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95 Cards in this Set
- Front
- Back
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Most common cancers
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Lung > Prostate > Breast
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Ennekings 4 questions
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location, matrix (bone, cartilage, fat, gland-like, spindle, mitotic figures- essentially histology) , what is tumor doing to bone, what is bone doing to tumor
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Hazards of Biopsy
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done by surgeon peforming definitive surgery; don’t give antibiotics until tissue taken, do not violate compartments
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Types of biopsy
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FNA (75% accuracy), Core (85% accuracy), Open (96% accuracy). Open Bx is gold standard and probably the right answer on a test
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Immunohistochemisty: S-100
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Neurve root tumors such as schwannoma, malignant peripheral nerve sheath tumor
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Immunohistochemisty: CD-99
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Ewings
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Immunohistochemisty: Keratin
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Synovial Cell Sarcoma
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Immunohistochemisty: Smooth muscle actin
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leiomyosarcoma
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Immunohistochemisty: Desmin
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Rhabdomyosarcoma
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Immunohistochemisty: CD 31 or 34
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endothelial cells/vascularity: Hemangioma, angiosarcoma
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Immunohistochemisty: CD 45
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Lymphoma; also LCA (leukocyte common antigen)
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Translocations: t(11;22)
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Ewings Sarcoma; gene is EWS-FLI1
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Translocations: t(X;18)
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Synovial Cell Sarcoma, gene is SSX1-SYT
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Translocations: t(12;16)
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Myxoid liposarcoma (between the ages of 12 and 16 your all mixed up)
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Translocations: t(2;13)
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Alveolar Rhabdomyosarcoma (it’s just bad, like a rabid dog)
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Translocations: t(12;22)
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Clear cell sarcoma (clearly, a 12 year old should not date a 22 year old)
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Vimentin positive
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Ewings
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Ennekings classifications
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Benign arabic, Malignant Roman; A is intracompartmental, B is extracompartmental; I is low grade, II is high grade, III is metastatic
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Ennekings classification of benign tumors
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1: latent, 2: active, 3: aggressive
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Prognostic factors in soft tissue tumors
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Size (>5cm), grade (most important), depth (deep to investing fascia)
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Types of surgical resection
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Incisional: within lesion; Marginal: at tumor margin; Wide: normal margin of tissue; Radical: entire compartment removed
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Adjuvant treatment for bone sarcomas
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chemotherapy and surgery for bone sarcomas (ewings and osteosarcoma)
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Soft tissue sarcomas adjuvant treatment
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typically radiotherapy plus surgery (wide excision)
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Heterotopic bone prophylaxis radiation dose
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5 Gy
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Soft tissue sarcoma radiation dose
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50-65 Gy
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Most common stage of osteosaroma at presentation
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IIB (high grade malignant, extracompartmental, not metastatic)
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Concern with atraumatic hematoma
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often a soft tissue sarcoma- sarcoma with hemorrhage looks identical to hematoma; biopsy before washout.
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Most prognositic feature of soft tissue sarcoma
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Histologic grade is more important than size and depth
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Distant metastasis in soft tissue sarcoma
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80% of mets go to lungs, 80% metastasize within 2 years
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Chemotherapy in soft tissue sarcomas
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No real role- radiotherapy is preferred by most musculoskeletal oncologists.
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Most common soft tissue tumors
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MFH, liposarcoma, synovial Sarcoma, leiomyosarcoma- know histology of these
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MRI imaging soft tissue sarcomas
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Bright on T2, dark on T1 except for low grade liposarcoma- looks like normal fat
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Benign Fibromatosis
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firm nodules- hand (Dupuytens) Feet (lederhosen’s disease) MRI- dark on T1 & T2; resection requires complete resection
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Lipoma versus Liposarcoma
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Determined by biopsy; larger lesions (>5-6cm) more likely to have some malignant component
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Schwannoma- clinical features
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often positive tinnels sign; can see egg-shaped or spherical tumor on nerve; very easy to resect surgically (not interdigitated with nerve)
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Neurofibroma- clinical features
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solitary lesions not associated with major nerve- often near skin; resect only if symptomatic
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Malignant peripheral nerve sheath tumor
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S-100 positive; arise spontaneously or in NF patients; tumor usually follows nerve; Treatment same as STS- radiation and wide excision
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Most common soft tissue tumor in children
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Rhabdomyosarcoma; if found in adults, however, basically a death sentence
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Small round blue cell tumors <LERNM>
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Lymphoma, ewings, rhabdomyosarcoma, Neuroblastoma (infants), Multiple Myeloma
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Hemangiomas
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phleboliths on x-ray, well organized vascular channels on histology; Bright on T2; Treatment is observation
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Angiosarcoma- clinical presentation
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cutaneous lesions, classically is patient with lymphedema post lymph node resection in breast cancer; CD 31 or 34
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Angiosarcoma- histology
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Histologically rudimentary disorganized vascular channels w/ spindle cells
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Synovial Sarcoma
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doesn’t necessarily come from synovium
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Epitheloid sarcoma
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small lesion most common in hand/forearm; tends to have lymph node mets; mimic other hand conditions
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Soft tissue sarcoma with lymph mets
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epithelioid sarcoma, angiosarcoma, rhabdomyosarcoma
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PVNS- treatment
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gold standard open synovectomy; if not diffuse, may be amenable to arthroscopy; can do chemotherapy if recalcitrant
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Synovial chondromatosis
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“rice bodies” often ossified and visible on x-ray
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Cat scratch disease and soft tissue sarcoma
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mimics soft tisue sarcoma; expansion of lymph node. Treatment is Antibiotics; organism is bartonella <distal humerus node>
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Order of Staging
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1. History and Physical 2. Local Staging 3. Systemic Staging 4. Biopsy
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Bone producing lesions
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osteoid osteoma, osteoblastoma, osteosarcoma
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Diagnosis and Staging of suspected multiple myeloma
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Diagnosis: SPEP/UPEP; Staging: skeletal survey (not bone scan)
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Osteoid osteoma: clinical features & Tx
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Nidus < 2cm; self limited growth, thickened cortex on one side, responds well to NSAIDs, sclerotic nidus on CT; Tx excise or radiofrequency ablation
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Osteoblastoma: clinical features & Tx
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age 10-25, >2cm on imaging, pain not limited by NSAIDS; may be mixed lytic/blastic and expansile diaphyseal lesion with central lucency; Histology shows osteoid; Tx: intralesional resection
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Pagets disease relationship to tumors
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can degenerate into osteosarcoma- bimodal distribution of osteosarcoma; tends to be more axial; very bad prognosis
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Parosteal osteosaroma
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usually posterior distal femur; suck-on appearance- no intramedullary connection (as opposed to osteochondroma); low grade; treat with wide excision only; no chemotherapy!
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Telangiectatic osteosarcoma: clinical features
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aggressive with large amounts of lysis; fluid/fluid levels (like ABC); malignant osteoid with vascular lakes and channels and some giant cells
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Telangiectatic osteosarcoma vs Aneurysmal Bone Cyst
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Malignant osteoid, both have vascular lakes; ABC has well organized gascular channels with mature bone around periphery
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Fibrosarcoma
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Herringbone pattern
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Enchondroma: clinical features and Tx
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Most common bone tumor in hands and feet; calcified cartilage nest in metaphysis, usually asymptomatic and incidentally found; No tratment needed, do not Biopsy
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Ollier’s Disease
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Inborn error of enchondral ossification with multiple enchondromas
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Mafucci’s Disease
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Multiple enchondromas with hemangiomas; higher risk than Ollier’s of malignant transformation (angiosarcoma and chondrosarcoma)
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Cartilage cap on osteochondroma size
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if greater than 2 - 2.5 cm higher risk of it being malignant
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Multiple osteochondromatosis
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EXT 1 and EXT 2 tumor suppressor genes (AD); malignant transformation possible
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Chondromyxoid fibroma: clinical features and Tx
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benign aggressive; eccentric, lytic lesion of metaphysis; managed by curettage and graft/cement- do not observe or send to PT
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Treatment of aggressive benign tumors
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do not observe; do procedural treatment as indicated
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Chondroblastoma
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Lytic epiphyseal lesion with stippled calcified matrix in skeletally immature; Biopsy to make sure diagnosis is correct, then curretage and graft
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Epiphyseal lesions
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Chondroblastoma, Brody’s abscess, clear cell sarcoma, Giant cell tumor
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Benign Aggressive tumors- examples
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chondroblastoma, chondromyxoid fibroma, Giant Cell Tumor, Adamantinoma
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Chondrosarcoma treatment
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Surgery only, no radiotherapy or chemotherapy
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Enchondroma versus chondrosarcoma
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chondrosarcoma has some bony architecture change- tumor is affecting surrounding bone; also chondrosarcoma is more likely to be really painful
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Osteofibrous dysplasia
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anterior tibial cortex, diaphyseal; multiloculated intracortical lucencies; often develops into adamantinoma; Tx: follow; excise if symptomatic
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Tumors with rimming osteoblasts
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Osteofibrous dysplasia (fibrous dysplasia does not have rimming)
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Fibrous dysplasia
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ground glass x-ray; shepherd’s crook deformity; increased production of cAMP due to upregulated adenylate cyclase; no rimming osteoblasts
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McCune Albright
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polyostotic fibrous dysplasia w/ coast of Maine Cafe au lait, precocious puberty
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Hemangioma of spine
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usually asymptomatic; honeycomb or jail-bar appearance; well organized vascular channels
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Lymphoma: clinical features and Tx
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Very little bony destruction but with large surrounding soft-tissue mass; diffuse bone involvement; treatment is chemo and radiation, sometimes surgery
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Multiple Myeloma: clinical features and Tx
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>50 years old; SPEP and UPEP; IgG monoclonal spikes, kappa and lambda light chains; clock face nuclei that are eccentric; cold on bone scan so stage with skeletal survey
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Chordoma: clinical features and Tx
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anterior sacral mass, bubbly appearing cytoplasm; treatment is wide resection- be aggressive because people die from local spread
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Langerhans Cell Histiocytosis
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AKA Eosinophilic granuloma; Histologically see histiocytes with Birbeck granuels (tennis racket inclusion bodies on EM); will also see eosinophils (red) on slide
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Hand-Schuller-Christian disease
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2-5 yrs; one and visceral; develop diabetes insipidus
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Giant cell tumor: clinical features and Tx
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benign aggressive lytic epiphyseal lesion; eccentric. Nucleus of cells in stroma have same appearance as nuclei in giant cell; Intralesional resection
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Most common classification at presentation of Ewings
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IIB (high grade malignant, extracompartmental, not metastatic)
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Treatment Ewings
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same as osteosarcoma- neoadjuvant chemo, surgery, followed by chemo
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Adamantinoma: clinical features and Tx
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diaphyseal tibia, soap bubble appearance with bowing on x-ray; Treatment is wide surgical excision. Local recurrance 90% with positive margins
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6-MP
- mech - toxicities - metabolized by what? added toxicity with what? |
blocks denovo purine synth.
actv by HGPRTase --> Purine analog BMS, GI, Liver. xanthine oxidase; thus trouble to give with allopurinol. |
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Unicameral (simple) Cyst
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postulated physeal disturbance; central location, less expansive, see in skeletally immature; fallen leaf sign. Tx: observe or inject with steroid or bone graft
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Tumors with fluid/fluid levels
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ABC, telangiectatic OSA, Giant cell tumor (at times)
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ABC versus UBC/simple cyst
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ABC in skeletally mature, RBC’s in channels, eccentric, no ‘fallen leaf’, Treat with open currettage and bone grafting
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Mirel’s criteria for prophylactic ORIF pathologic Fracture
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>50% cortical destruction, >2.5 cm lesion proximal femur, pain at presentation
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Treatment impending pathologic Fx
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Bisphosphonates; fix whole bone (no short nails), XRT post treatment (takes care of reamings) , PMMA <never bone graft>
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Urine marker in pagets
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NTX (N-telopeptide)
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Pagets versus Osteopetrosis
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Diaphyseal Tumors (AEIOU and sometimes Y)
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Adamantanoma, EG, infection, osteoid osteoma/osteoblastoma Uings (Ewings), Y- fibrous dYsplasia/ lYmphoma/ mYeloma
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Posterior element tumors
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Osteoid osteoma, osteoblastoma, ABC
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Most common soft tissue sarcoma?
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MFH
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