Soft Tissue Sarcomas and Lymphomas, C62 P464-468 Flashcards
SOFT TISSUE SARCOMAS
What are they?
P464
Soft tissue tumors, derived from mesoderm
SOFT TISSUE SARCOMAS
Sarcoma means what in
GREEK?
P464
“Fish flesh”
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)
SOFT TISSUE SARCOMAS
How common are they?
P464
0.6% of malignant tumors
SOFT TISSUE SARCOMAS
What is the median age at
diagnosis?
P464
55 years
SOFT TISSUE SARCOMAS
What are the risk factors?
P464
“RALES”: Radiation AIDS (Immunosuppression) Lymphedema Exposure to chemicals Syndromes (e.g., Gardner’s/Li-Fraumeni)
SOFT TISSUE SARCOMAS Name the following types of malignant sarcoma: Fat P464
Liposarcoma
SOFT TISSUE SARCOMAS Name the following types of malignant sarcoma: Gastrointestinal P464
GIST (GastroIntestinal Stromal Tumor)
SOFT TISSUE SARCOMAS Name the following types of malignant sarcoma: Myofibroblast P464
Malignant fibrous histiocytoma
SOFT TISSUE SARCOMAS Name the following types of malignant sarcoma: Striated muscle P464
Rhabdomyosarcoma
SOFT TISSUE SARCOMAS Name the following types of malignant sarcoma: Vascular endothelium P464
Angiosarcoma
SOFT TISSUE SARCOMAS Name the following types of malignant sarcoma: Fibroblast P464
Fibrosarcoma
SOFT TISSUE SARCOMAS Name the following types of malignant sarcoma: Lymph vessel P465
Lymphangiosarcoma
SOFT TISSUE SARCOMAS Name the following types of malignant sarcoma: Peripheral nerve P465
Malignant neurilemmoma or
schwannoma
SOFT TISSUE SARCOMAS Name the following types of malignant sarcoma: AIDS P465
Kaposi’s sarcoma
SOFT TISSUE SARCOMAS Name the following types of malignant sarcoma: Lymphedema P465
Lymphangiosarcoma
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
SOFT TISSUE SARCOMAS
How do most sarcomas
metastasize?
P465
Hematogenously (i.e., via blood)
SOFT TISSUE SARCOMAS What is the most common location and route of metastasis? P465
Lungs via hematogenous route
SOFT TISSUE SARCOMAS
What tests should be done
in the preoperative workup?
P465
CXR, ± chest CT, LFTs
SOFT TISSUE SARCOMAS What are the three most common malignant sarcomas in adults? P465
Fibrous histiocytoma (25%)
Liposarcoma (20%)
Leiomyosarcoma (15%)
SOFT TISSUE SARCOMAS
What are the two most
common in children?
P465
Rhabdomyosarcoma (about 50%),
fibrosarcoma (20%)
SOFT TISSUE SARCOMAS What is the most common type to metastasize to the lymph nodes? P465
Malignant fibrous histiocytoma
SOFT TISSUE SARCOMAS What is the most common sarcoma of the retroperitoneum? P465
Liposarcoma
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
- Differentiation
- Mitotic count
- 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)