Soft Tissue Sarcomas & Lymphomas Flashcards

1
Q

What are soft tissue sarcomas?

A

Soft tissue tumors, derived from mesoderm

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2
Q

What does “sarcoma” mean in Greek?

A

Fish flesh

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3
Q

Are sarcomas more common in upper or lower extremities?

A

Lower extremities (3.5 fold)

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4
Q

How common are soft tissue sarcomas?

A

0.6% of malignant tumors

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5
Q

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

A

55 years

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6
Q

What are the risk factors for soft tissue sarcoma?

A

RALES:

Radiation, AIDS, Lymphedema, Exposure (chemicals), Syndromes (Gardner’s, Li-Fraumeni)

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7
Q

What is a malignant sarcoma of fat called?

A

Liposarcoma

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8
Q

What is a malignant GI sarcoma called?

A

GIST (GastroIntestinal Stromal Tumor)

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9
Q

What is a malignant sarcoma of myofibroblasts called?

A

Malignant fibrous histiocytoma

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10
Q

What is a malignant sarcoma of striated muscle called?

A

Rhabdomyosarcoma

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11
Q

What is a malignant sarcoma of vascular endothelium called?

A

Angiosarcoma

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12
Q

What is a malignant sarcoma of fibroblasts called?

A

Fibrosarcoma

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13
Q

What is a malignant sarcoma of lymph vessels called?

A

Lymphangiosarcoma

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14
Q

What is a malignant sarcoma of peripheral nerves called?

A

Malignant neurilemmoma or schwannoma

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15
Q

What is a malignant sarcoma seen in AIDS?

A

Kaposi’s sarcoma

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16
Q

What is a malignant sarcoma associated with lymphedema?

A

Lymphangiosarcoma

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17
Q

What are the signs and symptoms of soft tissue sarcoma?

A

Soft tissue mass; pain from compression of adjacent structures

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18
Q

How do most sarcomas metastasize?

A

Hematogenously

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19
Q

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

A

Lung via hematogenous route

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20
Q

What tests should be done in the preoperative workup?

A

CXR, +/- chest CT, LFTs

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21
Q

What are the 3 most common malignant sarcomas in adults?

A

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

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22
Q

What are the 2 most common malignant sarcomas in children?

A

Rhabdomyosarcoma (50%), fibrosarcoma (20%)

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23
Q

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

A

Malignant fibrous histiocytoma

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24
Q

What is the most common sarcoma of the retroperitoneum?

A

Liposarcoma

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25
Q

How do sarcomas locally invade?

A

Usually along anatomic planes such as fascia, vessels

26
Q

How is the diagnosis of sarcoma made?

A

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

27
Q

What is an excisional biopsy?

A

Biopsy by removing the entire mass

28
Q

What is an incisional biopsy?

A

Biopsy by removing a piece of the mass

29
Q

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

A

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

30
Q

What is a core biopsy?

A

Large-bore needle that takes a core of tissue

31
Q

What determines histologic grade of sarcomas?

A
  1. Differentiation
  2. Mitotic count
  3. Tumor necrosis
    Grade 1 = well-differentiated
    Grade 2 = moderately differentiated
    Grade 3 = poorly differentiated
32
Q

What is stage I sarcoma?

A

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

33
Q

What is stage IIA sarcoma?

A

< 5 cm, grade 2 or 3

34
Q

What is stage IIB sarcoma?

A

> 5 cm, grade 2

35
Q

What is stage III sarcoma?

A

Positive nodes or > 5 cm and grade 3

36
Q

What is stage IV sarcoma?

A

Distant metastases

37
Q

What is a sarcoma pseudocapsule and what is its importance?

A

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
Q

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

A

Histologic grade of the primary lesion

39
Q

What is the treatment for sarcoma?

A

Surgical resection and radiation +/- chemo

40
Q

What surgical margins are obtained in sarcoma resection?

A

2 cm (1 cm minimum)

41
Q

What is the limb-sparing surgery for extremity sarcoma?

A

Avoidance of amputation with local resection and chemoradiation

42
Q

What is the treatment of pulmonary metastasis of sarcoma?

A

Surgical resection for isolated lesions

43
Q

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

A

PE, CXR, repeat CT/MRI

44
Q

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

A

Stewart-Treves syndrome

45
Q

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

A

Li-Fraumeni syndrome (p53 tumor suppressor gene mutation)

46
Q

How is the diagnosis of lymphoma made?

A

Cervical or axillary node excisional biopsy

47
Q

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

A

Reed-Sternberg cells

48
Q

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

A
  1. Nodular sclerosing
  2. Mixed cellularity
  3. Lymphocyte predominant
  4. Lymphocyte depleted
49
Q

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

A

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

50
Q

What is stage I Hodgkin’s lymphoma?

A

Single lymph node region

51
Q

What is stage II Hodgkin’s lymphoma?

A

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

52
Q

What is stage III Hodgkin’s lymphoma?

A

Involvement on both sides of the diaphragm

53
Q

What is stage IV Hodgkin’s lymphoma?

A

Diffuse and/or disseminated involvement

54
Q

What is stage A Hodgkin’s lymphoma?

A

Asymptomatic

55
Q

What is stage B Hodgkin’s lymphoma?

A

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

56
Q

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

A

Low stage: Radiotherapy

Advanced: Chemotherapy

57
Q

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

A

80%

58
Q

What is GI lymphoma?

A

Non-Hodgkin’s lymphoma arising in the GI tract

59
Q

What is the risk factor for gastric lymphoma?

A

H. pylori infection

60
Q

What are the signs and symptoms of GI lymphoma?

A

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

61
Q

What is the treatment of intestinal lymphoma?

A

Surgical resection with removal of draining lymph nodes and chemotherapy

62
Q

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

A

Stomach (MALToma)