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Pathology: Bone & Soft Tissue Sarcomas > Soft Tissue Sarcomas General > Flashcards

Soft Tissue Sarcomas General Flashcards

1
Q

What are Soft Tissue Sarcomas (STS)?

A

Group of tumors of the connective tissues except for bone, which is considered in a separate category.

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2
Q

What is the origin of Soft Tissue Sarcomas?

A

Part of the classification of solid tumors that arise from mesenchymal stem cells.

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3
Q

From which embryonic layer do Soft Tissue Sarcomas arise?

A

Arise from the mesoderm/connective tissue.

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4
Q

How many different types of Soft Tissue Sarcomas have been identified?

A

Over 50 identified soft tissue sarcomas.

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5
Q

Are Soft Tissue Sarcomas common?

A

Although they are rare, tumors that occur in soft tissue are more common than those in bone.

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6
Q

Why are Soft Tissue Sarcomas grouped in one classification?

A

They are grouped in one classification because there are approximately 50 different pathologic subtypes.

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7
Q

In which types of connective tissues do STS tumors arise?

A

STS tumors arise in connective tissues, including adipose tissue, fibrous muscle, nerve, and blood vessels.

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8
Q

What are sarcomas classified by?

A

Sarcomas are classified histologically and named according to the tissue in which they arise.

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9
Q

How many types of sarcomas are currently known?

A

Currently, more than 50 types of sarcomas are known.

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10
Q

What is the most common soft tissue sarcoma (STS) that affects children?

A

The most common STS that affects children is rhabdomyosarcoma.

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11
Q

What are the most common types of sarcomas among adults?

A

The most common types among adults are liposarcoma and leiomyosarcoma.

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12
Q

What is Rhabdomyosarcoma?

A

A sarcoma more common in children that occurs in the skeletal muscles.

Rhabdomyosarcoma is a malignant tumor of mesenchymal origin, typically affecting skeletal muscle.

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13
Q

What type of muscle does Leiomyosarcoma affect?

A

Smooth muscles, which are not under voluntary control.

Leiomyosarcoma is often found in the uterus, gastrointestinal tract, or lining of blood vessels.

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14
Q

Where is Hemangiosarcoma most commonly found?

A

In blood vessels, especially those in the arms, legs, and trunk.

Hemangiosarcoma is a rare and aggressive cancer that arises from blood vessels.

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15
Q

What is Kaposi’s sarcoma associated with?

A

Immune deficiencies, such as HIV/AIDS.

Kaposi’s sarcoma is a malignancy that occurs in blood vessel walls.

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16
Q

Which sarcoma affects lymph vessels and may be seen in limbs with chronic swelling?

A

Lymphangiosarcoma.

This condition can arise from prior radiation therapy or certain rare chronic infections.

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17
Q

What type of tissues does Synovial sarcoma typically affect?

A

Tissue around joints such as knees and ankles.

Synovial sarcoma usually occurs in children and young adults.

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18
Q

Neurofibrosarcoma occurs in which part of the body?

A

Peripheral nerves.

This type of sarcoma is associated with neurofibromatosis.

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19
Q

What type of tissue is affected by Liposarcoma?

A

Fatty tissue, often in the legs and trunk.

Liposarcoma is common in adults and can vary in its histological types.

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20
Q

Fibrosarcoma affects which types of tissues?

A

Fibrous tissue in the arms, legs, or trunk.

It is a malignant tumor that can arise in various locations in the body.

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21
Q

Where does Dermatofibrosarcoma typically grow?

A

In the tissue beneath the skin, often developing in the trunk or limbs.

Dermatofibrosarcoma is a rare type of skin cancer that can be locally aggressive.

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22
Q

Where do most soft tissue sarcomas (STS) occur?

A

Most STSs occur in the extremities, followed by the trunk, retroperitoneum, and head and neck.

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23
Q

In which age groups are STS most commonly seen?

A

Bimodal age groups: Children and Adults - 50 to 60s.

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24
Q

Where are tumors most often located in adults?

A

Tumors are most often seen in the lower extremities (thighs) of adults.

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25
Q

Where are tumors most often located in pediatric patients?

A

Tumors are most often seen in the orbital region (head and neck) of pediatric patients.

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26
Q

What is the incidence rate of STS compared to other malignancies?

A

In comparison with other malignancies, the incidence rate of STS is fairly low.

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27
Q

What is the annual mortality rate for STS?

A

Mortality is high, with about 5150 deaths per year.

28
Q

Is there a gender predominance in STS cases?

A

There is a slight male predominance.

29
Q

In which population are the highest rates of STS observed?

A

The highest rates of STS are amongst the African-American population.

30
Q

What percentage of new cancer cases does soft tissue cancer represent in the US?

A

Soft tissue cancer represents 0.7% of all new cancer cases in the US.

31
Q

What is the etiology of STS?

A

The etiology of STS is unknown; however, associations are found with a variety of genetic and environmental factors.

32
Q

What genetic syndrome is associated with a high risk of sarcomas?

A

Li-Fraumeni syndrome is associated with a high risk for the development of multiple types of cancer, including sarcomas.

33
Q

What is a risk associated with Li-Fraumeni syndrome?

A

Patients are very susceptible to secondary malignancies related to radiation therapy.

34
Q

What is Von Recklinghausen’s neurofibromatosis?

A

A familial disease that involves multiple benign tumors of the nerves.

35
Q

What are some environmental exposures linked to STS?

A

Phenoxyacetic acid herbicides, ionizing radiation exposure, and previous radiation therapy treatment.

36
Q

How do most patients with STS initially present?

A

Most patients initially present with a painless, gradually enlarging mass situated deep within tissue.

37
Q

What symptoms may a tumor in the abdomen/pelvis cause?

A

A tumor in the abdomen/pelvis may cause pain or bloating as it increases in size.

38
Q

Which areas are often diagnosed earlier for STS?

A

Head and neck and distal extremities are often diagnosed earlier than tumors of the thigh or retroperitoneum.

39
Q

What are some clinical symptoms of STS?

A

Paresthesia and edema are common symptoms.

40
Q

What can lack of clinical symptoms lead to in STS diagnosis?

A

Lack of clinical symptoms may lead to a delay of diagnosis of 4-28 months.

41
Q

What physical signs may be present in STS?

A

Presence of warmth or distended vascularity when fixed to other structures.

42
Q

How do tumors grow in STS?

A

Tumors grow longitudinally rather than radially.

43
Q

What is the initial step in the diagnostic work-up for STS?

A

Detailed history and physical examination to exclude other conditions.

44
Q

What laboratory studies may be valuable in STS diagnosis?

A

Cytogenetics may be of value because different tumor types have different specific chromosomal abnormalities.

45
Q

What is the diagnostic tool of choice for imaging studies in STS?

A

MRI - it provides exceptional soft tissue contrast and specificity for optimal visualization of tumor size and shape.

46
Q

What imaging study is widely used for retroperitoneal tumors?

A

CT - it is especially useful in the staging process because most STS metastasize to the lungs.

47
Q

What does angiography demonstrate in STS?

A

Angiography demonstrates tumor malignancy on the basis of prominent vascularity.

48
Q

What is the purpose of chest radiography in STS?

A

Chest radiography screens for pulmonary metastasis.

49
Q

What is the role of a bone scan in STS?

A

Bone scan is used for risk assessment and is an essential part of the staging and work-up.

50
Q

What is required to confirm the histology in STS?

A

Core Needle Biopsy - multiple samples are necessary to obtain an accurate diagnosis.

51
Q

Why is grading and staging essential in STS?

A

It is essential for determining prognosis and a treatment regimen.

52
Q

What are the two most common grading systems for STS?

A

The two most common systems are FNCLCC and AJCC.

53
Q

What is the recommended staging system for STS?

A

The recommended staging system is the AJCC or TNM system.

54
Q

What does the TNM system stand for?

A

Tumor, Node, Metastasis.

55
Q

What does ‘T’ represent in the TNM system?

A

‘T’ represents primary tumor size.

56
Q

What does ‘N’ represent in the TNM system?

A

‘N’ represents the extent of lymph node involvement.

57
Q

What does ‘M’ represent in the TNM system?

A

‘M’ indicates whether metastasis is present.

58
Q

What additional information is included in the TNM system?

A

The tumor depth grade is also included.

59
Q

What is the growth pattern of STS?

A

STS invades aggressively along local, anatomically defined planes composed of neurovascular structures, fascia, and muscle bundles.

60
Q

How does local invasion occur in STS?

A

Local growth pattern of STS follows the lines of least resistance in the longitudinal axis of the primary site compartment.

61
Q

What surrounds the primary STS?

A

The primary STS is surrounded by natural anatomic boundaries.

62
Q

Is lymphatic spread common in sarcomas?

A

Lymphatic spread is rare and not common for sarcomas.

63
Q

What is the primary route of spread for STS?

A

Hematogenous spread is the primary route of spread.

64
Q

What is the most common site for metastatic spread of STS?

A

The lung is the most common site for metastatic spread.

65
Q

What are other common sites for metastatic spread of STS?

A

Other common sites include bones, liver, and skin.

66
Q

What is the primary cause of death from STS?

A

Death from STS is more likely to result from metastatic disease, most commonly to the lungs.

Pathology: Bone & Soft Tissue Sarcomas (14 decks)

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