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22 Cards in this Set

  • Front
  • Back
Age distribution
M:F preference
Racial preference
Incidence: rare - <1% all new non-skin cancer diagnoses
Age distribution: median 40-60 years
M:F preference: M > F
Racial preference: None
1 Chronic lymphedema
--Lymphangiosarcoma (Stewart-Treves syndrome)
--Filarial infections
2 Postradiation sarcoma
--Incidence 0.03-0.22%
--Median latency 10 years
--Malignant fibrous histiocytoma most common
3 Exposure to phenoxyacetic acid herbcides (Agent Orange)
Hereditary sarcomas
1 Neurofibromatosis I (von Recklinghausen's disease)
2 Neurofibromatosis II (hereditary acoustic neuroma)
3 Hereditary retinoblastoma
4 Gardner's Syndrome
5 Li-Fraumeni Syndrome
6 Gene translocations
Neurofibromatosis I (von Recklinghausen's disease)
-Autosomal dominant
-Mutation causes lack of function in neurofibromin gene
-Mutliple benign neurofibromas
-High incidence of malignant peripheral nerve sheath tumors
--Malignant schwannoma
--Neurogenic sarcoma
Neurofibromatosis II (hereditary acoustic neuroma)
-Autosomal dominant disorder
-Mutant merlin protein
-Associated with meningioma, glioma, and schwannoma
Hereditary retinoblastoma
-Autosomal dominant
-Lack of function of retinoblastoma gene
-Associated with high risk of bone and soft-tissue sarcoma
Gardner's Syndrome
-Autosomal dominant
-Variant of hereditary polyposis coli
-Associated with desmoid tumors and rarely fibrosarcoma and liposarcoma
Li-Fraumeni Syndrome
-Autosomal dominant
-Incomplete penetrance
-Lack of function of p53
-Causes high incidence
--Breast cancer in younger patients than typical
Gene translocations
1 Myxoid liposarcoma t(12;16)
2 Clear-cell sarcoma
3 Alveolar rhabdomyosarcoma
4 Synovial sarcoma
5 Ewing's saroma
1 Myxoid liposarcoma t(12;16)
2 Clear-cell sarcoma t(12;22)
3 Alveolar rhabdomyosarcoma t(2;13)
4 Synovial sarcoma t(X;18)
5 Ewing's saroma t(11;22)
Most common soft-tissue sarcomas
MFH - 20-30%
Liposarcoma - 10-20%
Synovial sarcoma - 5-10%
Rhabdomyosarcoma - 5-10%
Leiomyosarcoma - 5-10%
Malignant peripheral nerve sheath tumor (malignant schwannoma) - 5-10%
Fibrosarcoma - 5-10%
Useful marker in histologic diagnosis
-Cytoskeletal protein usually in mesenchymal tissue
-Integral part of cytoskeleton of cardiac, skeletal, and smooth muscle
-Present in most rhabdomyosarcomas
-Present in half of leiomyosarcomas
-Present rarely in Ewing's, liposarcoma, and MFH
-Characteristic of epithelia
-Classical for carcinoma
-Also in epithelioid sarcoma, synovial sarcomas, and other STSs
S-100 protein
-Brain-specific protein
-Present in nearly all melanomas and clear-cell sarcomas
-Also found in malignant nerve sheath tumors and some carcinomas
Pathology of STSs
1 Type of tissue sarcoma
2 Grade of sarcoma
Criteria for grade
1 Cellular differentiation
2 Cellular pleomorphism
3 Degree of cellularity
4 Mitotic activity
5 Necrosis
6 Invasiveness
Staging systems
1 AJCC - four-tier
2 Common use
Desmoid tumors: Dose

- Gross disease

- Microscopic residual disease
Desmoid tumors: Dose

- Gross disease
-- 56Gy @ 2Gy/Fx
-- Equal to 60Gy @ 1.8Gy/Fx

- Microscopic residual disease
-- 50 Gy / 25 Fx

- From Ballo JCO 1999
Desmoid tumors

10-year recurrence
Desmoid tumors

10-year recurrence: 33%
Desmoid tumors

Bad prognostic factors
Desmoid tumors

Bad prognostic factors

- Positive margins
- Extremitity location
Desmoid tumors

Associated with what other syndromes?
Desmoid tumors

Associated with what other syndromes?

Familial adenomatous polyposis

Gardner's Syndrome
Desmoid tumors


Gender predilection

Age predilection
Desmoid tumors

Incidence: 3/Million

Gender predilection: F>M (except kids F=M)

Age predilection: 30-40s
Desmoid tumors

Resection categories

Desmoid tumors

Resection categories

R0: Negative margins
R1: Microscopic positive margins
R2: Macroscopic disease
Sarcoma staging: T stages
T1 <= 5 cm
- T1a Superficial
- T1b Deep
T2 > 5 cm
- T2a Superficial
- T2b Deep
Sarcoma staging: Group stages
I: T1a-b/T2a-bN0M0G1-2
II: T1a-b/T2a/N0M0G3-4
III: T2bN0M0G3-4
- Any T N1 M0 AnyG
- Any T N0M1 Any G