Soft Tissue Sarcomas General

Question 1

What are Soft Tissue Sarcomas (STS)?

Answer 1

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

Question 2

What is the origin of Soft Tissue Sarcomas?

Answer 2

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

Question 3

From which embryonic layer do Soft Tissue Sarcomas arise?

Answer 3

Arise from the mesoderm/connective tissue.

Question 4

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

Answer 4

Over 50 identified soft tissue sarcomas.

Question 5

Are Soft Tissue Sarcomas common?

Answer 5

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

Question 6

Why are Soft Tissue Sarcomas grouped in one classification?

Answer 6

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

Question 7

In which types of connective tissues do STS tumors arise?

Answer 7

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

Question 8

What are sarcomas classified by?

Answer 8

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

Question 9

How many types of sarcomas are currently known?

Answer 9

Currently, more than 50 types of sarcomas are known.

Question 10

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

Answer 10

The most common STS that affects children is rhabdomyosarcoma.

Question 11

What are the most common types of sarcomas among adults?

Answer 11

The most common types among adults are liposarcoma and leiomyosarcoma.

Question 12

What is Rhabdomyosarcoma?

Answer 12

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

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

Question 13

What type of muscle does Leiomyosarcoma affect?

Answer 13

Smooth muscles, which are not under voluntary control.

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

Question 14

Where is Hemangiosarcoma most commonly found?

Answer 14

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

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

Question 15

What is Kaposi’s sarcoma associated with?

Answer 15

Immune deficiencies, such as HIV/AIDS.

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

Question 16

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

Answer 16

Lymphangiosarcoma.

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

Question 17

What type of tissues does Synovial sarcoma typically affect?

Answer 17

Tissue around joints such as knees and ankles.

Synovial sarcoma usually occurs in children and young adults.

Question 18

Neurofibrosarcoma occurs in which part of the body?

Answer 18

Peripheral nerves.

This type of sarcoma is associated with neurofibromatosis.

Question 19

What type of tissue is affected by Liposarcoma?

Answer 19

Fatty tissue, often in the legs and trunk.

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

Question 20

Fibrosarcoma affects which types of tissues?

Answer 20

Fibrous tissue in the arms, legs, or trunk.

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

Question 21

Where does Dermatofibrosarcoma typically grow?

Answer 21

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.

Question 22

Where do most soft tissue sarcomas (STS) occur?

Answer 22

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

Question 23

In which age groups are STS most commonly seen?

Answer 23

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

Question 24

Where are tumors most often located in adults?

Answer 24

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

Question 25

Where are STS tumors most often located in pediatric patients?

Answer 25

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

Question 26

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

Answer 26

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

Question 27

Is there a gender predominance in STS cases?

Answer 27

There is a slight male predominance.

Question 28

In which population are the highest rates of STS observed?

Answer 28

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

Question 29

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

Answer 29

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

Question 30

What is Von Recklinghausen's neurofibromatosis?

Answer 30

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

Question 31

What are some environmental exposures linked to STS?

Answer 31

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

Question 32

How do most patients with STS initially present?

Answer 32

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

Question 33

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

Answer 33

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

Question 34

Which areas are often diagnosed earlier for STS?

Answer 34

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

Question 35

What are some clinical symptoms of STS?

Answer 35

Paresthesia and edema are common symptoms.

Question 36

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

Answer 36

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

Question 37

How do tumors grow in STS?

Answer 37

Tumors grow longitudinally rather than radially.

Question 38

What laboratory studies may be valuable in STS diagnosis?

Answer 38

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

Question 39

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

Answer 39

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

Question 40

What imaging study is widely used for retroperitoneal tumors?

Answer 40

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

Question 41

What does angiography demonstrate in STS?

Answer 41

Angiography demonstrates tumor malignancy on the basis of prominent vascularity.

Question 42

What is the purpose of chest radiography in STS?

Answer 42

Chest radiography screens for pulmonary metastasis.

Question 43

What is the role of a bone scan in STS?

Answer 43

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

Question 44

What is required to confirm the histology in STS?

Answer 44

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

Question 45

What are the two most common grading systems for STS?

Answer 45

The two most common systems are FNCLCC and AJCC.

Question 46

How does local invasion occur in STS?

Answer 46

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

Question 47

Is lymphatic spread common in sarcomas?

Answer 47

Lymphatic spread is rare and not common for sarcomas.

Question 48

What is the primary route of spread for STS?

Answer 48

Hematogenous spread is the primary route of spread.

Question 49

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

Answer 49

The lung is the most common site for metastatic spread.

Question 50

What are other common sites for metastatic spread of STS?

Answer 50

Other common sites include bones, liver, and skin.

Question 51

What is the primary cause of death from STS?

Answer 51

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