The Society of Surgical Oncology surgical practice guidelines focus
on the signs and symptoms of primary cancer, timely evaluation of the symptomatic
patient, appropriate preoperative evaluation for extent of disease, and
role of the surgeon in diagnosis and treatment. Separate sections on adjuvant
therapy, follow-up programs, or management of recurrent cancer have been
intentionally omitted. Where appropriate, perioperative adjuvant combined-modality
therapy is discussed under surgical management. Each guideline is presented
in minimal outline form as a delineation of therapeutic options.
Since the development of treatment protocols was not the specific aim of
the Society, the extensive development cycle necessary to produce evidence-based
practice guidelines did not apply. We used the broad clinical experience
residing in the membership of the Society, under the direction of Alfred
M. Cohen, MD, Chief, Colorectal Service, Memorial Sloan-Kettering Cancer
Center, to produce guidelines that were not likely to result in significant
Following each guideline is a brief narrative highlighting and expanding
on selected sections of the guideline document, with a few relevant references.
The current staging system for the site and approximate 5-year survival
data are also included.
The Society does not suggest that these guidelines replace good medical
judgment. That always comes first. We do believe that the family physician,
as well as the health maintenance organization director, will appreciate
the provision of these guidelines as a reference for better patient care.
Symptoms and Signs
- Painless mass growing for a variable length of time
- Mass after an injury that does not rapidly resolve
Evaluation of the Symptomatic Patient
- History and physical examination
- Comprehensive history and physical examinationpersonal or family history
of malignancy or genetic disease
- Nature and duration of symptoms
- Prior exposure to chemical toxins or radiation
- Assess the mobility of the mass vs fixity to deep structures.
- Assess the size, depth, and consistency of the mass.
- Ascertain clinical involvement of overlying skin or regional lymph
- Examine motor and sensory nerve function.
- Examine for swelling distal to the mass or for any ecchymosesoverlying
- Radiologic and laboratory studies
- Difficult to evaluate on physical examination. However, flank bulging
or mass effect on deep abdominal palpation should be evaluated further.
- Pelvic or rectal examination may raise the possibility of anunderlying
- Radiologic and laboratory studies
- Radiologic studies should be performed prior to biopsy.
- CTpelvis, abdomen, retroperitoneum
- Celiac axis arteriogram in selected patients with visceral sarcoma
- CBC, chemistry profile, and chest x-ray
- Incision oriented parallel to the long axis of extremity
- Core needle biopsy for superficial lesions
- Needle aspiration if cytopathology expertise available
- Excisional biopsy is reserved for extremity lesions £
- Appropriate timeliness of surgical referral
- Soft-tissue sarcoma can grow rapidly and disseminate: therefore, the
work-up and biopsy should be performed expeditously in the presence of
the above symptoms or signs.
Preoperative Evaluation for Extent of Disease
- Physical examination
- Chest CT
- Obtain chest CT in addition to primary tumor imaging and routine studies
in patients with lesions > 5 cm, grade II or III.
- CT scan of the chest, abdomen, and pelvis in selected patients
- Myxoid liposarcomas ³ 5 cm have tendency
to metastasize to themediastinum, root of the mesentery, and retroperitoneum.
- CT scan of the head in selected patients
- Alveolar soft-part sarcoma may metastasize selectively to the cen
tral nervous system.
- Liver ultrasound or abdominal CT
- Patients with intra-abdominal, genitourinary, or retroperitoneal leiomyosarcoma
have a marked tendency to develop liver metastases.
- As discussed above
Role of the Surgeon in Initial Management
- Preoperative evaluation
- The initial evaluation and diagnostic work-up should be coordinated
by the surgeon who will perform the definitive operation.
- Radiotherapy and chemotherapy treatments should be considered only
after the diagnostic work-up is completed.
- Surgical considerations
- Surgeon must be able to perform wide local excisions in combination
with interstitial or postoperative radiotherapy.Accurate intraoperative
surgical field delineation is critical.
- In patients who have received preoperative radiotherapy, the sarcoma
surgeon must be capable of resecting disease and managing wounds in an
- Radical amputation in selected patients with extremity sarcoma
- Radical multiorgan/vascular/orthopedic resections for selected in tra-abdominal/retroperitoneal
These guidelines are copyrighted by the Society of Surgical Oncology
(SSO). All rights reserved. These guidelines may not be reproduced in any
form without the express written permission of SSO. Requests for reprints
should be sent to: James R. Slawny, Executive Director, Society of Surgical
Oncology, 85 West Algonquin Road, Arlington Heights, IL 60005.
Soft-tissue sarcomas are tumors of putative mesodermal origin that
develop in the extraskeletal connective tissues. These tumors constitute
< 1% of adult malignancies and approximately 7% of pediatric malignancies,
and have an annual age-adjusted incidence of 2 cases per 100,000 population.
Approximately 6,300 new cases of soft-tissue sarcoma and 3,100 deaths are
reported annually in the United States.
Sarcomas occur most commonly in the extremity in adults and in the head
and neck in children. Overall, approximately 50% of soft-tissue sarcomas
occur in the extremities, 10% in the head and neck, and 40% in the trunk
There is little evidence to suggest a genetic predilection to the development
of soft-tissue sarcomas other than in Li-Fraumeni kindreds, in whom p53
tumor-suppressor gene mutations also create a predisposition to breast
carcinoma and a variety of other malignancies. Soft-tissue sarcomas also
occur with greater frequency in patients with several other genetic diseases,
including basal cell nevus syndrome, tuberous sclerosis, Werner's syndrome,
Gardner's syndrome, and von Recklinghausen's disease (patients with von
Recklinghausen's disease, for example, have a 10% likelihood of ultimately
developing a neurofibrosarcoma).
Environmental factors have not been convincingly demonstrated as relevant
to the etiology of soft-tissue sarcoma in humans. However, 3-methylcholanthrene
and several viruses have been implicated as etiologic factors in experimental
animal studies. There is no demonstrable connection between retained foreign
bodies or antecedent trauma and the subsequent development of soft-tissue
sarcoma, although previous exposure to ionizing radiation and chronic lymphedema
are predisposing factors. In this latter context, however, the most
common histology is extraskeletal osteosarcoma followed by malignant fibrous
Sarcomas are classified by histology as well as grade. The histologic
classification seeks to ascribe a putative cell of origin for the tumor.
Overall, malignant fibrous histiocytoma is the most common subtype (40%
of the total), followed by liposarcoma (25%).
Grade assignment is made on the basis of the number of mitoses per high-powered
microscopic field, nuclear morphology, degree of cellularity, cellular
anaplasia, degree of necrosis, or other criteria that vary from center
to center. Unfortunately, light microscopic determination of histopathology
and grade are imprecise, and discordancy rates as high as 40% have been
observed prospectively, even among expert sarcoma pathologists.
The American Joint Committee on Cancer (AJCC) staging system (Table
1) is most commonly applied in soft-tissue sarcoma for prognostic and
therapeutic purposes, although alternative staging systems have been proposed,
including those of the Musculoskeletal Tumor Society and the Memorial Sloan-Kettering
Cancer Center. The AJCC system uses the four criteria of tumor, nodal status,
grade, and metastasis (TNGM) to assign one of four disease stages.
Overall, tumor size and grade are the most important determinants of
prognosis. About two-thirds of all sarcomas are high-grade lesions.
Newer information suggests that the prognostic risk for disseminated disease
is most dependent on the histopathologic grade in the first 2 or 3 years,
but after that the size of the initial lesion becomes at least as important
as grade. The overall incidence of nodal metastasis is < 3% and is therefore
minimally relevant to prognosis in most patients.
Stage I tumors are low-grade lesions of any size and are generally treated
by wide local excision. The addition of radiotherapy is dictated by tumor
size and anatomic constraints to a resection with negative margins.
Stage II tumors are intermediate-grade tumors of any size that are usually
treated by surgery in combination with radiotherapy. Use of chemotherapy
is generally reserved for stage II patients with recurrent disease or patients
who need tumor cytoreduction prior to resection with limb sal
Stage III lesions are high-grade lesions of any size that have not developed
regional or distant metastases. High-grade tumors > 5 cm diameter have
the greatest tendency to metastasize and are treated by surgery in conjunction
with radiotherapy. Many patients with these tumors are also eligible for
prospective clinical trials of neoadjuvant or adjuvant multidrug chemotherapy.[7,8]
The two drugs with the greatest activity in soft-tissue sarcoma are
doxorubicin and ifosfamide (Ifex). However, overall response rates in the
best multimodality protocols are only 40% to 60%. Toxicities of chemotherapy
are significant and include permanent cardiac muscle damage, transient
urothelial injury, and temporary, although potentially life-threatening,
myelosuppression.[7,8] Because of these serious toxicities, systemic chemotherapy
for soft-tissue sarcoma should be administered only in prospective trials,
in which strict criteria for enrollment and follow-up monitoring can be
Chemotherapy is the mainstay of treatment for stage IV disease (ie,
soft-tissue sarcomas with regional or metastatic spread). In stage IV disease,
surgery or radiotherapy is usually used solely for palliation. Surgery
with curative intent is generally impossible in patients with stage IV
disease; the only exception is pulmonary metastasis, for which curative
metastatasectomy is possible in selected patients undergoing complete resection
of the primary tumor.
Radiotherapy can be administered by external beam preoperatively
or postoperatively or, alternatively, as a postoperative interstitial
implant (brachytherapy). These various radiotherapy approaches have
never been directly compared in prospective, randomized trials.
Preoperative radiotherapy requires approximately 50 Gy to achieve tumor
control, which is comparable to the 65-Gy dose required for local control
if radiotherapy is given postoperatively. The increased postoperative dose
is necessitated by the hypoxic postoperative wound environment, which also
mandates a wider radiotherapy field extending beyond the area of surgical
resection. However, postoperative radiotherapy avoids the significant wound
healing problems of preoperative radiation, the incidence of which approaches
30% in some series.
Brachytherapy results in local control rates comparable to either preoperative
or postoperative external-beam radiotherapy (7% to 15% rate of local recurrence
for surgery with definitive radiotherapy in most series). In addition,
brachytherapy is usually much more convenient for patients and appears
to be less expensive. Unlike external-beam radiation, there is minimal
scatter radiation with brachytherapy, making this technique optimal for
tumors near open epiphyseal plates, gonads, and other structures particularly
vulnerable to radiation toxicity.
Soft-tissue sarcomas of the retroperitoneum pose a special challenge
because they frequently grow to a large size before causing symptoms. Consequently,
resection can be difficult (50% to 60% rate of resectability in most series).
Central anatomic tumor involvement of the celiac axis, root of the mesentery,
great vessels, or porta hepatis usually precludes resection.
The liver and lungs are equally frequent sites of metastasis from primary
retroperitoneal sarcomas. Complete surgical resection is the primary determinant
of survival in patients with hepatic metastases; however, hepatic metastasectomy
has not been proven to be of value.
In the retroperitoneum, no prospective randomized trials have demonstrated
improved overall survival using chemotherapy and/or radiotherapy adjuvantly
or neoadjuvantly, although intraoperative radiotherapy may decrease local
Survival in soft-tissue sarcomas is driven by the reality that almost
80% of metastases are to the lungs and that these usually occur within
2 to 3 years of initial diagnosis. A 30% survival rate at 3 years can be
anticipated if the pulmonary metastases are resectable.
Size, grade, surgical margins, depth of tumor, anatomic location, and
previous surgery are all important determinants of outcome. However, these
factors have a variable impact on specific survival measures, such as overall
survival, disease-free survival, local recurrence, and distant disease-free
survival; ie, the prognostic factors predictive of local recurrence differ
from those predictive of distant metastases and overall survival.
Unlike some tumor systems, local recurrence of soft-tissue sarcoma is
not necessarily a harbinger of uncontrollable distant failure. Therefore,
local recurrence should be treated aggressively to preserve the highest
quality of life for the patient. Long-term follow-up is required because
late recurrence is noted in up to 10% of patients after 5 years.
Patient-based outcomes research, which differs from physician-defined
survival research, is still in its infancy for soft-tissue sarcoma. In
the future, treatments will be assessed for their impact on survival, cost,
and effect on quality of life as a means to maximize overall efficacy.
These latter considerations will appropriately assume increasing importance
as the percentage of soft-tissue sarcoma patients achieving long-term cure
increases beyond the current 50% plateau level.
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