Sarcoma Flashcards

1
Q

What are sarcomas?

A

Tumors that show evidence of mesenchymal differentiation

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2
Q

Most common location for sarcoma?

A

Extremities. Extremity sarcomas account for nearly 50% of adult sarcomas while retroperitoneal and intra-abdominal sarcomas represent approximately 15%.

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3
Q

Name several genetic syndromes associated with sarcoma development.

A

Gardner’s syndrome (multiple desmoid tumors), Li-Fraumeni syndrome (soft tissue sarcoma osteosarcoma), Neurofibromatosis I (malignant peripheral nerve sheath tumors)

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4
Q

Name several environmental factors associated with sarcoma development.

A

Herbicide exposure, pervious radiation exposure (> 3 year delay from exposure), lymphedema (angiosarcoma - Stewart-Treves Syndrome)

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5
Q

What is the primary treatment modality for soft-tissue sarcomas for all locations and the majority of histologic subtypes?

A

En-block surgical resection

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7
Q

How do extremity sarcomas typically present?

A

Either a mass in the area or a trivial traumatic event initially draws attention to the area.

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8
Q

What is the first step in management when a mass is discovered?

A

Biopsy, typically core needle biopsy

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9
Q

What are alternative ways to biopsy?

A

Open biopsy - excisional if the mass is < 3cm or incisional if > 3cm

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10
Q

Should you include the biopsy tract in the resection?

A

Yes absolutely

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11
Q

How is the histologic type of sarcoma determined?

A

Given >50 different types, classification is based on light microscopic appearance, presumed site of origin, and immunohistochemical and molecular markers.

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12
Q

What is the most common soft tissue sarcoma of the extremity?

A

Liposarcoma and malignant fibrous histiocytoma.

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13
Q

What is the most common soft tissue sarcoma of the extremity in the pediatric population?

A

Rhabdomyosarcoma and fibrosarcoma

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14
Q

What are the most important predictors of outcome in extremity sarcomas?

A

Histologic type, grade, and size

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15
Q

What are the most common imaging modalities for evaluation of soft tissue sarcomas?

A

CT and MRI

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16
Q

What is the most common site of metastasis from extremity soft tissue sarcoma?

A

Lungs - CT scan of the chest is indicated, particularly for high-grade lesions

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17
Q

How are sarcomas staged?

A

They are staged based off the standard TNM system, but grade is also included.

18
Q

List the different categories of the tumor type (T)

A

Tx (Primary tumor cannot be assessed), T0 (No evidence of primary tumor), T1 (Tumor 5cm in greatest dimension), T2a (Superficial tumor), T2b (Deep tumor)

19
Q

List the different categories of the regional lymph nodes (N)

A

Nx (Regional lymph nodes cannot be assessed), N0 (No lymph node metastasis), N1 (Regional lymph node metastasis)

20
Q

List the different categories of metastasis (M)

A

M0 (No distant metastasis), M1 (Distant metastasis)

21
Q

List the different categories of histologic grade (G)

A

Gx (Grade cannot be assessed), G1 (Grade 1), G2 (Grade 2), G3 (Grade 3)

22
Q

What typically surrounds a sarcoma?

A

Pseudocapsule

23
Q

Should the pseudocapsule be removed?

A

Absolutely - local recurrence rates decreased dramatically when this was recognized. Radical resection is often performed.

24
Q

What margins are acceptable for resection?

A

1-2 cm margins are ideal. (Wide excision alone with 1cm margins is sufficient for low grade sarcomas. When tumors are close to neural or bony structures, close margins are accepted to avoid significant functional impairment.

25
Q

When should adjuvant radiation therapy be considered?

A

For recurrent sarcoma or in lesions > 5cm, particularly if margins are < 1 cm

26
Q

Should neoadjuvant radiation therapy be considered?

A

Depends: higher wound complication rates are risked without significant benefit to recurrence or survival. It is generally considered for those with tumors initially considered too large to resect with acceptable morbidity.

27
Q

What is brachytherapy?

A

Intraoperative placement of catheters within the resection bed followed by loading of radioactive isotope.

28
Q

What are the advantages of brachytherapy?

A

Less radiation scatter, much shorter duration o therapy

29
Q

What are the indications for brachytherapy?

A

High grade lesions (XRT used for high grade and low grade lesions).

30
Q

Should adjuvant chemotherapy be considered?

A

Individual trials have failed to demonstrate a survival benefit. It is not considered standard therapy.

31
Q

How should a local recurrence be treated?

A

Aggressive reexcision

32
Q

What is isolated limb perfusion?

A

Regional therapy where a limb is infused with chemo therapeutic agents.

33
Q

What are the critical determinants of sarcoma specific survival?

A

Size of local recurrence and time to recurrence.

34
Q

How is local recurrence of sarcomas treated?

A

Aggressive reexcision encompassing previous scar and drain sites with consideration of additional chemoradiation and brachytherapy considered.

35
Q

What is the most common type of sarcoma of the retroperitoneum?

A

Liposarcoma and leiomyosarcoma

36
Q

What is the most common type of visceral sarcoma?

A

GIST, Leiomyosarcoma, and desmoid

37
Q

What is the prognosis for retroperitoneal and visceral sarcomas?

A

Poor due to its location for surgical resection, radiation administration, and delayed diagnosis.

38
Q

How are suspected retroperitoneal and visceral sarcomas initially treated?

A

Imaging (CT or MRI), percutaneous biopsy is not usually performed due to concerns for seeding unless lymphoma or germ cell rumors are suspected.

39
Q

What is the most effective treatment for retroperitoneal and visceral sarcomas?

A

Surgical resection. Chemotherapy has t been shown to be effective and radiation risks damage to other organs.

40
Q

What if the retroperitoneal or visceral sarcoma is unresectable?

A

Neoadjuvant chemoradiation should be considered to reduce the size of the tumor to allow for complete surgical resection.