Soft Tissue Sarcomas & Lymphomas Flashcards
What are soft tissue sarcomas?
Soft tissue tumors, derived from mesoderm
What does “sarcoma” mean in Greek?
Fish flesh
Are sarcomas more common in upper or lower extremities?
Lower extremities (3.5 fold)
How common are soft tissue sarcomas?
0.6% of malignant tumors
What is the median age of diagnosis of soft tissue sarcoma?
55 years
What are the risk factors for soft tissue sarcoma?
RALES:
Radiation, AIDS, Lymphedema, Exposure (chemicals), Syndromes (Gardner’s, Li-Fraumeni)
What is a malignant sarcoma of fat called?
Liposarcoma
What is a malignant GI sarcoma called?
GIST (GastroIntestinal Stromal Tumor)
What is a malignant sarcoma of myofibroblasts called?
Malignant fibrous histiocytoma
What is a malignant sarcoma of striated muscle called?
Rhabdomyosarcoma
What is a malignant sarcoma of vascular endothelium called?
Angiosarcoma
What is a malignant sarcoma of fibroblasts called?
Fibrosarcoma
What is a malignant sarcoma of lymph vessels called?
Lymphangiosarcoma
What is a malignant sarcoma of peripheral nerves called?
Malignant neurilemmoma or schwannoma
What is a malignant sarcoma seen in AIDS?
Kaposi’s sarcoma
What is a malignant sarcoma associated with lymphedema?
Lymphangiosarcoma
What are the signs and symptoms of soft tissue sarcoma?
Soft tissue mass; pain from compression of adjacent structures
How do most sarcomas metastasize?
Hematogenously
What is the most common location and route of metastasis of sarcoma?
Lung via hematogenous route
What tests should be done in the preoperative workup?
CXR, +/- chest CT, LFTs
What are the 3 most common malignant sarcomas in adults?
Fibrous histiocytoma (25%), liposarcoma (20%), leiomyosarcoma (15%)
What are the 2 most common malignant sarcomas in children?
Rhabdomyosarcoma (50%), fibrosarcoma (20%)
What is the most common type of sarcoma to metastasize to the lymph nodes?
Malignant fibrous histiocytoma
What is the most common sarcoma of the retroperitoneum?
Liposarcoma
How do sarcomas locally invade?
Usually along anatomic planes such as fascia, vessels
How is the diagnosis of sarcoma made?
Imaging (MRI > CT)
Mass 3 cm: incisional biopsy or core biopsy
What is an excisional biopsy?
Biopsy by removing the entire mass
What is an incisional biopsy?
Biopsy by removing a piece of the mass
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
What is a core biopsy?
Large-bore needle that takes a core of tissue
What determines histologic grade of sarcomas?
- Differentiation
- Mitotic count
- Tumor necrosis
Grade 1 = well-differentiated
Grade 2 = moderately differentiated
Grade 3 = poorly differentiated
What is stage I sarcoma?
Well-differentiated (grade 1), any size, no nodes, no metastases
What is stage IIA sarcoma?
What is stage IIB sarcoma?
> 5 cm, grade 2
What is stage III sarcoma?
Positive nodes or > 5 cm and grade 3
What is stage IV sarcoma?
Distant metastases
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
What is the most important factor in the prognosis for sarcoma?
Histologic grade of the primary lesion
What is the treatment for sarcoma?
Surgical resection and radiation +/- chemo
What surgical margins are obtained in sarcoma resection?
2 cm (1 cm minimum)
What is the limb-sparing surgery for extremity sarcoma?
Avoidance of amputation with local resection and chemoradiation
What is the treatment of pulmonary metastasis of sarcoma?
Surgical resection for isolated lesions
What tests should be done in the followup for sarcoma resection?
PE, CXR, repeat CT/MRI
What syndrome of lymphangiosarcoma arises in chronic lymphedema after axillary dissection for breast cancer?
Stewart-Treves syndrome
What syndrome is associated with breast cancer and soft tissue sarcoma?
Li-Fraumeni syndrome (p53 tumor suppressor gene mutation)
How is the diagnosis of lymphoma made?
Cervical or axillary node excisional biopsy
What cell type is associated with the histology of Hodgkin’s lymphoma?
Reed-Sternberg cells
What are the 4 histopathologic types of Hodgkin’s lymphoma?
- Nodular sclerosing
- Mixed cellularity
- Lymphocyte predominant
- Lymphocyte depleted
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
What is stage I Hodgkin’s lymphoma?
Single lymph node region
What is stage II Hodgkin’s lymphoma?
Two or more lymph node regions on the same side of the diaphragm
What is stage III Hodgkin’s lymphoma?
Involvement on both sides of the diaphragm
What is stage IV Hodgkin’s lymphoma?
Diffuse and/or disseminated involvement
What is stage A Hodgkin’s lymphoma?
Asymptomatic
What is stage B Hodgkin’s lymphoma?
Symptomatic (e.g. weight loss, fever, night sweats)
What treatments are used for low vs. advanced stage Hodgkin’s lymphoma?
Low stage: Radiotherapy
Advanced: Chemotherapy
What percentage of patients with Hodgkin’s lymphoma can be cured?
80%
What is GI lymphoma?
Non-Hodgkin’s lymphoma arising in the GI tract
What is the risk factor for gastric lymphoma?
H. pylori infection
What are the signs and symptoms of GI lymphoma?
Abdominal pain, obstruction, GI hemorrhage, GI tract perforation, fatigue
What is the treatment of intestinal lymphoma?
Surgical resection with removal of draining lymph nodes and chemotherapy
What is the most common site of primary GI tract lymphoma?
Stomach (MALToma)
