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30 Cards in this Set

  • Front
  • Back
The majority of soft tissue tumors are __ tumors

benign tumors are at least 100X more common than maligant soft tissue tumors
Benign soft tissue tumors are typically located ___ and are less than ___ cm
superficially (99% of benign soft tissue tumors!)

Most STS (soft tissue sarcomas) occur in the ___(___%) with 10% in the ___ and ___
extremity (75%)

10% (trunk wall and retroperitoneum)
__% of soft tissue sarcomas are classified as either:
1. high grade pleomorphic sarcoma (MFH-like)
2. liposarcoma
3. leiomyosarcoma
4. synovial sarcoma
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 ___
50 GY or more

10 years
Breast cancer patients treated with XRT: most secondary sarcomas are classified as __
>50% are MFH
Patients with germline mutations in the retinoblastoma gene have a significantly increased risk of post-irradiation sarcoma, usually __
Sarcomas associated with virus:

1. HHV8
2. EBV
HHV8 - Kaposi sarcoma

EBV: smooth muscle tumors in immunodeficient patients
Breast cancer patients that are status post lymphadenectomy are at risk for what sarcoma
angiosarcoma arsing in the setting of chronic lymphedema
(Stewart-Treves syndrome)
Gardners syndrome
desmoid tumors
adenomatous polyposis
epidermal cysts
In __% of patients with NF type 1, a MPNST develops in a benign neurofibroma
2% (uncommmon!)
two examples of benign soft tissue tumors requring wide-local excision similar to that for a sarcoma
desmoid-type fibromatosis
intramuscular hemangioma

- recurrences are frequent
Excisional biopsy of a sarcoma is __

should get a diagnostic biopsy for any superifical tumor >5cm or any size deep tumor prior to excision
Most soft tissue sarcomas present as seemingly __ tumors
innocent tumors
- often painless
- incidentally noticed
- no effect on general health or function
A superficial soft tissue tumor >5cm or any deep soft tissue tumor has a __% chance of being a sarcoma
With all new sarcoma diagnosis the patient should get a baseline ___
chest CT
- for accurate staging
- evidence of metastasis
- 10% pts present with metastatic disease (most commonly to the lung)
Soft tissue tumors are divided into 4 categories (behavior)
1. benign
- usually do not recur locally
- if they do, they are non-destructive and easily cured by complete excision

2. intermediate (locally
- 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
Two most important parameters in grading STS
1. mitotic index
2. extent of tumor necrosis
STS grading mainly indicates the probability of __ and __, but is poor at predicting ___
1. metatasis
2. overall survival

3. local recurrence (this is mainly related to the quality of the excision)
French Sarcoma Group grading systems evaluates __ parameters:
3 parameters:
1. tumor differentiation
2. mitotic count
3. tumor necrosis
French Sarcoma Group grading system: tumor differentiation is scored__
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
What 3 soft tissue sarcomas (that are graded) are automatically scored a 3 for tumor differentiation
1. synovial sarcoma
2. osteosarcoma

Therefore these tumors are automatically grade 2 or 3
French Sarcoma Group grading system: histologic grades are based on total score
Grade1: score 2-3 (low grade)
Grade 2: score 4-5 (intermediate grade)
Grade 3: score 6-8 (high grade)
French Sarcoma Group grading system: mitotic count is scored
score 1: 0-9/10 hpf
score 2: 10-19/ 10 hpf
score 3: >/=20/ 10 hpf
French Sarcoma Group grading system: tumor necrosis is scored
score 0: no necrosis
score 1: <50% tumor necrosis
score 2: >/= 50%
In contrast to the French Sarcoma Group, the NCI sarcoma grading system uses __% tumor necrosis to separate grade 2 from grade 3
STS for which grading is NOT recommended: (6 entities)
- angiosarcoma
- extraskeletal myxoid chondrosarcoma
- alveolar soft part sarcoma
- clear cell sarcoma
- epithelioid sarcoma
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
Among STS, adjuvant and neoadjuvant or primary CTX is given for __ and __
1. Ewing's sarcoma
2. rhabdomyosarcoma
Most common retroperitoneal sarcoma __
WD liposarc/dediff liposarc

- recur locally and multifocally in the retroperitoneum
- distant mets ONLY in 20% cases of dediff liposarcoma

- high grade retroperitoneal leiomyosarcoma: >50% develop distant mets (lung and liver)