Soft Tissue sarcoma Flashcards

(95 cards)

1
Q

Rare tumors

A
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2
Q

Most common site of soft tissue sarcoma

A

Extremeity 59%
Trunk 19%
Retroperitoneum 13%
Head and neck 9%

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3
Q

Most common histologic types of soft tissue sarcoma in adults (excluding Kaposis sarcoma)

A
Malignant fibrous histiocytoma 28%
Leiomyosarcoma 12%
Liposarcoma 15%
Synovial sarcoma 10%
Malignant peripheral nerve sheath tumors 6%
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4
Q

Most common soft tissue sarcoma of childhood

A

Rhabdomyosarcoma

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5
Q

Sarcomas do not seem to result from the progression or differentiation of benign soft tissue tumors except

A

Malignant peripheral nerve sheath tumors

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6
Q

Clinical behavior of most soft tissue sarcomas is similar and iis determined by

A

Anatomic location

Depth
Grade
Size

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7
Q

The most dominant pattern of metastasis is

A

Hematogenous in the lungs

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8
Q

Lymphnode metastases are

A

Rare

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9
Q

Is a well established risk factor for soft tissue sarcoma

A

External radiation therapy

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10
Q

Incidence of sarcomas has been reported among patients treated for cancer

A
Breast
Cervix
Ovary
Testes
Lymphatic system
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11
Q

Radiation exposure

Post irradiation sarcomas, the most common histologic types were

A

Osteogenic sarcoma
Malignant fibrous histiocytoma
Angiosarcoma
Lymphangiosarcoma

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12
Q

Radiation exposure

Usually diagnosed at advanced stages and generally have a poor prognosis

A

Post irradiation

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13
Q

Chemicals linked to soft tissue sarcoma

A

Phenoxyacetic acid

Chlorophenols

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14
Q

Several chemical carcinogen shave been associated with hepatic angiosarcomas

A

Thorium oxide (thoratrast)
Vinyl chloride
Arsenic

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15
Q

Patient with soft tissue sarcoma linked to chemicals died due to

A

Massive blood loss

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16
Q

No casual relationship has been establish

A

Trauma

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17
Q

Filarial infections

A

Lymphangiosarcoma

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18
Q

Oncogenes identified in association with soft tissue sarcoma

A

MDM2
N-MYC
C-erB2
ras

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19
Q

Oncogenes produce specific oncoproteins that either play a role

A

Nuclear function
Cellular signal transduction
Function as growth factors
Growth factor receptors

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20
Q

Two tumor suppressor genes that are most relevant to soft tissue tumors

A

Retinoblastoma tumor suppressor gene

P53 tumor suppressor gene (most common 30-60%)

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21
Q

Can lead to development of retinoblastoma or sarcoma of soft tissue and bone

A

Mutations or deletion of RB

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22
Q

Most common mutation in human solid tumors and have been reported in 30-60% of soft tissue

A

Mutations in the p53

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23
Q

Known as recklinghausen’s disease

Occurs in approximately 1 every 3000 persons

A

Neurofibromatosis type 1

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24
Q

Neurofibromatosis type 1

Due to mutations in the

A

NF-1 tumor suppressor gene

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25
Neurofibromatosis type 1 How many percent have mutation in NF-1
50%
26
Initial assessment Most commonly presents as
Asymptomatic mass
27
Initial assessment The size at presentation is usually associated with the
Location of the tumor
28
Initial assessment Smaller tumor located at the
Distal extremities
29
Initial assessment Tumors can grow quite large before becoming apparent
Proximal extremities and | Retroperitoneum
30
Initial assessment Soft tissue sarcoma often grow in a
Centrifugal fashion
31
Initial assessment Impingement on bone or neurovascular bundles produces
Pain Edema Swelling
32
Initial assessment Retroperitoneal soft tissue sarcoma almost always presents as a
Large asymptomatic mass
33
Initial assessment Patient may present with
Obstructive gastrointestinal symptoms | Neurologic symptoms
34
Initial assessment The differential diagnosis of a soft tissue mass includes benign lesions including
Lipomas Lymphangiomas Leiomyomas Neuromas
35
Initial assessment In addition to sarcomas, other malignant lesions such as
Primary or metastatic carcinomas Melanomas Lymphomas
36
Initial assessment Small lesion not changed for years
Closely observed
37
Initial assessment All other tumors considered
Biopsy to establish definitive diagnosis
38
Diagnostic imaging Purposes
``` Local extent of tumor Stage Biopsy Benign or Monitor tumor changes Recurrences ```
39
Diagnostic imaging Provide useful information on primary bone tumors but they are not useful in the evaluation of soft tissue sarcomas of the extremeties
Radiographs X- ray
40
Diagnostic imaging For primary sarcomas to assess for lung metastases
Chest radiography
41
Diagnostic imaging High grade lesions Tumor larger than 5cm T2
Computed tomography
42
Diagnostic imaging Preferred technique for evaluating retro peritoneal sarcomas
CT scan
43
Diagnostic imaging Soft tissue sarcomas of the extremities
MRI
44
Diagnostic imaging Guiding fine needle aspiration or core biopsy for initial diagnosis or at recurrence
Ultrasonography | CT scan
45
Ultrasonography For patient cannot undergo
MRI
46
Ultrasonography Useful adjunct to MRI when findings are
Indeterminate | Delineating adjacent
47
Assess the extent of soft tissue tumor
Contrast-enhanced CT
48
Detailed survey of the abdomen and pelvis and delineate adjacent organs and vascular structures
Current CT technique
49
CT may be useful if MRI is not available or cannot be used
For extremity sarcoma
50
CT of the abdomen and pelvis should be done when histologic assessment of an extremity sarcoma reveals
Myxoid liposarcoma
51
Accurately delineates muscle groups and distinguishes among bone, vascular structures, and tumor.
MRI
52
Soft tissue sarcomas of the extremities ususally present on MRO as
Heterogenous mass
53
MRI May be seen in tumor
Hemorrhage | Cystic or necrotic changes
54
Performed if adjacent vascular structures must be delineated
Magnetic resonance angiography
55
Diagnosing most soft tissue sarcomas
Fine needle aspiration
56
Procedure choice to confirm or rule out the presence of a metastatic focus or local recurrence
Fine needle aspiration
57
Fine needle aspiration Often subjected to fine needle aspiration biopsies in the clinical setting
Superficial lesions
58
Fine needle aspiration Require an interventional radiologist to perform the technique under sonographic or CT guidance
Deeper tumors
59
Fine needle aspiration Diagnostic accuracy rates for the fine needle aspiration biopsy of primary tumors range
60-96%
60
Safe, accurate, economical diagnostic procedure for diagnosing sarcomas
Core needle biopsy
61
Core needle biopsy Sample obtained used for several Dx test such as
Electron microscopy Cytogenic analysis Flow cytometry
62
Core needle biopsy Reported complication rate
Less than 1 percent
63
Core needle biopsy More accurately pinpointing the location of tumor
Use of CT guide
64
Core needle biopsy Accuracy
93% compared with diagnostic given at the time of definitive treatment
65
Reliable diagnostic method that allows adequate tissue to be sampled for definitive and specific histologic identification of bone or soft tissue sarcomas
Open or incisional biopsy
66
Most reliable of the diagnostic methods, Accurate histologic diagnosis Grading in more than 95% of soft tissue sarcomas
Open biopsy
67
Entire tumor is remove
Excisional biopsy
68
Superficial extremity or truncal lesions smaller than 3cm
Excisional biopsy
69
Excisional biopsy Should not be done for lesions of
Hands and feet
70
Excisional biopsy Percent of recurrence
30-40%
71
May cause postoperative complications that could ultimately delay definitive therapy
Excisional biopsy
72
Pathologic classification
More prognostic significance
73
Pathologic classification Tumors with limited metastatic potential include
Desmoids also called well differentiated liposarcoma Dermatofibrosarcoma protuberans Hemangiopericytomas
74
Pathologic classification Tumors with an intermediate risk of metastatic spread usually have a large myxoid component and include
Myxoid liposarcoma Myxoid malignant fibrous histiocytoma Extra skeletal chondrosarcoma
75
Pathologic classification Highly aggressive tumors that have substantial metastatic potential are
``` Angiosarcoma Clear cell sarcomas Rhabdomyosarcoma- most aggressive Synovial sarcomas Leiomyosarcomas ```
76
Primary tumor
T1 tumor
77
Regional lymph nodes
N0 no regional lymph node | N1 regional
78
Distance metastasis
M0 - no distant | M1- distant
79
Histologic grade
G1- well differentiated G2- moderately differentiated G3- poorly differentiated G4- undifferentiated
80
Most important prognostic factor for patients with sarcomas
Histologic grade
81
Histologic grade Feature that define grade are
``` Cellularity Differentiation Pleomorphism Necrosis Number of mitoses ```
82
Histologic grad Shown to predict the development of metastases and overall survival
Tumor grade
83
Histologic grade Metastatic potential
5-10% low grade 25-30% intermediate 50-60% high grade tumors
84
Recognized to be an important prognostic variable in soft tissue sarcoma
Tumor size
85
Tumor size T1 lesion T2 lesion
5cm or smaller | Larger than 5cm
86
Tumor size can provide more accurate prognostic information
5 year survival rate percentage 15 cm - 33%
87
Lymph node metastasis of soft tissue is rare,
Less than 5% manifest nodal spread
88
Nodal metastasis Yung may mga incidence
Rhabdomyosarcoma Epithelioid sarcoma Malignant fibrous histiocytoma
89
Nodal disease
Designated as stage 4 disease
90
Distant metastasis Occur most often in
Lungs
91
Distant metastasis Pulmonary metastasis
May survive for long periods after surgical resection and chemotherapy
92
Distant metastasis Other potential sites
Bone Brain Liver
93
Distant metastasis | High incidence of liver and peritoneal metastasis
Visceral and retro peritoneal sarcoma
94
Yung part 2 start sa treatment,
Sayo na kung gawan mo ba ng BS
95
Sarcomas represent
1% of adult tumors | 15% of pediatric tumors