Presentation on primary bone tumors

Submitted by srrpenna on Sat, 2008-07-12 09:53.
::
This is my presentation at SHO teaching at Warrington hospital on 8/07/08.
content Slide 1: Primary tumors of bone Mr Sreeram Penna www.eyst.org
Slide 2: Malignant Tumors of Bone Osteosarcoma  Chondrosarcoma  Ewing Sarcoma  Chordoma  Adamantinoma  Malignant Vascular tumors  Malignant fibrous histocytoma and  fibrosarcoma  Multiple myeloma, Plasmacytoma and Lymphoma  Metastatic bone tumors www.eyst.org
Slide 3: Benign/Aggressive tumors Giant cell tumor   Chondroblastoma  Chondromyxoid fibroma  Osteoblastoma  Langerhans cell histiocytosis www.eyst.org
Slide 4: Benign tumors Bone-forming Osteoid Osteoma  Bone island  Cartilage Lesions Chondroma  Osteochondroma  Fibrous lesions Non ossifying fibroma  Cortical desmoid  Benign fibrous histocytoma  Fibrous displasia  Osteofibrous dysplasia  Desmoplastic fibroma  www.eyst.org
Slide 5: Benign tumors cont Cystic Lesions Unicameral bone cyst  Aneurismal bone cyst  Intraosseous ganglion cyst  Epideromoid cyst  Fatty tumors Lipoma  Vascular tumors Hemangioma  www.eyst.org
Slide 6: Non neoplastic conditions Pagets disease  Brown tumor of hyperparathyroidism  Bone infarct  Osteomyelitis  Stress fracture  Posttraumatic osteolysis  www.eyst.org
Slide 7: Principles of mx - Investigations Plain radiographs of the bone  CT scans  MRI  Bone scans  Chest X-ray  CT of chest  www.eyst.org
Slide 8: Grading Done histologically  Biopsy need to be performed by team prepared to do the  resection. www.eyst.org
Slide 9: Osteosarcoma Tumor characterised by production of osteoid by  malignant cells. 2nd most commonest primary bone tumor, 20% primary  malignancies,1-3 per 1 million population. Age: Primary high grade osteosarcoma – 10 – 20 yrs ,  Parosteal osteosarcoma – 3rd and 4th decade, Secondary osteosarcoma in older population M>F (except parosteal osteosarcoma)  Genetic association: Rothmund-Thompson syndrome, Li-  Fraumani syndrome, hereditary form of retinoblastoma. Location: distal femur, proximal tibia, and proximal  humerus. www.eyst.org
Slide 10: Osteosarcoma Symptoms: Progressive pain, night pain or painless mass.  Mostly metaphyseal, 10% diaphyseal, 1% epiphyseal  Classification :  - Primary Osteosarcoma: conventional low grade intramedullary parosteal periosteal high grade surface, telangectic small cell - Secondary Osteosarcoma www.eyst.org
Slide 11: Conventional Osteosarcoma High grade  Common type of  Osteosarcoma Common radiographic  appearance is aggressive lesion producing osteoid matrix. Periosteal reaction may take the form of “codmans triangle” or “sunbrust” or “hair on end” appearence www.eyst.org
Slide 12: Periosteal Osteosarcoma Intemidate grade  Arises from surface of  bone Commonly on femur and  tibia. www.eyst.org
Slide 13: Intramedullary Osteosarcoma Rare  Low grade  www.eyst.org
Slide 14: Parosteal Osteosarcoma Low grade malignancy  Rare  Arises on surface of bone  and invades medullary cavity only at later stages. It has peculiar tendency  to occur as a lobulated mass on the posterior aspect of femur www.eyst.org
Slide 15: High grade surface Osteosarcoma High grade  Least Common  Radiographs show  invasive lesions with with ill defined borders www.eyst.org
Slide 16: Telangectic Osteosarcoma Lytic lesion  www.eyst.org
Slide 17: Small cell Osteosarcoma Rare  High grade  Resemble Ewing sarcoma  or Lymphoma www.eyst.org
Slide 18: Secondary Osteosarcoma These occur at the site of another disease process  Older patients  E.g. Pagets disease, previous radiation treatment, fibrous  dysplasia, bone infarcts, osteochondromas, chronic osteomyelitis, dedifferentiated chondrosarcomas, melorhestosis and osteogenesis imperfecta. www.eyst.org
Slide 19: Investigations Plain radiographs  CT scan  Bone scan  MRI  Chest radiographs and CT for staging  www.eyst.org
Slide 20: Osteosarcoma treatment Neoadjuvant chemotherapy  Wide or radical surgery (resection or amputation)  Adjuvant chemotherapy  www.eyst.org
Slide 21: Chondrosarcoma 9% of primary malignancies of bone  Age: broad, primary chondrosarcoma peak around 40 –  60yrs, secondary chondrosarcoma 25 – 45 yrs. Any location but common around pelvis, proximal femur,  proximal humerus. Most common malignancy in hand.  Clinically: increasing pain and palpable mass. Pain in  absence of pathological fracture is helpful to differentiate between enchondroma and low grade chondrosarcoma www.eyst.org
Slide 22: Secondary Chondrosarcoma Olliers disease (multiple enchondromatosis)  Maffuci syndrome (multiple enchondromatosis + soft  tissue haemangioma) Multiple hereditary exostoses  Solitary osteochondroma  Synovial chondromatosis  Chondromyxoid fibroma  Periosteal Chondroma  Chondroblastoma  Previous radiation treatment  Firbours dysplasia  www.eyst.org
Slide 23: Chondrosarcoma - xrays ppearence similar to enchondroma, it is a lesion arising in medullary cavity with irregular matrix calcification. Pattern is described as “punctate,” “popcorn,” or “comma-shaped”. Compared with enchondroma, chondrosarcoma has more aggressive appearence with bone destruction and cortical erosions, periosteal reaction, and rarely soft-tissue mass. www.eyst.org
Slide 24: Mesenchymal chondrosarcoma www.eyst.org
Slide 25: Clear cell chondrosarcoma Epiphyseal Gaint Cell Tumor Chondroblastoma Clear cell chondrosarcoma www.eyst.org
Slide 26: Dedifferentiated chondrosarcoma Radiographic features of dedifferentiated chondrosarcoma often show a more aggressive radiolucent area juxtaposed on a otherwise typical chondrosarcoma. www.eyst.org
Slide 27: Chondrosarcomas Treatment Low grade – Extended curettage with use of  intraoperative adjuvant treatment. High grade – Wide or radical resection or amputation.  Radiotherapy as palliative for inaccessible lesions.  www.eyst.org
Slide 28: Ewing sarcoma 4th most common primary malignancy  9% of primary malignancy of bone  Age: most occur in 5 – 25 yrs  Commonly metaphyses of long bones(often extension to  diaphyses) and flat bones of shoulder and pelvic girdle. Males > Females  Clinically: Pain(insidious onset, mild and intermittent  initially), fever, erythema, swelling. Investigation: elevated ESR, raised CRP  www.eyst.org
Slide 29: Ewing sarcoma – xrays estructive lesion in the diaphyses of longbone with and “onion skin” periosteal reaction. www.eyst.org
Slide 30: Ewing sarcoma - MRI To evaluate full extent of  lesion www.eyst.org
Slide 31: Ewing sarcoma - investigations CXR  CT chest  Bone scan  Bone marrow aspirate  www.eyst.org
Slide 32: Ewing sarcoma – treatment Radiosensitive  Large central unresectable mass – radiotherapy.  Smaller more accessible lesions surgery.  Neo adjuvant and adjuvant chemotherapy  www.eyst.org
Slide 33: Chordoma Rare malignant neoplasm arises from notochord  remnants. Second most common in spine, most common in sacrum.  50% of chordomas arise in sacrococcygeal region, 30%  arise in base of the skull. Age sacrococcygeal 50 – 70 yrs spenooccipital 40 – 60 yrs  Clinically: spenooccipital: headaches, symptoms of cranial  nerve compression, retropharyngeal abscess. Sacrococcygeal: lower back pain, sciatic pain, bowel and bladder problems. Palpable mass on PR. Spinal: presents with nerve root or cord compression. www.eyst.org
Slide 34: Chordoma radiology Destructive lesions, virtually arising from  midline. www.eyst.org
Slide 35: Adamantinoma Arises from aberrant  epithelial cells. Prediliction to tibia  2nd and 3rd decade  Clinically pain and  palpable mass. Multiple sharply  demarcated lesions in tibial diaphyses DD: Osteofibrous  dysplasia. (diff by aggressive appearance) www.eyst.org
Slide 36: Malignant Vascular tumors Hemangioendothelio Angiosarcoma – high   ma – low grade grade www.eyst.org
Slide 37: Malignant fibrous histocytoma and fibrosarcoma 3 – 5 % primary bone  malignancies All ages except 1st decade  Both sexes affected equally  Tendency to occur in distal femur  and proximal tibia. 25% considered secondary from  paget’s disease, irradiation, giant cell tumor and bone infarction. Clinically presents with pain and  pathological fracture(20%) Radiologically aggressive  appearance and are typically purely lytic with indistinct borders. www.eyst.org
Slide 38: Giant Cell Tumor 5% of bone neoplasms  Age: 20 to 40 yrs  Female>Male  Location: distal femur> proximal tibia > distal  radius Benign but pulmonary metastasis can occur  Clinically: progressive pain, pathological  fractures(10 – 30 %) Radiologically lesions are eccentrically located in  the epiphysis of long bones and usually abut subchondral bone. Lesions are purely lytic. MRI useful for determining extent of disease.  www.eyst.org
Slide 39: Giant cell tumor – x rays www.eyst.org
Slide 40: Giant cell tumor – MRI Dark in T1 weighted image and Bright in T2 weighted images www.eyst.org
Slide 41: Giant cell tumor – treatment Aggressive extended curettage and use of adjuvants like  argon beam coagulator. And fill the bone cavity with cement or bone graft Inoperable lesions in pelvis use radiation or embolisation.  Follow up is very imp as lesions can reccur.  www.eyst.org
Slide 42: Chondroblastoma Age: 10 – 25 yrs  M:F is 2:1  Commonly distal femur, proximal humerus, and proximal  tibia. Older patients flat bones Clinically: progressive pain  Radiologically: well circumscribed lesion centered in  epiphyses of long bone, or an apophysis like greater trochanter or greater tuberosity. Often it has surrounding rim of reactive bone. In children well circumscribed epiphyseal lesion that  crosses open growth plate is diagnostic. In Adults DD is giant cell tumor and clear cell chondrosarcoma. Treatment is extended curettage and adequate follow-up  www.eyst.org
Slide 43: Chondroblastoma - adults www.eyst.org
Slide 44: Chondroblastoma - kids www.eyst.org
Slide 45: Chondromixoid Fibroma 10 – 30 yrs  Commonly proximal tibia  Clinically pain or swelling  Xrays – well circumscribed lesion with rim of sclerosis in  metaphyses of long bone. Intra lesional calcium is absent. Treatment – resection or extended curettage with bone  grafting www.eyst.org
Slide 46: Chondromixoid Fibroma www.eyst.org
Slide 47: Osteoblastoma Bone forming neoplasm  10 – 30 yrs old  M:F is 3:1  Any bone but commonly in spine  Clinically: Pain worse at night relived by NSAID, in spine  scoliosis or root or cord compression. Xrays: Spine it shows bone forming neoplasm in posterior  elements(DD aneurismal bone cyst and Osteoid osteoma). Out side spine calcified central nidus with surrounding radiolucent halo and reactive sclerosis. (DD Osteoid osteoma) Treatment extended curettage or resection. In spine may  require instrumented fusion. www.eyst.org
Slide 48: Osteoblastoma www.eyst.org
Slide 49: Langerhans Cell Histiocytosis Also known as Histocytosis X  Isolated bony lesions called as eosinophilic granuloma  5 – 20 yrs  Clinically progressive pain, fever, local signs of  inflammation Commonly affects vertebral bodies, flat bones and  diaphyses of long bones. X-rays – in spine vertebra plana. In flat bones lesions are  well circumscribed and “punched out” purely lytic lesions. In diaphyses of long bones aggressive permeative appearance with periosteal reactive bone formation. Biopsy is required for diagnosis  Treatment conservative (steroid therapy, radiation,  curettage) www.eyst.org
Slide 50: Langerhans Cell Histiocytosis www.eyst.org
Slide 51: Osteoid osteoma 2nd and 3rd decades of life  Any bone, but femur and tibia commonly  cortical or cancellous  Typically pain worse at night relieved by aspirin or  NSAID. Radiologically lesion consists of a small (< 1.5 cm) central  nidus with surrounding bony sclerosis Treatment – medical treatment, percutaneous  radiofrequency ablation, or surgical removal www.eyst.org
Slide 52: Osteoid osteoma www.eyst.org
Slide 53: Bone Island – Enostoses Benign lesions of cancellous bone  Any bone  Asymptomatic  Osteopoikilosis is multiple bone islands through out  skeleton X rays: typically small, round or oval areas of  homogeneous increased density with in cancellous bone. Treated with observation and serial radiographs.  If painful biopsy to exclude other conditions  www.eyst.org
Slide 54: Bone islands www.eyst.org
Slide 55: Chondroma Benign lesions of hyaline cartilage.  Phalanges of hand are most commonly affected.  Usually asymptomatic rarely pathological fracture  Enchondromas – arise from medullary canal, Periosteal  chondromas or Juxtacortical chondromas arise on surface Multiple enchondromatosis – Ollier’s disease  Multiple enchondromatosis + Soft tissue hemangiomas =  Maffuci syndrome Radiographically: benign appearing tumors with  intralesional calcification. Calcification is irregular and described as “strippled” “punctate” or “popcorn”. DD Chondrosarcoma Treatment is observation with serial radiographs ,  curettage if it grows www.eyst.org
Slide 56: Chondroma www.eyst.org
Slide 57: Osteochondroma Bony mass with form of a stalk produced by progressive  endochondral ossification of growing cartilage. Usually found on the metaphysis of long bones near  physis. Commonly distal femur, proximal tibia, and proximal  humerus. Clincally: pain, fracture, pressure symptoms like false  aneurisms, neuropathies, palpable mass. Multiple hereditary exostoses AD  Two types: Pedunculated or Sessile  Surgery(enbloc resection) is indicated when unsightly,  symptomatic or any suggestion of malignancy. www.eyst.org
Slide 58: Osteochondroma www.eyst.org
Slide 59: Non Ossifying fibroma Developmental abnormalities  Metaphyseal region of long bones (40% distal femur, 40%  tibia, 10% fibula) 2 – 20 yrs  Plain radiographs well lobulated lesion located  eccentrically in metaphysis. Asymptomatic  www.eyst.org
Slide 60: Non Ossifying fibroma www.eyst.org
Slide 61: Cortical Desmoid Is a irregularity in posteromedial aspect of distal femoral  metaphysis and usually is seen in boys 10 to 15 yr old. www.eyst.org
Slide 62: Benign fibrous Histiocytoma Diaphysis or epiphysis  30 – 40 yrs  Radiographically it is well defined lytic, expanding lesion  with little periosteal reaction Extended curettage or wide resection  www.eyst.org
Slide 63: Fibrous dysplasia Developmental anomaly  Replacement of normal bone and marrow by fibrous  tissue and small, woven spicules of bone. Radiographic appearance is lucent area with lucent area  having a granular, ground-glass appearance Surgical treatment is indicated when there is severe  deformity, pathological fracture occurs or when significant pain exists. Bisphosphonates treatment is probably beneficial.  www.eyst.org
Slide 64: Fibrous dysplasia www.eyst.org
Slide 65: Osteofibrous Dysplasia Ossifying fibroma of long bones  Tibia and Fibula (middle third of Tibia)  Diaphyseal  Tibia is enlarged and often bowed anteriorly  No pain unless pathological fracture  Radiographs show eccentric intracortical osteolysis with  expansion of cortex www.eyst.org
Slide 66: Osteofibrous Dysplasia www.eyst.org
Slide 67: Desmoplastic fibroma Locally aggressive  2nd and 3rd decades  Pain is complaint  Radiographs show well circumscribed lytic lesion with a  narrow zone of transition and frequently a reactive bone. Treatment wide resection  www.eyst.org
Slide 68: Desomplastic fibroma www.eyst.org
Slide 69: Unicameral bone cyst First 2 decades  M:F is 2:1  Common in proximal humerus and femur, in adults  common in pelvis and calcaneum. Asymptomatic unless pathological fracture  Radiographs centrally located , purely lytic lesion with well  marginated outline. Fallen fragment sign Small, asymptomatic lesions treated with observation and  serial radiographs Large, symptomatic, and lesions in lower extremity are  treated with curettage or aspiration and injection. www.eyst.org
Slide 70: Unicameral bone cyst www.eyst.org
Slide 71: Aneurysmal bone cyst Locally destructive blood filled reactive lesions of bone  Proximal humerus, distal femur, proximal tibia and spine  < 20 yrs  Mild to moderate pain, neurological symptoms in spinal  patient. Radiographically expansile lytic lesion that elevates  periosteum, but remains contained by a thin shell of cortical bone. Treatment with extended curettage and grafting  www.eyst.org
Slide 72: Aneurysmal bone cyst www.eyst.org
Slide 73: Intraosseous Ganglion Cyst Distal tibia, around knee and shoulder  Intra osseous extensions of ganglia of local soft tissues  www.eyst.org
Slide 74: Epidermoid cyst Skull  Radiographically they appear as rarefied defects  surrounded by sclerotic bone www.eyst.org
Slide 75: Lipoma Asymptomatic  www.eyst.org
Slide 76: Hemangioma Vertebral bodies and skull  Radiographically in spine  it is characteristic with thickened, vertically oriented trabecuale giving the classic “jailhouse” appearance www.eyst.org