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
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: excisional biopsy 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?
< 5 cm, grade 2 or 3
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)