Bone tumors • Rare tumors (< 2%) • More in young ages Outline • • • • Introduction (signs, symptoms, investigation) Benign tumor Malignant tumor Metastatic bone tumor Classification • Origin – Primary – Secondary (95%): breast, lung, prostate, kidney and thyroid • cell type: – Bone Osteoma, osteosarcoma – Cartilage Chondroma, Chondrosarcoma – Marrow Hemangioma, angiosarcoma – Fibrous tissueFibroma, fibrosarcoma • Tumor type: – Benign: Osteoma, osteochondroma – Malignant:: Osteosarcoma, chondrosarcoma Symptoms and Signs • • • • • • • • Asymptomatic and discovered accidentally. General: fatigue, fever, wt. loss Mass can be felt at the tumor site. Pain: – May worsen at night and awakes patient, caused by – Tumor compression on surrounding tissue – Hemorrhage in the tumor – Pathological fractures Swelling Local tenderness Warmth Pathological fracture: may be the first sign. Malignant vs. Benign Tumors • Rapid growth • Warmth • Tenderness • Ill defined edges All are suggestive of malignancy. Investigations • • • • History and examination Imaging (x-ray, CT, MRI & radionuclide scanning) Biopsy Labs • Ca & P: Greater than normal levels may indicate bone metastasis. • PTH: Lower than normal levels may indicate bone metastasis. • ALP isoenzyme: Higher than normal ALP levels may indicate Paget's disease, osteoblastic bone cancers, osteomalacia and rickets. • LDH: High values indicate poor prognosis Plain x-ray • Most useful • Could see: • Lump • Cysts • Bone destruction • Cortical thickening & periosteal reaction • Important to notice: • Where • How many • Cystic or not • Margins • Destruction Periosteal reaction • Periosteal hypertrophy which develops in response to periosteal irritation. They are a non-specific sign. • They have many causes: - Infections - Tumors (both benign & malignant) - Benign tumors are thick & smooth or completely absent - Malignant tumors are thinner & irregular (wavy). - Healing fractures - Chronic stress Periosteal reactions seen in the distal tibia and fibula. Periosteal reaction at the distal radius with irregular edges: malignant. Osteosarcoma of the distal femur: Codman’s triangle can be seen: periosteum is being “lifted off”. Midshaft periosteal reaction with smooth + thick edges: this is a benign osteoma. • Periosteal reaction of tibia healing from a fracture showing a smooth and thick. • CT and MRI • Asses the extent of the tumor • Relation to surrounding structures • Radionuclide scanning: • Helpful in revealing site of a small tumor • Skip lesions • Silent secondary deposits Multiple hot spots seen: lung cancer which has metastasized to vertebrae. Biopsy • Allows us to reach a diagnosis. • Allows us to plan for treatment. Methods: • Open biopsy: surgical procedure done under GA. • Zonal biopsy: open biopsy from transition zone. • Excisional biopsy: excision of the entire tumor. • Large–bore needle biopsy: aspirating cells. Complications: 1. Hemorrhage 2. Wound break down 3. Infection 4. Pathological fractures Benign tumors Benign bone tumors • • • • • • Non ossifying fibroma Osteochondroma (the Commonest) Osteoid osteoma Enchondroma Giant cell tumor of bone Osteoblastoma • • • • Most bone tumors are benign Most often are asymptomatic Unlikely to spread Usually found in the biggest bones (femur, tibia & humerus) • Some types are more common in specific places (near the growth plates) • Appearance and location of mass on radiographs are keys to diagnosis. Osteochondroma (exostosis) • Commonest benign tumor of bone • More in male <25y. • Solitary • Mature bone with cartilaginous cap. • Common sites are the fast growing sites of long bones (lower end of femur and upper end of tibia), crest of ileum & shoulder. • Flattened (sessile) or stalk-like (exostosis) • Most likely caused by Congenital defect or trauma of the perichondrium, which results in the herniation of a fragment of the epiphyseal growth plate through the periosteal bone cuff. • Osteochondromas Small risk of malignant transformation(<1%) Symptoms: • A hard, immobile, detectable mass that is painless • Lower-than-normal-height for age • Muscle soreness • One leg or arm may be longer than other • Pressure or irritation with exercise • X-Ray: • Exostosis: well defined bony projection • Calcified cartilage in large lesions Treatment: excision if becoming bigger and more painful. Well defined bony projection • Solitary osteochondroma. • Lateral radiograph of a sessile osteochondroma of the distal femur Non-ossifying fibroma • Fibrous tissue within bone ossiffy in time • Asymptomatic, incidental findings • Metaphysis of long bones • Occasionally multiple lesion • Developmental defect, seen in children • As bone grows, defect becomes less obvious • May enlarge and cause pathological fracture • No treatment, unless there is pathological fracture • • Well marginated radiolucent lesion Distinct multilocular appearance Osteoid osteoma • • • • • • • Neoplastic proliferation of osteoid and fibrous tissue More in male < 30 year more in vertebra or long bones Less in mandible or other craniofacial bones. Small in size (<1.5 cm) Oval or rounded shape Pain (severe) worse at night ,not relieved by rest but relieved by aspirin. • Types: Cortical, cancellous, subperiosteal. • Treatment: Complete removal after careful localization by CT. X-Ray • Nidus: tiny radiolucent area • Diaphysis surrounded by dense bone and thickend cortex • Metaphysis less cortical thickening • • Male 23 years old History of increasing pain in the knee & relieved by aspirin. • Plain radiograph in a 25-year-old male with cortical osteoid osteoma. shows a radiolucent nidus surrounded by fusiform cortical thickening a 25-year-old male with cortical osteoid osteoma. • Radionuclide bone scan shows focal intense activity at tumor site. • 6y old child who presented with left hip pain. • Well-defined area of sclerosis surrounded by a ring of radiolucency in the left femoral neck. • Note the absence of periosteal reaction that suggests intramedullary or cancellous osteoid osteoma. • Transaxial CT scan through the proximal shaft of the right femur in a 17-year-old boy. • Osteoid osteoma adjacent to the endosteal margin of the cortex. • Central sclerotic focus in the radiolucent nidus Osteoid osteoma of transverse process of vertebra Malignant tumors • Primary • Secondary (metastasis) • • • • Uncommon cancer Most commonly affects the long bones 20 % of pediatric bone tumors are malignant. 66% of adult bone tumors are malignant, most commonly mets. • The most common type of bone cancer in adults is metastatic cancer from other organs Primary bone cancer Risk factors: 1. Radiotherapy & chemotherapy 2. Paget's disease 3. Family Hx (hereditary retinoblastoma) Signs & symptoms • Fever, Night sweats, Fatigue & Unintended weight loss • Bone pain that often is nocturnal • Swelling & tenderness near the affected area • Pathological fractures Osteosarcoma • • • • Most common primary bone malignancy Incidence: 2.8 per million M >F Age 10-25 years (the 8th most common childhood cancer) Prognosis • Aggressive tumor • Metastasis to the lung • 5-year survival – Without mets is 70% – With mets is 25% Where Mainly affects metaphysis of long bones More in: • Knee – Distal femur – Upper tibia • Humerus (prox.end) • Maxilla Clinical features • Pain: – – – – • • • • • Dull aching Progressive Constant Worse at night Swelling Redness Hotness Tenderness Pathological fracture DDX • • • • • • • • • Stress fracture Ewing's sarcoma Osteomyelitis Osteochondroma Osteoblastoma Bone cysts Chondroblastoma Chondrosarcoma Giant cell tumor Diagnosis: • Radiological studies: 1. X-Ray 2. CT-scan 3. Bone scan & MRI • Bone biopsy, the only definitive method to determine whether a tumor is malignant or benign. Treatment: • Surgical resection • Preoperative & postoperative chemotherapy X-ray findings 1. 2. 3. 4. Lesion Cortical destruction Extension to the marrow or soft tissue Codman’s triangle a term used to describe the triangular area of new subperiosteal bone that is created when a lesion, often a tumour, raises the periosteum away from the bone. 5. Sunburst Effect Osteosarcomas can be • Predominantly osteolytic • Predominantly osteoblastic • Mixture Sunburst Appearance • • • Clinical appearance of a teenager who presented with osteosarcoma of the proximal humerus Swelling throughout the deltoid region Disuse atrophy of the pectoral muscule • White arrow: codman triangle • Black arrow: soft tissue mass Patholigical fracture Ewing sarcoma • • • • • • • A malignant round-cell tumor. Rare disease (incidence 0.6 per million 2nd most common bone malignancy in pediatrics. M>F Age 10-20 years Usually the lesions are diaphyseal Mets (30%), most commonly in the lungs & other bones & less commonly in the bone marrow. Most common areas: • Pelvis • Femur • Humerus • Ribs • Clavicle Clinical feature: • Pyrexia • Pain: – Constant – Increase with movement • Limping • Swelling, warm, tender & red Radiological studies: 1. X-Ray 1. Lytic medullary lesion 2. Onion skin appearance 2. CT-scan 3. Bone scan & MRI • • • White arrow: onion skin apperance Red circle: sunburst periosteal reaction Blue circle: osteolytic lesion • • Periosteal reaction Osteolytic lesion • • Periosteal reaction Osteolytic lesion • • Periosteal reaction Osteolytic lesion Treatment: 1. Local radiotherapy combined with systemic chemotherapy 2. In young children amputation may be necessary due to severe compromise of bone growth. Prognosis, 5-year survival • 50% with the 1st approach • 75% with the 2nd approach Metastatic bone tumor • Most common malignant lesion of the bone. • The most common sites of spread of cancers are lung, liver & skeleton. • Carcinomas are much more likely to metastasize to bone than sarcomas • Typically multifocal BUT renal and thyroid carcinomas produce only a solitary lesion. • Common sites for metastasis: – Vertebrae, pelvis, proximal parts of the femur, ribs, proximal part of the humerus, and the skull. – Batson venous plexus >> more mets to the axial skeleton – Hands & feet are rare – 50% of them originate from lung neoplasms. • Mets: 1. Direct extension 2. Retrograde venous flow 3. Seeding with tumor emboli via the blood circulation Destructive expanded osteolytic lesion in the metacarpal of the thumb in a 55-year-old man with lung carcinoma. Mets (adults) • Osteoblastic behaviour Prostate Stomach Bladder Breast Osteolytic behaviour Lung Kidney Colon Thyroid Breast Typical x-ray appearance of osteolytic bone metastases. This plain pelvic xray film of a 75-year-old patient with breast carcinoma shows multiple osteolytic bone lesions. =>decrease in bone density. Typical x-ray appearance of osteoblastic bone metastases. This plain pelvic x-ray film of a patient with prostate cancer shows multiple osteoblastic metastases to the pelvis and lumbar (L4) and sacral (S1) vertebral bodies.=>increase in bone density Mets (kids) • • • • • Neuroblastoma Wilm’s tumor Osteosarcoma Ewing’s sarcoma Rhabdomyosarcoma Presentation • • • • • Pain which results in reduced mobility. Bone weakness which predispose to pathologic fractures. Palpable masses (large bony lesions). Neurologic impairment due to spinal epidural compression. Anemia (decreased red blood cell production) is a common blood abnormality in these patients. • Past history of primary malignant tumor symptoms, BUT others did not complain of anything before. • A radiograph of a destructive pathological fracture on the left hip, in a man with metastatic renal cell cancer. • The patient underwent replacement of the upper femur due to extensive destruction of the bone around the hip. Approach • History & physical examination • Radiological studies – – – – Plain X-ray MRI CT scan Bone scan (Technetium-99m) • Laboratory studies • Biopsy Radiological studies • X-ray: destruction of bone and/or lucent Lesions of Bone • Bone scan: most cost-effective and available whole-body screening test for the assessment of bone metastases. • CT – Useful in evaluating suspicious bone scintiscan findings – Useful in guiding needle biopsy, particularly in vertebral lesions. • MRI – Useful in evaluating suspicious bone scintiscan findings – Help in detecting metastatic lesions before changes in bone metabolism – Helpful in determining the extent of local disease in planning surgery or radiation therapy. Hot spots: Increased osteoclastic activity X-ray • • • RadioIsotope Pt. presented with pain in the right upper thigh X-ray showing METS in upper 1/3 of the femur Radioisotope scan revealed many deposits in other parts of the skeleton. Treatment Divided into: 1. Systemic therapy • • • Chemotherapy Hormone therapy Immunotherapy 2. Local therapy • • Radiation therapy Surgery Treatment • Radiation therapy combined with chemotherapeutic or hormonal agents, is the most common treatment modality. • Early use of radiation and bisphosphonates (zoledronic acid, pamidronate) slows bone destruction. • Some tumors are more likely to heal after radiation therapy: – Blastic lesions of prostate and breast – Lytic destructive lesions of lung and renal cell • Surgery is indicated in fractures or large metastatic mass. • A woman with advanced metastatic breast cancer to bone • Pain in both her right and left hips • A special partial hip replacement was necessary on the right because the hip joint was involved. • On the left, a special nail could be used to strengthen the femur bone below the hip. Thank You Good Luck