Malignant Tumors Rahaf Jreisat

Approach • History

-Age: most primary lesions appear in childhood and adolescence while lesions in elderly are usually metastatic -Pain: indicates nature and site of tumor -Swelling: Size indicates tumor progression

-Other symptoms: Fever, weight loss, night sweats Neurological symptoms (compression)

• Examination -Lump: SPACESPIT -Lymph nodes -Joint effusion -Spine

• Imaging: Plain X-Ray is the gold standard diagnostic tool CT: Staging and Mets identification MRI Bone Scan: skip lesions

• Lab Tests: CBC, ESR Alkaline Phosphatase Bence Jones Proteins in urine

• Biopsy:

All imaging studies should be completed before undertaking a biopsy, which may itself distort the appearances. Tumor biopsy should never be regarded as a ‘minor’ procedure. Complications include haemorrhage, wound breakdown, infection and pathological fracture.

Primary malignant bone tumours

• Multiple Myeloma • Osteosarcoma

-Paget Sarcoma • Chondrosacoma • Ewing Sarcoma

■ The most common malignant lesions in bone are metastatic tumors, while the most common primary malignant lesion is Multiple Myeloma.

Osteosarcoma

• a highly malignant tumour arising within the bone and spreading rapidly outwards to the periosteum and surrounding soft tissues.

• The histological appearances show considerable variation.

Clinical features • occurs predominantly in children and adolescents. • It may affect any bone but most commonly the

long-bone metaphyses, especially around the knee and at the proximal end of the humerus.

• Pain is usually the first symptom; it is constant, worse at night and gradually increases in severity.

• sometimes the patient presents with a lump. • The erythrocyte sedimentation rate (ESR) is

usually raised and there may be an increase in serum alkaline phosphatase.

Imaging -On x-ray: mixed lytic and blastic, the

tumour margins are poorly defined. -Often the cortex is breached and the

tumour extends into the adjacent tissues; when this happens, streaks of new bone appear, radiating outwards from the cortex – the so-called ‘sunburst’ effect. -Where the tumor emerges from the cortex, reactive new bone forms in the angle between periosteum and cortex (Codman’s triangle).

About 10% of patients have pulmonary metastases by the time they are first seen which is detected by CT

Treatment

• Multi-agent chemotherapy is given for 8–12 weeks and then, provided that the tumor is resectable and there are no skip lesions, a wide resection is carried out.

• It is important to eradicate the primary lesion completely; the mortality rate after local recurrence is far worse than following effective ablation at the first encounter.

Paget’s sarcoma

• Paget’s disease of bone occasionally undergoes malignant transformation; most osteosarcomas appearing after the age of 50 years fall into this category.

• This tumour is more malignant than classic osteosarcoma

• Most patients have pulmonary metastases by the time the tumour is diagnosed.

• Even with radical resection or amputation and chemotherapy the 5-year survival rate is low. If the lesion is definitely extra compartmental, palliative treatment by radiotherapy may be preferable; chemotherapy is usually difficult because of the patient’s age and uncertainty about renal and cardiac function.

Chondrosarcoma

• Chondrosarcoma occurs either as a primary tumour or as a secondary change in a pre-existing benign chondroma or osteochondroma.

• Cartilage capped exostoses of the pelvis and scapula seem to be more susceptible than others to malignant change, but perhaps this is simply because at these sites the tumour can grow without being detected and removed at an early stage.

Clinical features • highest incidence in the fourth and fifth

decades, men are more affected.

• Patients may complain of a dull ache or a gradually enlarging lump.

Imaging • Primary chondrosarcoma can occur in any

bone that develops in cartilage but is usually seen in the metaphysis of long bones. X-ray examination shows a radiolucent area with central flecks of calcification.

• Secondary chondrosarcoma usually arises in the cartilage cap of an osteochondroma that has been present since childhood.

Treatment • Chondrosarcomas are usually slow-growing and

metastasize late. • They present the ideal case for wide excision

and prosthetic replacement, provided it is certain that the lesion can be completely removed without exposing the tumour and without causing an unacceptable loss of function. Otherwise amputation may be a safer option.

• The tumour does not respond to either radiotherapy or chemotherapy

Ewing’s sarcoma • Ewing’s sarcoma is believed to arise from

endothelial cells in the bone marrow. • Translocation 11/22 • It occurs most commonly between the ages of

10 and 20 years, more in males

11 + 22 = 33

Patrick Ewing

Clinical features • The patient presents with pain – often

throbbing in character – and swelling. • Generalized illness and pyrexia, together with

a warm, tender swelling and a raised ESR

imaging • X-ray usually shows an

area of bone destruction which, predominantly in the mid diaphysis.

• New bone formation may extend along the shaft and sometimes it appears as fusiform layers of bone around the lesion – the so-called ‘onion-peel’ effect.

• Treatment The prognosis is always poor and surgery alone does little to improve it. Chemotherapy offers a 5-year survival rate of about 50%.

• The best results are achieved by a combination of all three methods: a course of preoperative neoadjuvant chemotherapy; then wide excision if the tumour is in a favourable site, and then a further course of chemotherapy for 1 year.

Metastatic bone disease • The commonest source is carcinoma of the breast;

next in frequency are carcinomas of the prostate, kidney, lung, thyroid, bladder and gastrointestinal tract.

• In about 10% of cases no primary tumour is found. The commonest sites for bone metastases are the vertebrae, pelvis, the proximal half of the femur and the humerus.

• Metastases are usually osteolytic, and pathological fractures are common. Osteoblastic lesions are uncommon; they usually occur in prostatic carcinoma.

Routes of mets

• Hematogenous: mc route • Lymphatic: rare • Direct implantation like surgical procedures

and biopsy • CSF: spread of malignant lesions from

meninges to vertebrae

Clinical features • The patient is usually aged 50–70 years; with

any destructive bone lesion in this age group, the differential diagnosis must include metastasis.

• Pain is the commonest – and often the only – clinical feature.

• Symptoms of hypercalcaemia may occur. These include anorexia, nausea, thirst, polyuria, abdominal pain, general weakness and depression.

Imaging

• If x-rays do not show anything, a radionuclide scan might. Some deposits remain clinically silent and are discovered incidentally on x-ray, or after a pathological fracture.

• Sudden collapse of a vertebral body or a fracture of the midshaft of a long bone in an elderly person are ominous signs; if there is no history and no clinical clue pointing to a primary carcinoma, a biopsy of the fracture area is essential.

Special investigations

• The ESR may be increased and the haemoglobin concentration is usually low.

• The serum alkaline phosphatase concentration is often increased, and in prostatic carcinoma the acid phosphatase also is elevated

Treatment

• By the time a patient has developed secondary deposits, the prognosis for survival is almost hopeless.

• In the great majority of cases, and certainly in those with multiple secondaries, treatment is entirely symptomatic.

• Analgesics, the more powerful narcotics should be reserved for the terminally ill. Unless specifically contraindicated, radiotherapy is used both to control pain and to reduce metastatic growth.

• Secondary deposits from breast or prostate can often be controlled by hormone therapy.

• Hypercalcaemia should be treated by ensuring adequate hydration, reducing the calcium intake and, if necessary, administering bisphosphonates.

• Treatment of shaft fractures should almost always be treated by internal fixation.

• Spinal stabilization for vertebral fractures. If the spine is stable, a well-fitting brace may be sufficient. However, spinal instability may cause severe pain, making it almost impossible for the patient to sit or stand – with or without a brace. For these patients, operative stabilization is indicated

MM Osteosarcoma

Chondrosarcoma

Ewing sarcoma

Secondary tumors

Age 6th decade 2nd and 4th decade

4th decade 10-20 years

Over 50 years

Symptoms

Pain and swelling

Pain and swelling

Pain, swelling, systemic symptoms

XRay Multiple punched out lesions purely lytic

Sunburst and Codman’s triangle Lytic and blastic

calcificatons Onion-skin periosteal reaction

Mostly lytic lesions

Tx Hematological tx Surgery for fracture and spinal compression

Neoadjuvant chemotherapy and surgery

Surgery Not sensitive to chemo or radiation

Neoadjuvant chemotherapy and surgery

Surgery is palliative