Bone neoplasias Bone tumours General principles of tumours

Bone neoplasias

Bone tumours • General principles of tumours HISTORY : • Pain , mass , disability • Anorexia , weight loss and fever • Onset : – Benign : insidious – Malignant : acute

Clinical examination General examination : – Anaemia – Cachexia (Physical wasting with loss of weight and muscle mass caused by disease ) – Lymphadenopathy Local examination : – Extent , plane of the tumour , presence of pathological # Joint examination : Neurological examination : Assessment : – Arterial and / or venous circulation

Investigations Routine examination : – – Hb % - decreased TC and DC – increased or decreased ESR – increased Serum calcium and phosphorous – increased Special investigation : – – – – Chest X – ray ( secondaries ) CT scan ( metastasis ) Arteriography ( spread of tumour ) USG ( limited role ) MRI ( bone and soft tissue ) Bone scan ( bone metastasis ) Biopsy (diagnosing bone tumour )

Surgical treatment Surgical techniques : Curettage (removal of tissue or growths from a body cavity ) Resection or excision : Types : – – – Debulking or intralesional excision (within the lesion) Marginal margins ( pseudocapsule ) Wide margin ( surrounding normal tissues ) Radical resection (all normal tissues ) Radical amputation ( at high level ) Adjunctive therapy : – Radiotherapy – chemotherapy

Nomenclature and classification A. Bone – forming tumours : Benign : Osteoblastoma , Osteoid osteoma Intermediate : Aggressive osteoblastoma Malignant : Osteosarcoma B. Cartilage – forming tumours : Benign : – Osteochondroma – Chondroblastoma Malignant : chondrosarcoma

Cont. . C. Gaint cell tumours (GCT ) – Benign GCT – Intermediate GCT – Malignant GCT D. Marrow tumours – – – Malignant : Ewing`s sarcoma Plasma cell tumour Multiple myeloma Lymphoma E. Vascular tumours : – Benign : Haemangioma , Glomangioma – Malignant : Angiosarcoma

Cont … Others : Benign : - Neurilemmoma , - Neurofibroma Malignant : - Liposarcoma Tumour – like lesions : – Bone cyst – Fibrous dysplasia – Eosinophilic granuloma

Enchondroma ( chondroma , chondromyxoma ) • A benign tumour consisting of a lobulated mass of cartilage encapsulated by fibrous tissues , occurring in the metaphysis of cylindric bones • Especially of the hands and feet. • May arise in any bone which develops from enchondral ossification. • Common age : - 10 – 50 years. • Site : metaphysis

Clinical features- Usually asymptomatic. - Diagnosis is incidental. - Becomes symptomatic when there is malignant transformation. • Signs of malignant transformation. - pain, - increase in size of bone, - change of skin, - breakage of cortex.

• X-ray- centrally situated radiolucent shadow at the junction of diaphysis and metaphysis with some fleeks of calcification. Treatment- Leave as such if asymptomatic. - If symptomatic- curretage and bone grafting required.

Simple bone cyst. (unilateral – solitary bone cyst). - common in teenagers. - commonest site - upper end of humerus, may be present in metaphyseal region of long bones. - no malignant transformation.

Clinical features. - Usually asymptomatic. - Incidental diagnosis. - Spontaneous healing X – ray - centrally located radiolucent shadow at the metaphysis extending up to growth plate with well defined margin. Treatment- leave as such if asymptomatic. -intra lesional injection of steroid. -if no improvement – curettage + bone grafting.

Giant cell tumor. • Osteoclastoma (malignant tumor) • Common age – 20 – 35 yrs. Common sites : - – lower end of femur, – upper end of tibia, – lower end of radius, – upper end of humerus (elbow).

Clinical features- Located at the epiphysis - Pain , - Swelling, - stretching of skin , - Pathological fracture.

Fig : GCT - solitary - expansile ( tending to expand ) - eccentric shadow with thin subcortical bone and thin cortex. - Lytic lesion, - soap bubble appearance. X ray - common in one ends of bone - no calcification within tumor.

• Treatment – – A. Excision – B. Excision with reconstruction – C. Curettage – D. Arthrodesis – E. Amputation – F. Radiotherapy

Ewing's sarcoma • A highly malignant tumor, usually arising as a central tumor in long bone. • Common age- 10 - 20 yrs. Common sites : – • long bones ( two – thirds cases ) – diaphysis of tibia, fibula, femur , • One third in flat bones : pelvis , clavicle.

• Clinical Findings. • Most common symptoms are localized pain and swelling • Additional symptoms may include – Fever – Weight loss – Anemia – Leukocytosis – Elevated erythrocyte sedimentation rate

Investigation. Xray- Premature type of lesion situated at diaphysis with bony destruction + new bone formation in layers – onion peel apperance. - ESR (increased). - MRI, - CT – for staging.

Ewing sarcoma of femur. Frontal radiograph and lateral radiographs of the femur demonstrate osteolytic lesion (blue circle) with poorly marginated edges in the diaphysis of the bone. There is sunburst periosteal reaction (red circle) and lamellated periosteal reaction (white arrows).

Treatment- - It is radiosensitive tumor. - radiotherapy (8 -12 wks) : control of local tumor - chemotherapy (upto 7 yrs) : control of metastasis

Osteosarcoma (Osteogenic sarcoma) • Second most common and a highly malignant tumor. • Common age- 2 nd decade. • Common site – metaphysis around knee , proximal humerus.

Clinical features. - H / o trauma is a common feature. Pain (specially at night) is the first symptom. Swelling at metaphyseal region. Skin over the tumor is strecthed , shiny and mobile. - Local temperature is increased. - Dilated veins are present. - Patient is usually anaemic.

Investigation- Increased ESR. - Increased alkaline phosphatase ( osteoblastic activity). - Xray – features of destruction + formation of bone. - Sun ray appearance. - Codeman’s triangle ( bone formation parallel to the bone and triangular in shape ). - Biopsy. - MRI/ CT for staging.

Treatment. - Chemotherapy (8 -12 wks). - wide radical excision/ amputation with prosthesis. - If the results are satisfactory- continue the same chemotherapagic agent, otherwise change. Codeman`s triangle

Chondorsarcoma. • Malignant tumor from cartilage. • Slow growing but is - aggressive tumor. • Common age- 40 - 50 yrs. • Types- primary. - secondary.

Primary chondrosarcoma. Common site- metaphysis of tubular bone. Clinical features- usually asymptomatic, pain might be present but is uncommon. Xray- lytic lesion at the physis with flecks(spot) of calcification.

Secondary chondrosarcoma. • Common site- pelvis and shoulder. • Clinical features- usually asymptomatic. • Xray- exotosis with surrounding area of patchy calcification. • Biopsy. • CT, MRI.

Treatment • Radio/ chemo therapy not effective. • Surgery- wide resection + prosthetic replacement

Multiple Myeloma (Plasmacytoma) lasmacytoma Normal Cells • Plasma cells and other white blood cells are part of the immune system, which helps protect the body from infection and disease. • All white blood cells begin their development in the bone marrow. • Certain white blood cells leave the bone marrow and mature in other parts of the body. • Some of these develop into plasma cells when the immune system needs them to fight substances that cause infection and disease.

Cont. .

Cont. . • Plasma cells produce antibodies, proteins that move through the bloodstream to help the body get rid of harmful substances. • Each type of plasma cell responds to only one specific substance by making a large amount of one kind of antibody. • These antibodies find act against that one substance.

Myeloma Cells • When cancer (uncontrolled growth ) involves plasma cells, the body keeps producing more and more of these cells. • The unneeded plasma cells--all abnormal and all exactly alike--are called myeloma cells.

Cont. . • Myeloma cells tend to collect in the bone marrow • Sometimes they collect in only one bone and form a single mass, or tumor, called a plasmacytoma. • In most cases, however, the myeloma cells collect in many bones, often forming many tumors and causing other problems. When this happens, the disease is called multiple myeloma

Cont. . • As myeloma cells increase in number, they damage and weaken bones, causing pain and sometimes fractures. • When bones are damaged, calcium is released into the blood. Hypercalcemia— – cause loss of appetite, nausea, thirst, fatigue, muscle weakness, restlessness, and confusion. • Myeloma cells prevent the bone marrow from forming normal plasma cells and other white blood cells that are important to the immune system. Patients may not be able to fight infection and disease. • prevent the growth of new red blood cells, causing anemia.

symptoms • In the earliest stage of the disease, there may be no symptoms. • When symptoms do occur, patients commonly have bone pain, often in the back or ribs. • Patients also may have broken bones, weakness, fatigue, weight loss, or repeated infections. • When the disease is advanced : - symptoms may include nausea, vomiting, constipation, problems with urination, and weakness or numbness in the legs. - These are not sure signs of multiple myeloma; they can be symptoms of other types of medical problems.

X-ray findings • Osteoporosis is most common skeletal abnormality in this disease • Lesions are usually multiple and found in vertebrae, ribs, skull, pelvis, and femur • Over 50% of solitary lesions are found in vertebrae • Mandible involved in 1/3 of patients with diffuse involvement • Widespread lucencies in bone – Discrete, “punched-out” lesions – Uniform in size

Treatment • Tumor is radiosensitive Chemotherapy Surgery : – Laminectomy ( if tumor compress the spinal nerve ) – Intramedullary fixation ( pathological #) Prognosis : – Death occurs within 3 years in majority of cases and in all by five years

Complications – Pathological # of the ribs – Spinal cord or nerve root compression – Anaemia , leucopenia , thrombocytopenia – Renal failure