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30 Cards in this Set
- Front
- Back
The majority of soft tissue tumors are __ tumors
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benign
benign tumors are at least 100X more common than maligant soft tissue tumors |
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Benign soft tissue tumors are typically located ___ and are less than ___ cm
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superficially (99% of benign soft tissue tumors!)
<5cm |
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Most STS (soft tissue sarcomas) occur in the ___(___%) with 10% in the ___ and ___
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extremity (75%)
10% (trunk wall and retroperitoneum) |
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__% of soft tissue sarcomas are classified as either:
1. high grade pleomorphic sarcoma (MFH-like) 2. liposarcoma 3. leiomyosarcoma 4. synovial sarcoma 5. MPNST |
75%!!
|
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Breast cancer patients treated with XRT are at increased risk for sarcoma. The risk increase with dose (most received ___GY or more). The typical interval between XRT and tumor diagnosis is ___
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50 GY or more
10 years |
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Breast cancer patients treated with XRT: most secondary sarcomas are classified as __
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>50% are MFH
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Patients with germline mutations in the retinoblastoma gene have a significantly increased risk of post-irradiation sarcoma, usually __
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osteosarcoma
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Sarcomas associated with virus:
1. HHV8 2. EBV |
HHV8 - Kaposi sarcoma
EBV: smooth muscle tumors in immunodeficient patients |
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Breast cancer patients that are status post lymphadenectomy are at risk for what sarcoma
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angiosarcoma arsing in the setting of chronic lymphedema
(Stewart-Treves syndrome) |
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Gardners syndrome
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desmoid tumors
adenomatous polyposis osteomas epidermal cysts |
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In __% of patients with NF type 1, a MPNST develops in a benign neurofibroma
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2% (uncommmon!)
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two examples of benign soft tissue tumors requring wide-local excision similar to that for a sarcoma
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desmoid-type fibromatosis
intramuscular hemangioma - recurrences are frequent |
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Excisional biopsy of a sarcoma is __
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inappropriate!
should get a diagnostic biopsy for any superifical tumor >5cm or any size deep tumor prior to excision |
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Most soft tissue sarcomas present as seemingly __ tumors
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innocent tumors
- often painless - incidentally noticed - no effect on general health or function |
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A superficial soft tissue tumor >5cm or any deep soft tissue tumor has a __% chance of being a sarcoma
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10%
|
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With all new sarcoma diagnosis the patient should get a baseline ___
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chest CT
- for accurate staging - evidence of metastasis - 10% pts present with metastatic disease (most commonly to the lung) |
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Soft tissue tumors are divided into 4 categories (behavior)
WHO |
1. benign
- usually do not recur locally - if they do, they are non-destructive and easily cured by complete excision 2. intermediate (locally aggressive) - often recur locally - infiltrative, locally destructive growth - DO NOT metatasize!! - require wide excision - example: desmoid-type fibromatosis 3. intermediate (rarely metastazing) - locally aggressive, as above - DO have potential for occasional distant metatasis (<2% chance) - metastatic risk cannot be reliable predicted based on morphology 4. malignant |
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Two most important parameters in grading STS
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1. mitotic index
2. extent of tumor necrosis |
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STS grading mainly indicates the probability of __ and __, but is poor at predicting ___
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1. metatasis
2. overall survival 3. local recurrence (this is mainly related to the quality of the excision) |
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French Sarcoma Group grading systems evaluates __ parameters:
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3 parameters:
1. tumor differentiation 2. mitotic count 3. tumor necrosis |
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French Sarcoma Group grading system: tumor differentiation is scored__
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1-3/3
1: sarcoma resembles normal adult mesenchymal tissue 2: sarcoma with recognizable histologic type 3: includes: - embryonal and undifferentiated sarcoma - sarcomas of doubtful type - synovial sarcomas - osteosarcomas - PNET |
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What 3 soft tissue sarcomas (that are graded) are automatically scored a 3 for tumor differentiation
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1. synovial sarcoma
2. osteosarcoma 3. PNET Therefore these tumors are automatically grade 2 or 3 |
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French Sarcoma Group grading system: histologic grades are based on total score
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Grade1: score 2-3 (low grade)
Grade 2: score 4-5 (intermediate grade) Grade 3: score 6-8 (high grade) |
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French Sarcoma Group grading system: mitotic count is scored
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score 1: 0-9/10 hpf
score 2: 10-19/ 10 hpf score 3: >/=20/ 10 hpf |
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French Sarcoma Group grading system: tumor necrosis is scored
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score 0: no necrosis
score 1: <50% tumor necrosis score 2: >/= 50% |
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In contrast to the French Sarcoma Group, the NCI sarcoma grading system uses __% tumor necrosis to separate grade 2 from grade 3
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15%
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STS for which grading is NOT recommended: (6 entities)
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- angiosarcoma
- extraskeletal myxoid chondrosarcoma - alveolar soft part sarcoma - clear cell sarcoma - epithelioid sarcoma - MPNST |
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STS staging is based on both __ and __.
TNM systems incorporates: |
grade and clinical findings
1. histologic grade 2. tumor size and depth 3. regional LN involvement 4. distant metastasis |
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Among STS, adjuvant and neoadjuvant or primary CTX is given for __ and __
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1. Ewing's sarcoma
2. rhabdomyosarcoma |
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Most common retroperitoneal sarcoma __
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WD liposarc/dediff liposarc
- recur locally and multifocally in the retroperitoneum - distant mets ONLY in 20% cases of dediff liposarcoma CONTRAST: - high grade retroperitoneal leiomyosarcoma: >50% develop distant mets (lung and liver) |