

Methods of Treatment:

1. Tumour excision-

a. Intra-capsular (intralesional) excision and curatase

b. Marginal excision

c. Wide excision

d. Radical resection

e. Limb sparing surgery

f. Amputation – for high grade tumour.

The preferred method for intra-capsular lesion is now wide excision together with pre and postoperative chemotherapy.

The resulting defect is dealt with in one of several ways:

1. Vascularized or nonvascularized bone graft.

2. Custom made implants

3. Large allografts

4. Custom made prosthesis

5. Allograft prosthetic composites

6. Extendible implants

7. Grafting and arthrodesis

8. Distraction osteosynthesis.

2. Multi-agent Chemotherapy:

This is now the preferred neoadjuvant and adjuvant treatment for malignant bone and soft tissue tumours. Modern chemotherapy regimes effectively reduces the size of the primary lesion. Prevent metastatic seeding and improve the chances of survival.

Drugs currently in use are methotrenate doxorubicin (adriamycin), cyclophosphamide, vincristine and cisplatin, Treatment is started 8-12 weeks preoperatively and the effect is assessed by examining the resected tumour. If there is little or no neurosis a different drug is selected for postoperative treatment. Maintenance chemotherapy is continued for another 6-12 months.

3. Radiotherapy:

High energy irradiation has long been used to destroy radio scrsitive tumour or as adjuvant therapy before operation. indications- for

1. Highly sensitive tumours (such as Ewing sarcoma)

2. Tumours in inaccessible sites

3. Metastatic deposit and marrow cell tumour (i.e. myeloma & malignant lymphoma).

OSTEOCHONDROMA

(Cartilage capped exostosis)

Mushroom like or cauliflower like tumour with degeneration and calcification in the canter of the cartilage cap. – osteochondroma.

Ø Origin- from epiphyscal cartilage.

Ø It is the commonest benign fumour of bone.

Ø The stalk and part of base of the tumour are of bone, but it is capped by cartilage.

Ø Tumour appears to migrate along the shaft towards its centre as the bone grows in length.

Ø Theory of histogenesis- ‘perichondrium’ perverted activity of periousteum which form cartilage & bone or accumulation of embryonic connective tissue at the points of tendinous insertion.

Clinical Features:

Ø Circumscribed hard swelling near a joint of a (knee, Ankle, hip, shoulder, elbow) teenager or young adult.

Ø Pain and tenderness suggests bursitis, malignant change, fracture of bony stalk, pressure on nerve etc.

Ø Any further enlargement often the end of the growth suggestive of malignant transformation.

Differential Diagnosis

1. Hereditary multiple exostosis

2. Ivory exostosis (compact osteoma)

Investigations

X-ray:

Ø Well defined exostosis emerging from the metaphysic.

Ø It looks smaller than it fells because the cartilage cap is not seen on X-ray.

Indication of Operation:

i. Joint interference- large osteochondroma obstruct joint movement

ii. Painful bursitis

iii. Of bony stalk due to traum

iv. Malignent change

v. Pressure on the neighboring vessels & nerves give rise to neurovascular complications.

Operation- Excision of the tumour along with its periosteal cover to prevent recurrence of the tumour.

Sudden increase in size & pain suggests malignancy.

Ewings Sarcoma

Multiple Myeloma

Osteclastoma

Osteocarcoma

Bone Tumor

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