- Basics
- Site of Origin
- 59% Extremity
- 19% Trunk, ant abd wall (desmoid), flank
- 15% Retroperitoneum
- 9% Head and neck
- Site of Origin
- Risk Factors
- Trauma: burn, scar, chronic tissue irritation, chronic lymphedema
- Genetics
- Neurofibromatosis: Von Recklinghausen’s Disease
- Gardner’s Syndrome
- Tuberous Sclerosis
- Li-Fraumeni Syndrome
- Fap- desmoid
- Radiation: 10 year latency after radiation therapy or exposure
- Periphery of radiation (bad)
- Chemical: polyvinyl chloride, burns
- Lymphedema: Stewart-Treves Syndrome
- Classification: Based on soft tissue they resemble
- Presentation: MC painless lump, slow growing
- Radiologic Eval
- Plain Xray: location of bone tumor assoc fractures and cortical disruption
- CT scan abd & pelvis: always use IV contrast
- CT scan chest: look for mets
- Pre op cxr v ct: cxr adequate
- metadata > 1 cm mets matter
- MRI: if lesion is >4cm
- Bone Scan: detects mets, eval cortical and intraosseus extent of bone tumors
- Angiogram if necessary to assess vascular relationship to tumor
- Staging TGNM
- Tumor Size
- T1 <5cm: a superficial to fascia, b invades
- T2 >5cm
- Grade: Extent of differentiation mitotic rate, necrosis
- Path most imp for grade
- >25 mitosis for 50 high power fields = high grade = radiation tx
- Subtype not very imp
- Histo: Most important prognostic factors
- Low grade: 5-10% metastic
- Intermed: 25-30% metastatic
- High Grade: 50-60% metastatic
- Mets: MC pulm, retroperitoneal spread to liver first
- Route of tumor dissemination: hematogenous because they are mesenchymal cell origin
- Extremity and bone tumors usually spread to lung and then bone
- Abd, pelvic, and retroperitoneal: go to liver and lung
- Sarcoma rarely met to LN
- Primitive tumors: small cell, synovial, clear cell, angiosarcoma, rhabdo, epithelioid
- Chemo sensitive
- Pos nodes means complete LND
- Stage 4: mets or nodes
- Tumor Size
- Dx: made by biopsy
- FNA: not very helpful, small cells lots of fibrosis
- Core Needle Biopsy: ok but need to tract out in line of incision
- Excisional: tumor <3cm: ok but don’t enucleate
- Incisional Biopsy: orient biopsy scar with proposed resection lines
- Most deep, fixed, within musc
- Exception: DMFB: firm in SubQ
- Imaging before doesn’t’ change anything unless worry about neurvasc involvement
- Tx
- Complete surgical resection including biopsy sites
- Radical Surgery: improves local control
- <10% recurrence if: <5cm tumor, superficial, 1 fascial compartment, margin of 2cm
- DO NOT SHELL OUT: Wide local excision: 2cm margin preferable
- Muscle group excision
- Compartmental excision
- Drain inline with incision
- Amputation: reserved for recurrences and tumors that render extremity useless
- Radiation: Intermed to high grade tumor (high grade or low grade >5cm; high mitosis >10)
- Improves local control: no survival benefit
- high grade do respond
- Inadequate margins: limb preserving procedures, close margins around nerves or vasc structures
- all op area 5000 cgy and tumor bed 6500: external beam = brachytherapy
- Pre op vs post op:
- pre less fibrosis and edema worse wound healing
- post opposite; high grade in sensitive loc do pre
- Chemo
- No survival benefit after complete resection
- Doxorubicin, dacarbazine, ifosfamide (20-50% respond)
- Postop beneficial with positive margins and high grade histo
- DTIC clinical trial: adriamycin; Iphosphomide
- Adriamycin and ifosfamide demonstrate response rates of 20-30%
- 80% of skeletal sarc will develop recurrence
- 50% of pt with high grade sts will recur
- Adriamycin and ifosfamide demonstrate response rates of 20-30%
- Neoadjuvant best in osteosarc, ewings, rhabdomyosarc
- Improves limb sparing, improves overall survival
- Metastatic Dz = metastectomy
- 44% occur in lungs: f/u with Chest CT
- Tx: Surgery should be considered even if multiple and bilateral
- Stapled metastasectomy with minimal lung removed
- Not an option if:
- Primary dz not well controlled
- other systemic sites involved as well
- recurrent pulm nodules within 1 year of prior lung resection
- Complete surgical resection including biopsy sites
- Recurrence
- RF: >50 yo, >grade 2, retroperitoneal, tumor depth, size >5cm, pos margins, path: mfh, angiosarc, rhabdomyosarc
- Up to 50% of retroperitoneal and intra-abdominal sarcomas will recur after initial resection
- Retroperitoneal Sarcomas: local recurrence, liver and lung mets
- Extremity sarcomas: pulmonary metastases
- Aggressive f/u is life prolonging
- Follow up imaging q 4 mo
- Sarcomas that recur usually do so within 5 years
- Poor prognostic features: liposarc >1cm/mo, large >5cm, short disease free interval
- Slow growing sarcomas with long dz free interval should consider re-resection
- R0 resection, resect all prior incision, drain and specimen bed sites
- Consider neoadjuvant chemo/xrt if lesion is large, fast growing, near critical structures or difficult to achieve limb-spaing approach
- Resection is preferred treatment for recurrences
- Amputation
- Pulmonary metastasectomy: up to 40% 5 year survival
- Chem and radiation generally palliative at best
- Subtypes
- MC: malignant fibrous histiocytoma, liposarcoma, leiomyosarcoma
- MFH: mc soft tissue sarcoma, lower extremities, 20% of all extremity sarcoma
- Pleomorphic high grade sarc categorized here as well
- Liposarcoma: 2nd mc sts, usually lower extremities, 25% of extremity sarcomas and 45% retroperitoneal sarcomas
- Leiomyosarcoma: 20% of retroperitoneal sarcoma
- Bone sarcomas are classified according to the matrix they produce
- Osteosarcoma
- MC bone tumor
- Usually teenagers (15)/adolescents: male predominate
- In pt over 40 yo it is usually assoc with pagets dz or prior bone irradiation
- Chronic remodeling
- 80-90% occur in metaphysis of long bones:
- distal femur > proximal tibia > proximal humerus
- MCsx: painful soft tissue swelling and tenderness
- Tx: neoadjuvant chemo followed by limb sparing surgery
- Chondrosarcoma
- Usually 40-60yo
- Commonly occur along pelvis, femur and shoulder girdle
- Rarely responds to chemo (may be considered in young patient with high grade tumor
- Ewing’s sarcoma
- Almost always occur around 20yo, Caucasian
- 90% assoc with distinct chromosomal translocation 11:22 q24 q12 which results in a chimeric protein EWS/FLI-1
- Destructive intramedullary lesions that affect the diaphysis
- Osteosarcoma
- Dermatofibrosarcoma: lateral microscopic extensions
- Check CD34, 30-50
- Tx/ wide local excision with 4cm
- GIST: AKA leiomyosarcoma
- Arise from interstitial cells of cajal: gastric pacemaker cells
- Expresses CD34 (hematopoetic progenitor cell)
- Expresses CD 117/c-kit (growth factor receptor)
- Ckit is an oncogene
- Can affect any portion of GI tract: stomach most common
- Sx appear late and are subtle
- Bleeding and ulceration may occur
- Obstruction from larger tumor
- Malignant GIST similar presentation to benign but:
- Size >5cm
- High mitotic rate (>10 mitoses/high power field)
- Necrosis
- Invasion of other structures
- Metastases
- Endoscopy: EUS: shows submucosal tumor: possibly with ulceration
- Biopsies often nondiagnostic
- CT scanning of Abd/Pelvis
- Complete surgical resection best tx if it is possible: 2-4cm margin optimal
- Survival dependent on size/resectability
- 75% 5-year survival for smaller resected tumors
- 20-40% overall for malignant GIST tumors
- Chemo: C-kit inhibitor: imatinib
- Indication: metastatic, locally advanced and incompletely resected gist tumor
- 54% demonstrate tumor reduction: 20% tumor stability
- ckit oncogene expression: 40% recurrence
- targets tyrosine kinase inhibitor
- Recurrence in 40-90% surgically tx pt
- RF: tumor size, mitotic index, tumor location, tumor rupture during surgery
- Desmoid: nsaid, sulindac, tamoxifen
- Retroperioneal Sarcoma:
- Ddx
- renal cell, adrenal (primary adrenal coricocarc, pheo)
- lymphoma (other nodes, b sx, tumor marker ldh)
- non seminomatous extra gonadal germ cell tumors (Bhcg, afp)
- Represent 15% of sarcoma
- Dx delayed d/t lack of sx
- Usually >10cm at time of presentation
- 50% are larger then 20cm at diagnosis
- Liposarc, MFH, and leiomyosarcomas most common
- Radiologic assessment
- Chest, abd and pelvic ct scan with IV/PO contrast
- Assess for liver and lung mets
- Assess vascular invasion
- Tx: Complete surgical resection
- Difficult to achieve tumor free margins
- on the test you must get 1cm
- in practice very hard: attempt to get grossly clear margin
- Achievable in 40-60% of pt
- Survival 103 mos v 18 mo with incomplete resection
- Often involves radical extirpative procedures: bowel resection, nephrectomy, pancreatectomy
- Gluteal tumor: abut the sciatic nerve
- Fem nerve involvement: lose knee extension
- Sciatic nerve involvement: lose most leg
- Local recurrence: chemo and rad pos
- Difficult to achieve tumor free margins
- 5 yr survival 40-50%
- Ddx
- MC: malignant fibrous histiocytoma, liposarcoma, leiomyosarcoma
Sarcoma
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