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63 Cards in this Set
- Front
- Back
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What are soft tissue sarcomas? |
Soft tissue tumors, derived from mesoderm |
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Sarcoma means what in Greek? |
Fish flesh |
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Sarcomas are more common in upper or lower extremities? |
50% are in the extremities and are 3.5x more common in LOWER extremity (thigh) |
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How common are soft tissue sarcomas? |
0.6% of malignant tumors |
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What is the median age at diagnosis of soft tissue sarcomas? |
55 years |
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What are the risk factors for soft tissue sarcomas? |
RALES: - Radiation - AIDS (immunosuppression) - Lymphedema - Exposure to chemicals - Syndromes (eg, Gardner's / Li-Fraumeni) |
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What is the name of a "fat" malignant sarcoma? |
Liposarcoma |
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What is the name of a "gastrointestinal" malignant sarcoma? |
GIST (Gastro-Intestinal Stromal Tumor) |
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What is the name of a "myofibroblast" malignant sarcoma? |
Malignant Fibrous Histiocytoma |
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What is the name of a "striated muscle" malignant sarcoma? |
Rhabdomyosarcoma |
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What is the name of a "vascular endothelium" malignant sarcoma? |
Angiosarcoma |
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What is the name of a "fibroblast" malignant sarcoma? |
Fibrosarcoma |
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What is the name of a "lymph vessel" malignant sarcoma? |
Lymphangiosarcoma |
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What is the name of a "peripheral nerve" malignant sarcoma? |
Malignant neurilemmoma or schwannoma |
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What is the name of a malignant sarcoma seen in AIDS patients? |
Kaposi's sarcoma |
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What is the name of a "lymphedema" malignant sarcoma? |
Lymphangiosarcoma |
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What are the signs/symptoms of soft tissue sarcomas? |
- Soft tissue mass - Pain from compression of adjacent structures - Often noticed after minor trauma to area of mass |
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How do most sarcomas metastasize? |
Hematogenously (ie, via blood) |
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What is the most common location and route of metastasis of sarcomas? |
Lungs via hematogenous route |
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What tests should be done in the pre-op workup of sarcomas? |
- CXR - +/- CT chest - LFTs |
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What are the three most common malignant sarcomas in adults? |
- Fibrous histiocytoma (25%) - Liposarcoma (20%) - Leiomyosarcoma (15%) |
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What are the two most common sarcomas in children? |
- Rhabdomyosarcoma (about 50%) - Fibrosarcoma (20%) |
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What is the most common type of sarcoma to metastasize to the lymph nodes? |
Malignant fibrous histiocytoma |
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What is the most common sarcoma of the retroperitoneum? |
Liposarcoma |
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How do sarcomas locally invade? |
Usually along anatomic planes such as fascia, vessels, etc |
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How do you diagnose a sarcoma? |
- Imaging workup: MRI > CT (in terms of distinguishing tumor from adjacent structures) - Mass <3 cm: excisional biopsy - Mass >3 cm: incisional biopsy or core biopsy |
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What is an excisional biopsy? |
Biopsy by removing the ENTIRE mass |
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What is an incisional biopsy? |
Biopsy by removing a PIECE of the mass |
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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 |
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What is a core biopsy? |
Large-bore needle that takes a core of tissue (like a soil sample) |
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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 |
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Define the American Joint Committee for Cancer Staging (AJCC) Sarcoma Stage I? |
Well differentiated (grade I), any size, no nodes, no metastases |
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Define the American Joint Committee for Cancer Staging (AJCC) Sarcoma Stage IIA? |
<5 cm, grade 2 or grade 3
Grade 2 = moderately differentiated Grade 3 = poorly differentiated |
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Define the American Joint Committee for Cancer Staging (AJCC) Sarcoma Stage IIB? |
>5 cm, grade 2
Grade 2 = moderately differentiated
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Define the American Joint Committee for Cancer Staging (AJCC) Sarcoma Stage III? |
Positive nodes or >5 cm and grade 3
Grade 3 = poorly differentiated |
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Define the American Joint Committee for Cancer Staging (AJCC) Sarcoma Stage IV? |
Distant metastases
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What is a 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 phenomenon and not just be "shelled out" like a benign growth |
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What is the most important factor in prognosis with sarcomas? |
Histologic grade of primary lesion |
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What is the treatment of sarcomas? |
Surgical resection and radiation (with or without chemotherapy) |
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What surgical margins are obtained with sarcomas? |
2 cm (1 cm minimum) |
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What is the "limb-sparing" surgery for extremity sarcoma? |
Avoidance of amputation with local resection and chemoradiation |
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What is the treatment of pulmonary metastasis from sarcoma? |
Surgical resection for isolated lesions |
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What tests should be done in the follow-up after resection of sarcoma? |
- Physical exam - CXR - Repeat CT/MRI of area of resection to look for recurrence |
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What syndrome of lymphangiosarcoma rises in chronic lymphedema after axillary dissection for breast cancer? |
Stewart-Treves Syndrome |
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What syndrome is associated with breast cancer and soft tissue sarcoma? |
Li-Fraumeni syndrome (p53 tumor suppressor gene mutation) |
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How do you diagnose lymphoma? |
Cervical or axillary node excisional biopsy |
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What cell type is associated with the histology of Hodgkin's disease? |
Reed-Sternberg cells |
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What are the four histopathologic types of Hodgkin's disease? |
1. Nodular sclerosing (most common ~50% of cases) 2. Mixed cellularity 3. Lymphocyte predominant (best prognosis) 4. Lymphocyte depleted |
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What are the indications for a "staging laparotomy" in Hodgkin's disease? |
- Rarely performed - Most experts rely on CT scans, PET scans, bone marrow biopsy, and other directed imaging and biopsies |
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What is the definition of the Ann Arbor Hodgkin's disease Stage I? |
Single lymph node region (I = one region) |
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What is the definition of the Ann Arbor Hodgkin's disease Stage II? |
Two or more lymph node regions on the SAME SIDE of diaphragm (II = two regions) |
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What is the definition of the Ann Arbor Hodgkin's disease Stage III? |
Involvement on BOTH sides of the diaphragm |
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What is the definition of the Ann Arbor Hodgkin's disease Stage IV? |
Diffuse and/or disseminated involvement |
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What is Stage A Hodgkin's disease? |
Asymptomatic |
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What is Stage B Hodgkin's disease? |
Symptomatic: weight loss, fever, night sweats, etc (B = Bad) |
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What ist he "E" on the staging of Hodgkin's disease? |
Extralymphatic site involvement (E = extralymphatic) |
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What treatments are used for low vs advanced stage Hodgkin's lymphoma? |
- Low stage: radiotherapy - Advanced stage: chemotherapy |
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What percentage of patients with Hodgkin's disease can be cured? |
~80% |
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What is a GI lymphoma? |
Non-Hodgkin's lymphoma arising in the GI tract |
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What is the risk factor for gastric lymphoma? |
H. pylori |
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What are the signs/symptoms of GI lymphoma? |
- Abdominal pain - Obstruction - GI hemorrhage - GI tract perforation - Fatigue |
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What is the treatment of intestinal lymphoma? |
Surgical resection with removal of draining lymph nodes and chemotherapy |
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What is the most common site of primary GI tract lymphoma? |
Stomach (66%) (maltoma) |
