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464 Section II / General Surgery

C h a p t e r 62

Soft Tissue

Sarcomas and

Lymphomas

SOFT TISSUE SARCOMAS

What are they?

Sarcoma means what in GREEK?

Sarcomas are more common in upper or lower extremities?

How common are they?

What is the median age at diagnosis?

What are the risk factors?

Name the following types of malignant sarcoma:

Fat

Soft tissue tumors, derived from mesoderm

“Fish flesh”

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

0.6% of malignant tumors

55 years

“RALES”: Radiation

AIDS (Immunosuppression) Lymphedema

Exposure to chemicals

Syndromes (e.g., Gardner’s/Li-Fraumeni)

Liposarcoma

Gastrointestinal

GIST (GastroIntestinal Stromal Tumor)

Myofibroblast

Malignant fibrous histiocytoma

Striated muscle

Rhabdomyosarcoma

Vascular endothelium

Angiosarcoma

Fibroblast

Fibrosarcoma

Chapter 62 / Soft Tissue Sarcomas and Lymphomas 465

Lymph vessel

Lymphangiosarcoma

Peripheral nerve

Malignant neurilemmoma or

 

schwannoma

AIDS

Kaposi’s sarcoma

Lymphedema

Lymphangiosarcoma

What are the signs/

Soft tissue mass; pain from compression

symptoms?

of adjacent structures, often noticed after

 

minor trauma to area of mass

How do most sarcomas

Hematogenously (i.e., via blood)

metastasize?

 

What is the most common

Lungs via hematogenous route

location and route of

 

metastasis?

 

What tests should be done

CXR, chest CT, LFTs

in the preoperative workup?

 

What are the three most

Fibrous histiocytoma (25%)

common malignant sarcomas

Liposarcoma (20%)

in adults?

Leiomyosarcoma (15%)

What are the two most

Rhabdomyosarcoma (about 50%),

common in children?

fibrosarcoma (20%)

What is the most common

Malignant fibrous histiocytoma

type to metastasize to the

 

lymph nodes?

 

What is the most

Liposarcoma

common sarcoma of the

 

retroperitoneum?

 

How do sarcomas locally

Usually along anatomic planes such as

invade?

fascia, vessels, etc.

How is the diagnosis made?

Imaging workup—MRI is superior to

 

CT at distinguishing the tumor from

adjacent structures

Mass 3 cm: excisional biopsy

Mass 3 cm: incisional biopsy or core biopsy

466 Section II / General Surgery

Define excisional biopsy.

Define incisional biopsy.

What is the orientation of incision for incisional biopsy of a suspected extremity sarcoma?

Define core biopsy.

What determines histologic grade of sarcomas?

Define the following American Joint Committee for Cancer Staging (AJCC) Sarcoma Stages:

Stage I

Stage IIA

Stage IIB

Stage III

Stage IV

Biopsy by removing the entire mass

Biopsy by removing a piece of the mass

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

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

1.Differentiation

2.Mitotic count

3.Tumor necrosis

Grade 1 well differentiated Grade 2 moderately differentiated Grade 3 poorly differentiated

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

5 cm, grade 2 or grade 3

5 cm, grade 2

Positive nodes or 5 cm and grade 3

Distant metastases

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

What is the most important

Histologic grade of the primary lesion

factor in the prognosis?

 

Chapter 62 / Soft Tissue Sarcomas and Lymphomas 467

What is the treatment?

What surgical margins are obtained?

What is the “limb-sparing” surgery for extremity sarcoma?

What is the treatment of pulmonary metastasis?

What tests should be done in the follow-up?

What syndrome of lymphangiosarcoma arises in chronic lymphedema after axillary dissection for breast cancer?

Surgical resection and radiation (with or without chemotherapy)

2 cm (1 cm minimum)

Avoidance of amputation with local resection and chemoradiation

Surgical resection for isolated lesions

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

Stewart-Treves syndrome

What syndrome is associated

Li-Fraumeni syndrome (p53 tumor

with breast cancer and soft

suppressor gene mutation)

tissue sarcoma?

 

 

LYMPHOMA

 

 

 

 

How is the diagnosis made?

Cervical or axillary node excisional biopsy

What cell type is associated

Reed-Sternberg cells

with the histology of

 

 

Hodgkin’s disease?

 

 

What are the four

1.

Nodular sclerosing (most common;

histopathologic types

 

50% of cases)

of Hodgkin’s disease?

2.

Mixed cellularity

 

3.

Lymphocyte predominant (best

 

 

prognosis)

 

4.

Lymphocyte depleted

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

468 Section II / General Surgery

 

Define the stages (Ann Arbor)

 

of Hodgkin’s disease:

 

Stage I

Single lymph node region (Think:

 

Stage 1 1 region)

Stage II

Two or more lymph node regions on

 

the same side of the diaphragm

 

(Think: Stage 2 2 regions)

Stage III

Involvement on both sides of the

 

diaphragm

Stage IV

Diffuse and/or disseminated involvement

What is stage A Hodgkin’s?

Asymptomatic (Think: Asymptomatic

 

stage A)

What is stage B Hodgkin’s?

Symptomatic: weight loss, fever, night

 

sweats, etc. (Think: Stage B Bad)

What is the “E” on the

Extralymphatic site involvement

staging?

(E Extralymphatic)

What treatments are used

Low stage: radiotherapy

for low versus advanced

Advanced stage: chemotherapy

stage Hodgkin’s lymphoma?

 

What percentage of patients

80%

with Hodgkin’s disease can

 

be cured?

 

GI LYMPHOMA

 

 

 

What is it?

Non-Hodgkin’s lymphoma arising in the

 

GI tract

What is the risk factor for

Helicobacter pylori

gastric lymphoma?

 

What are the signs/

Abdominal pain, obstruction, GI

symptoms?

hemorrhage, GI tract perforation, fatigue

What is the treatment of

Surgical resection with removal of

intestinal lymphoma?

draining lymph nodes and chemotherapy

What is the most common

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

site of primary GI tract

 

lymphoma?

 

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