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BONE TUMORS
What are bone tumors ?
Abnormal and uncontrollable neoplastic growth of the bones that can form a mass or lump of tissue is known as bone tumor. Bone tumors are classified into: Primary bone tumors Secondary bone tumors ( Metastasis) Mostare classified according to the normal cell of origin and apparent pattern of differentiation BONE TUMOR CLASSIFICATION Bone-forming tumors Cartilage-forming tumors Miscellaneous tumors BONE FORMING TUMORS COMMON LOCATIONS AGE (yr)
BENIGN
OSTEOMA Facial bones, skull. 40-50
OSTEOID OSTEOMA 10-20
Metaphysis of femur & tibia.
OSTEOBLASTOMA 10-20 Vertebral column
MALIGNANT
PRIMARY OSTEOSARCOMA Metaphysis of distal femur, 10-20
proximal tibia & humerus. SECONDARY OSTEOSARCOMA >40 Femur , humerus ,pelvis CARTILAGINOUS TUMORS COMMON LOCATION AGE (yr)
BENIGN
OSTEOCHONDROMA Metaphysis of long tubular 10-30
bones. CHONDROMA 30-50 Small bones of hands & feet. MALIGNANT
CHONDROSARCOMA Bones of shoulder , pelvis, 40-60
proximal femur , ribs . MISCELLANEOUS
GIANT CELL TUMOR Epiphysis of long bones. 20-40
(USUALLY BENIGN)
EWING TUMOR 10-20
(MALIGNANT ) Diaphysis & metaphysis. BENIGN TUMORS OSTEOMA Osteomas are benign lesions of bone. Most commonly present in the head , neck and paranasal sinuses. Most commonly occurs in middle age OSTEOID OSTEOMA arise during teenage years & 20s. More common in males than females( 2:1) arise most often in the proximal femur and tibia less than 2cm Imaging: sclerotic thickening of the shaft Osteoblastoma: bone-forming lesion that may be found within the cortex, medullary canal, or periosteal tissues arise most often in vertebral column larger than 2.0 cm in diameter TREATMENT Chemotherapy. Limb salvage therapy ( long term survival in 60-70%) . CHONDROMA Benign tumors of hyaline cartilage. When they arise within the bone, termed as enchondromas. When they grow outwards from a bone, termed as periosteal chondromas or ecchondroma. Two syndromes are associated with chondromas; Ollier disease (dyscondroplasia): characterized by multiple chondromas preferentially involving one side of the body. Arise in the region of the growing epiphysial cartilages, interfere with normal growth and may lead to shortening or deformity Maffucci syndrome is characterized by multiple chondromas associated with benign soft tissue angiomas. CLINICAL FEATURES Pathological fractures. Most common at spine (sacral region). Periosteal chondroma – arise in hands or feet , or from flat bones such as scapula or ilium Painless lesions. Progressive disorder. Swelling of jaws. RADIOLOGICAL FINDINGS O-ring sign Mineralization Calcification OSTEOCHONDROMA Also known as osseocartilaginous exostosis Usually present in 10 – 20 age group Tumor originates from the growing epiphysial cartilage plate Diaphysial Aclasis (multiple exostosis) – involvement of several bones Clinical Features: Hard swelling near a joint Usually painless May be painful if the pressure affects adjacent nerves or vascular structures Imaging: Shows mushroom like stalk of the bony tumor Treatment: Tumor should be excised if it causes pain or if it enlarges after puberty GIANT CELL TUMOR OF BONE Also known as Osteoclastoma type of benign tumor that grows at the ends of the body's long bones Tumors develop at the lower end of the femur and upper end of the tibia , lower end of radius, and the upper end of humerus May also occur in spine and sacrum Occurs in young adults in the 20-40 age group Common in females. CLINICAL FEATURES Pathological fractures Bone pain — pain tends to increase when active and improve when the area is rested. Swelling A lump — with or without pain. Tenderness. Decreased mobility in the joint near to the tumor. Build-up of fluid in a joint near to the tumor. DIAGNOSTIC PROCEDURES Xrays. CT-scan MRI Bone scan. Blood test. TREATMENT Radiation therapy Embolization, minimally invasive surgical technique. The purpose is to prevent blood flow to an area of the body, which can effectively shrink a tumor or block an aneurysm. Amputation. Wide resection. Intralesional surgery. MALIGNANT TUMORS CHONDROSARCOMA Tumor of cartilage Second most common type of bone cancer (30-60 age group) develop in the interior of the bone (central chondrosarcoma) – commonly in the femur, tibia or humerus upon its surface (peripheral chondrosarcoma) – affect a flat bone such as innominate bone, sacrum or scapula Rarely involves distal extremities. Metastasize to lungs and skeleton. CLINICAL FEATURES Pain Localized swelling Pathological fracture TREATMENT Surgical excision. Chemotherapy. OSTEOSARCOMA Osteosarcoma is a bone producing malignant tumor. Occurs most commonly in 10-25 age group Accounts for 20% of primary bone cancers. Often occurs as a complication of paget’s disease Tumors arise in the metaphyseal region of long bones of the extremities Common sites are: Lower end of femur, upper end of tibia, and the upper end of humerus CLINICAL FEATURES Pathologicalfractures Local bone pain with gradually increased swelling Thickness near the end of bone Overlying skin is warmer than normal Imaging: triangular shadow on x-ray between the cortex and raised periosteum (Codman triangle) . 10-20% patients have pulmonary metastasis. Treatment: Chemotherapy Drugs currently used include: Methotrexate, doxorubicin, cisplatin, ifosfamide EWING SARCOMA Also known as endothelial sarcoma of bone This is the most common cancer among children, it is rare in adults. Ewing’s sarcoma is a very rare type of cancerous tumor that grows in bones or the soft tissue around bones, such as cartilage or the nerves. Common in shaft of femur, tibia or humerus Unlike osteosarcoma, it arises in diaphysis rather than the metaphysis of bone It usually affects people from the ages of 5 to 20 Males are more prone . CLINICAL FEATURES A lump near skin that feels warm and soft to the touch Constant low fever Limited range of motion Bone pain that gets worse when you exercise or during the night Broken bones without an obvious cause Weight loss Always being tired Paralysis or loss of bladder control if the tumor is near spine. WORK UP / DIAGNOSIS X-rays Bone scan Magnetic resonance imaging (MRI) CT scan Positron emission tomography scan ( PET scan) Biopsy Bone marrow aspiration and biopsy X-rays shows destruction of bone (onion-peel appearance) TREATMENT
dactinomycin, doxorubicin) Surgery: remove the tumor to stop its spread. In some cases, amputation of an arm or leg is done if the tumor has spread a lot. Radiation MYELOMA Also known as myelomatosis or plasmacytoma Arises from the plasma cells of bone marrow, in 50-70 age group Spreads to many parts of the skeleton through blood stream CLINICAL FEATURES: Pathological fractures Local pain Patient is pale from associated anemia Local tenderness over affected bones Prone to develop infections due to suppression of normal antibody production ESR and serum globulin increased Imaging: X-rays show lytic lesions especially in bones containing red marrow such as ribs, vertebral bodies, pelvic bones, skull and proximal ends of femur and humerus Treatment: Radiotherapy Chemotherapy (drugs – thalidomide, dexamethasone, proteasome inhibitor, bortezomib)