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Flashcards in Soft Tissue Sarcomas & Lymphomas Deck (62):
1

What are soft tissue sarcomas?

Soft tissue tumors, derived from mesoderm

2

What does "sarcoma" mean in Greek?

Fish flesh

3

Are sarcomas more common in upper or lower extremities?

Lower extremities (3.5 fold)

4

How common are soft tissue sarcomas?

0.6% of malignant tumors

5

What is the median age of diagnosis of soft tissue sarcoma?

55 years

6

What are the risk factors for soft tissue sarcoma?

RALES:
Radiation, AIDS, Lymphedema, Exposure (chemicals), Syndromes (Gardner's, Li-Fraumeni)

7

What is a malignant sarcoma of fat called?

Liposarcoma

8

What is a malignant GI sarcoma called?

GIST (GastroIntestinal Stromal Tumor)

9

What is a malignant sarcoma of myofibroblasts called?

Malignant fibrous histiocytoma

10

What is a malignant sarcoma of striated muscle called?

Rhabdomyosarcoma

11

What is a malignant sarcoma of vascular endothelium called?

Angiosarcoma

12

What is a malignant sarcoma of fibroblasts called?

Fibrosarcoma

13

What is a malignant sarcoma of lymph vessels called?

Lymphangiosarcoma

14

What is a malignant sarcoma of peripheral nerves called?

Malignant neurilemmoma or schwannoma

15

What is a malignant sarcoma seen in AIDS?

Kaposi's sarcoma

16

What is a malignant sarcoma associated with lymphedema?

Lymphangiosarcoma

17

What are the signs and symptoms of soft tissue sarcoma?

Soft tissue mass; pain from compression of adjacent structures

18

How do most sarcomas metastasize?

Hematogenously

19

What is the most common location and route of metastasis of sarcoma?

Lung via hematogenous route

20

What tests should be done in the preoperative workup?

CXR, +/- chest CT, LFTs

21

What are the 3 most common malignant sarcomas in adults?

Fibrous histiocytoma (25%), liposarcoma (20%), leiomyosarcoma (15%)

22

What are the 2 most common malignant sarcomas in children?

Rhabdomyosarcoma (50%), fibrosarcoma (20%)

23

What is the most common type of sarcoma to metastasize to the lymph nodes?

Malignant fibrous histiocytoma

24

What is the most common sarcoma of the retroperitoneum?

Liposarcoma

25

How do sarcomas locally invade?

Usually along anatomic planes such as fascia, vessels

26

How is the diagnosis of sarcoma made?

Imaging (MRI > CT)
Mass 3 cm: incisional biopsy or core biopsy

27

What is an excisional biopsy?

Biopsy by removing the entire mass

28

What is an incisional biopsy?

Biopsy by removing a piece of the mass

29

What is the orientation of incision for incisional biopsy of a suspected extremity sarcoma?

Longitudinal, not transverse, so that the incision can be incorporated in a future resection if biopsy for sarcoma is positive

30

What is a core biopsy?

Large-bore needle that takes a core of tissue

31

What determines histologic grade of sarcomas?

1. Differentiation
2. Mitotic count
3. Tumor necrosis
Grade 1 = well-differentiated
Grade 2 = moderately differentiated
Grade 3 = poorly differentiated

32

What is stage I sarcoma?

Well-differentiated (grade 1), any size, no nodes, no metastases

33

What is stage IIA sarcoma?

34

What is stage IIB sarcoma?

> 5 cm, grade 2

35

What is stage III sarcoma?

Positive nodes or > 5 cm and grade 3

36

What is stage IV sarcoma?

Distant metastases

37

What is a sarcoma pseudocapsule and what is its importance?

Outer layer of a sarcoma that represents compressed malignant cells.
Microscopic extensions of tumor cells invade through the pseudocapsule into adjacent structures, thus definitive therapy must include a wide margin of resection to account for this

38

What is the most important factor in the prognosis for sarcoma?

Histologic grade of the primary lesion

39

What is the treatment for sarcoma?

Surgical resection and radiation +/- chemo

40

What surgical margins are obtained in sarcoma resection?

2 cm (1 cm minimum)

41

What is the limb-sparing surgery for extremity sarcoma?

Avoidance of amputation with local resection and chemoradiation

42

What is the treatment of pulmonary metastasis of sarcoma?

Surgical resection for isolated lesions

43

What tests should be done in the followup for sarcoma resection?

PE, CXR, repeat CT/MRI

44

What syndrome of lymphangiosarcoma arises in chronic lymphedema after axillary dissection for breast cancer?

Stewart-Treves syndrome

45

What syndrome is associated with breast cancer and soft tissue sarcoma?

Li-Fraumeni syndrome (p53 tumor suppressor gene mutation)

46

How is the diagnosis of lymphoma made?

Cervical or axillary node excisional biopsy

47

What cell type is associated with the histology of Hodgkin's lymphoma?

Reed-Sternberg cells

48

What are the 4 histopathologic types of Hodgkin's lymphoma?

1. Nodular sclerosing
2. Mixed cellularity
3. Lymphocyte predominant
4. Lymphocyte depleted

49

What are the indications for a staging laparotomy in Hodgkin's lymphoma?

Rarely performed; Usually rely on CT scans, PET scans, bone marrow biopsy, and other directed imaging and biopsies

50

What is stage I Hodgkin's lymphoma?

Single lymph node region

51

What is stage II Hodgkin's lymphoma?

Two or more lymph node regions on the same side of the diaphragm

52

What is stage III Hodgkin's lymphoma?

Involvement on both sides of the diaphragm

53

What is stage IV Hodgkin's lymphoma?

Diffuse and/or disseminated involvement

54

What is stage A Hodgkin's lymphoma?

Asymptomatic

55

What is stage B Hodgkin's lymphoma?

Symptomatic (e.g. weight loss, fever, night sweats)

56

What treatments are used for low vs. advanced stage Hodgkin's lymphoma?

Low stage: Radiotherapy
Advanced: Chemotherapy

57

What percentage of patients with Hodgkin's lymphoma can be cured?

80%

58

What is GI lymphoma?

Non-Hodgkin's lymphoma arising in the GI tract

59

What is the risk factor for gastric lymphoma?

H. pylori infection

60

What are the signs and symptoms of GI lymphoma?

Abdominal pain, obstruction, GI hemorrhage, GI tract perforation, fatigue

61

What is the treatment of intestinal lymphoma?

Surgical resection with removal of draining lymph nodes and chemotherapy

62

What is the most common site of primary GI tract lymphoma?

Stomach (MALToma)