Soft Tissue Sarcomas and Lymphomas, C62 P464-468 Flashcards by Tiggee R

SOFT TISSUE SARCOMAS
What are they?
P464

Soft tissue tumors, derived from mesoderm

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SOFT TISSUE SARCOMAS
Sarcoma means what in
GREEK?
P464

“Fish flesh”

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SOFT TISSUE SARCOMAS
Sarcomas are more common
in upper or lower
extremities?
P464

50% of sarcomas are in the extremities
and are 3.5 more common in the
lower extremity (thigh)

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SOFT TISSUE SARCOMAS
How common are they?
P464

0.6% of malignant tumors

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SOFT TISSUE SARCOMAS
What is the median age at
diagnosis?
P464

55 years

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SOFT TISSUE SARCOMAS
What are the risk factors?
P464

“RALES”:
    Radiation
    AIDS (Immunosuppression)
    Lymphedema
    Exposure to chemicals
    Syndromes (e.g., Gardner’s/Li-Fraumeni)

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SOFT TISSUE SARCOMAS
Name the following types of
malignant sarcoma:
Fat
P464

Liposarcoma

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SOFT TISSUE SARCOMAS
Name the following types of
malignant sarcoma:
Gastrointestinal
P464

GIST (GastroIntestinal Stromal Tumor)

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SOFT TISSUE SARCOMAS
Name the following types of
malignant sarcoma:
Myofibroblast
P464

Malignant fibrous histiocytoma

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SOFT TISSUE SARCOMAS
Name the following types of
malignant sarcoma:
Striated muscle
P464

Rhabdomyosarcoma

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SOFT TISSUE SARCOMAS
Name the following types of
malignant sarcoma:
Vascular endothelium
P464

Angiosarcoma

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SOFT TISSUE SARCOMAS
Name the following types of
malignant sarcoma:
Fibroblast
P464

Fibrosarcoma

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SOFT TISSUE SARCOMAS
Name the following types of
malignant sarcoma:
Lymph vessel
P465

Lymphangiosarcoma

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SOFT TISSUE SARCOMAS
Name the following types of
malignant sarcoma:
Peripheral nerve
P465

Malignant neurilemmoma or

schwannoma

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SOFT TISSUE SARCOMAS
Name the following types of
malignant sarcoma:
AIDS
P465

Kaposi’s sarcoma

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SOFT TISSUE SARCOMAS
Name the following types of
malignant sarcoma:
Lymphedema
P465

Lymphangiosarcoma

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SOFT TISSUE SARCOMAS
What are the signs/
symptoms?
P465

Soft tissue mass; pain from compression
of adjacent structures, often noticed after
minor trauma to area of mass

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SOFT TISSUE SARCOMAS
How do most sarcomas
metastasize?
P465

Hematogenously (i.e., via blood)

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SOFT TISSUE SARCOMAS
What is the most common
location and route of
metastasis?
P465

Lungs via hematogenous route

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SOFT TISSUE SARCOMAS
What tests should be done
in the preoperative workup?
P465

CXR, ± chest CT, LFTs

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SOFT TISSUE SARCOMAS
What are the three most
common malignant sarcomas
in adults?
P465

Fibrous histiocytoma (25%)
Liposarcoma (20%)
Leiomyosarcoma (15%)

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SOFT TISSUE SARCOMAS
What are the two most
common in children?
P465

Rhabdomyosarcoma (about 50%),

fibrosarcoma (20%)

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SOFT TISSUE SARCOMAS
What is the most common
type to metastasize to the
lymph nodes?
P465

Malignant fibrous histiocytoma

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SOFT TISSUE SARCOMAS
What is the most
common sarcoma of the
retroperitoneum?
P465

Liposarcoma

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SOFT TISSUE SARCOMAS How do sarcomas locally invade? P465

Usually along anatomic planes such as | fascia, vessels, etc.

SOFT TISSUE SARCOMAS How is the diagnosis made? P465

``` Imaging workup—MRI is superior to CT at distinguishing the tumor from adjacent structures Mass 3 cm: incisional biopsy or core biopsy ```

SOFT TISSUE SARCOMAS Define excisional biopsy. P466

Biopsy by removing the entire mass

SOFT TISSUE SARCOMAS Define incisional biopsy. P466

Biopsy by removing a piece of the mass

``` SOFT TISSUE SARCOMAS What is the orientation of incision for incisional biopsy of a suspected extremity sarcoma? P466 ```

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

SOFT TISSUE SARCOMAS Define core biopsy. P466

Large-bore needle that takes a core of | tissue (like a soil sample)

SOFT TISSUE SARCOMAS What determines histologic grade of sarcomas? P466

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

``` SOFT TISSUE SARCOMAS Define the following American Joint Committee for Cancer Staging (AJCC) Sarcoma Stages: Stage I P466 ```

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

``` SOFT TISSUE SARCOMAS Define the following American Joint Committee for Cancer Staging (AJCC) Sarcoma Stages: Stage IIA P466 ```

< 5 cm, grade 2 or grade 3

``` SOFT TISSUE SARCOMAS Define the following American Joint Committee for Cancer Staging (AJCC) Sarcoma Stages: Stage IIB P466 ```

> 5 cm, grade 2

``` SOFT TISSUE SARCOMAS Define the following American Joint Committee for Cancer Staging (AJCC) Sarcoma Stages: Stage III P466 ```

Positive nodes or >5 cm and grade 3

``` SOFT TISSUE SARCOMAS Define the following American Joint Committee for Cancer Staging (AJCC) Sarcoma Stages: Stage IV P466 ```

Distant metastases

SOFT TISSUE SARCOMAS What is a pseudocapsule and what is its importance? P466

``` 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 phenomenon and not just be “shelled-out” like a benign growth ```

SOFT TISSUE SARCOMAS What is the most important factor in the prognosis? P466

Histologic grade of the primary lesion

SOFT TISSUE SARCOMAS What is the treatment? P467

Surgical resection and radiation (with or | without chemotherapy)

SOFT TISSUE SARCOMAS What surgical margins are obtained? P467

2 cm (1 cm minimum)

``` SOFT TISSUE SARCOMAS What is the “limb-sparing” surgery for extremity sarcoma? P467 ```

Avoidance of amputation with local | resection and chemoradiation

SOFT TISSUE SARCOMAS What is the treatment of pulmonary metastasis? P467

Surgical resection for isolated lesions

SOFT TISSUE SARCOMAS What tests should be done in the follow-up? P467

Physical examination, CXR, repeat CT/ MRI of the area of resection to look for recurrence

``` SOFT TISSUE SARCOMAS What syndrome of lymphangiosarcoma arises in chronic lymphedema after axillary dissection for breast cancer? P467 ```

Stewart-Treves syndrome

``` SOFT TISSUE SARCOMAS What syndrome is associated with breast cancer and soft tissue sarcoma? P467 ```

Li-Fraumeni syndrome (p53 tumor | suppressor gene mutation)

LYMPHOMA How is the diagnosis made? P467

Cervical or axillary node excisional biopsy

``` LYMPHOMA What cell type is associated with the histology of Hodgkin’s disease? P467 ```

Reed-Sternberg cells

``` LYMPHOMA What are the four histopathologic types of Hodgkin’s disease? P467 ```

``` 1. Nodular sclerosing (most common; ≈50% of cases) 2. Mixed cellularity 3. Lymphocyte predominant (best prognosis) 4. Lymphocyte depleted ```

``` LYMPHOMA What are the indications for a “staging laparotomy” in Hodgkin’s disease? P467 ```

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

``` LYMPHOMA Define the stages (Ann Arbor) of Hodgkin’s disease: Stage I P468 ```

Single lymph node region (Think: | Stage 1 = 1 region)

``` LYMPHOMA Define the stages (Ann Arbor) of Hodgkin’s disease: Stage II P468 ```

Two or more lymph node regions on the same side of the diaphragm (Think: Stage 2 = >2 regions)

``` LYMPHOMA Define the stages (Ann Arbor) of Hodgkin’s disease: Stage III P468 ```

Involvement on both sides of the | diaphragm

``` LYMPHOMA Define the stages (Ann Arbor) of Hodgkin’s disease: Stage IV P468 ```

Diffuse and/or disseminated involvement

LYMPHOMA What is stage A Hodgkin’s? P468

Asymptomatic (Think: Asymptomatic | stage A)

LYMPHOMA What is stage B Hodgkin’s? P468

Symptomatic: weight loss, fever, night | sweats, etc. (Think: Stage B = Bad)

LYMPHOMA What is the “E” on the staging? P468

Extralymphatic site involvement | E = Extralymphatic

``` LYMPHOMA What treatments are used for low versus advanced stage Hodgkin’s lymphoma? P468 ```

Low stage: radiotherapy | Advanced stage: chemotherapy

``` LYMPHOMA What percentage of patients with Hodgkin’s disease can be cured? P468 ```

≈80%

GI LYMPHOMA What is it? P468

Non-Hodgkin’s lymphoma arising in the | GI tract

GI LYMPHOMA What is the risk factor for gastric lymphoma? P468

Helicobacter pylori

GI LYMPHOMA What are the signs/ symptoms? P468

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

GI LYMPHOMA What is the treatment of intestinal lymphoma? P468

Surgical resection with removal of | draining lymph nodes and chemotherapy

``` GI LYMPHOMA What is the most common site of primary GI tract lymphoma? P468 ```

Stomach (66%) (see Maltoma, p. 281)

Soft Tissue Sarcomas and Lymphomas, C62 P464-468 Flashcards

(63 cards)