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22 Cards in this Set

  • Front
  • Back
Incidence
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
Etiologies
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
--Neurofibrosarcoma
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
--MFH
--Rhabdomyosarcoma
--Osteosarcoma
--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
Vimentin
-Cytoskeletal protein usually in mesenchymal tissue
Desmin
-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
Cytokeratin
-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
--Low
--Intermediate
--High
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

Incidence

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

R0
R1
R2
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
IV
- Any T N1 M0 AnyG
- Any T N0M1 Any G