Soft Tissue Sarcomas General Flashcards
What are Soft Tissue Sarcomas (STS)?
Group of tumors of the connective tissues except for bone, which is considered in a separate category.
What is the origin of Soft Tissue Sarcomas?
Part of the classification of solid tumors that arise from mesenchymal stem cells.
From which embryonic layer do Soft Tissue Sarcomas arise?
Arise from the mesoderm/connective tissue.
How many different types of Soft Tissue Sarcomas have been identified?
Over 50 identified soft tissue sarcomas.
Are Soft Tissue Sarcomas common?
Although they are rare, tumors that occur in soft tissue are more common than those in bone.
Why are Soft Tissue Sarcomas grouped in one classification?
They are grouped in one classification because there are approximately 50 different pathologic subtypes.
In which types of connective tissues do STS tumors arise?
STS tumors arise in connective tissues, including adipose tissue, fibrous muscle, nerve, and blood vessels.
What are sarcomas classified by?
Sarcomas are classified histologically and named according to the tissue in which they arise.
How many types of sarcomas are currently known?
Currently, more than 50 types of sarcomas are known.
What is the most common soft tissue sarcoma (STS) that affects children?
The most common STS that affects children is rhabdomyosarcoma.
What are the most common types of sarcomas among adults?
The most common types among adults are liposarcoma and leiomyosarcoma.
What is Rhabdomyosarcoma?
A sarcoma more common in children that occurs in the skeletal muscles.
Rhabdomyosarcoma is a malignant tumor of mesenchymal origin, typically affecting skeletal muscle.
What type of muscle does Leiomyosarcoma affect?
Smooth muscles, which are not under voluntary control.
Leiomyosarcoma is often found in the uterus, gastrointestinal tract, or lining of blood vessels.
Where is Hemangiosarcoma most commonly found?
In blood vessels, especially those in the arms, legs, and trunk.
Hemangiosarcoma is a rare and aggressive cancer that arises from blood vessels.
What is Kaposi’s sarcoma associated with?
Immune deficiencies, such as HIV/AIDS.
Kaposi’s sarcoma is a malignancy that occurs in blood vessel walls.
Which sarcoma affects lymph vessels and may be seen in limbs with chronic swelling?
Lymphangiosarcoma.
This condition can arise from prior radiation therapy or certain rare chronic infections.
What type of tissues does Synovial sarcoma typically affect?
Tissue around joints such as knees and ankles.
Synovial sarcoma usually occurs in children and young adults.
Neurofibrosarcoma occurs in which part of the body?
Peripheral nerves.
This type of sarcoma is associated with neurofibromatosis.
What type of tissue is affected by Liposarcoma?
Fatty tissue, often in the legs and trunk.
Liposarcoma is common in adults and can vary in its histological types.
Fibrosarcoma affects which types of tissues?
Fibrous tissue in the arms, legs, or trunk.
It is a malignant tumor that can arise in various locations in the body.
Where does Dermatofibrosarcoma typically grow?
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.
Where do most soft tissue sarcomas (STS) occur?
Most STSs occur in the extremities, followed by the trunk, retroperitoneum, and head and neck.
In which age groups are STS most commonly seen?
Bimodal age groups: Children and Adults - 50 to 60s.
Where are tumors most often located in adults?
Tumors are most often seen in the lower extremities (thighs) of adults.
Where are STS tumors most often located in pediatric patients?
Tumors are most often seen in the orbital region (head and neck) of pediatric patients.
What is the incidence rate of STS compared to other malignancies?
In comparison with other malignancies, the incidence rate of STS is fairly low.
Is there a gender predominance in STS cases?
There is a slight male predominance.
In which population are the highest rates of STS observed?
The highest rates of STS are amongst the African-American population.
What genetic syndrome is associated with a high risk of sarcomas?
Li-Fraumeni syndrome is associated with a high risk for the development of multiple types of cancer, including sarcomas.
What is Von Recklinghausen’s neurofibromatosis?
A familial disease that involves multiple benign tumors of the nerves.
What are some environmental exposures linked to STS?
Phenoxyacetic acid herbicides, ionizing radiation exposure, and previous radiation therapy treatment.
How do most patients with STS initially present?
Most patients initially present with a painless, gradually enlarging mass situated deep within tissue.
What symptoms may a tumor in the abdomen/pelvis cause?
A tumor in the abdomen/pelvis may cause pain or bloating as it increases in size.
Which areas are often diagnosed earlier for STS?
Head and neck and distal extremities are often diagnosed earlier than tumors of the thigh or retroperitoneum.
What are some clinical symptoms of STS?
Paresthesia and edema are common symptoms.
What can lack of clinical symptoms lead to in STS diagnosis?
Lack of clinical symptoms may lead to a delay of diagnosis of 4-28 months.
How do tumors grow in STS?
Tumors grow longitudinally rather than radially.
What laboratory studies may be valuable in STS diagnosis?
Cytogenetics may be of value because different tumor types have different specific chromosomal abnormalities.
What is the diagnostic tool of choice for imaging studies in STS?
MRI - it provides exceptional soft tissue contrast and specificity for optimal visualization of tumor size and shape.
What imaging study is widely used for retroperitoneal tumors?
CT - it is especially useful in the staging process because most STS metastasize to the lungs.
What does angiography demonstrate in STS?
Angiography demonstrates tumor malignancy on the basis of prominent vascularity.
What is the purpose of chest radiography in STS?
Chest radiography screens for pulmonary metastasis.
What is the role of a bone scan in STS?
Bone scan is used for risk assessment and is an essential part of the staging and work-up.
What is required to confirm the histology in STS?
Core Needle Biopsy - multiple samples are necessary to obtain an accurate diagnosis.
What are the two most common grading systems for STS?
The two most common systems are FNCLCC and AJCC.
How does local invasion occur in STS?
Local growth pattern of STS follows the lines of least resistance in the longitudinal axis of the primary site compartment.
Is lymphatic spread common in sarcomas?
Lymphatic spread is rare and not common for sarcomas.
What is the primary route of spread for STS?
Hematogenous spread is the primary route of spread.
What is the most common site for metastatic spread of STS?
The lung is the most common site for metastatic spread.
What are other common sites for metastatic spread of STS?
Other common sites include bones, liver, and skin.
What is the primary cause of death from STS?
Death from STS is more likely to result from metastatic disease, most commonly to the lungs.
