Soft Tissue Sarcoma

Author: Patrick O'Donnell

Topic updated on 06/09/13 6:32pm

Introduction

Soft tissue sarcomas are rare
- 7000 new case/year in the United States
Proper evaluation is critical
- unplanned excision of a soft-tissue sarcoma may negatively impact patient outcome
- delay in diagnosis can adversely affect ultimate outcome
Metastasis
- lung is most common site of metastasis
Prognosis
- poor prognostic factors include
  - high grade
  - size > 5 cm
  - tumor location below the deep fascia

Classification

Over 50 histological types of soft tissue sarcomas exist
- previously tested soft tissue sarcomas include
  - synovial sarcoma
  - liposarcoma
  - rhabdomyosarcoma
  - fibrosarcoma
  - leiomyosarcoma
  - angiosarcoma

Presentation

Symptoms
- enlarging painless mass
Physical exam
- palpable soft tissue mass
  - differentiation of whether mass is above or below fascia has prognostic importance
  - size greater than 5cm in cross-section is a poor prognostic factor

Imaging

Radiographs
- obtain plain radiographs in two planes
MRI
- is mandatory to evaluate soft tissue lesions in the extremities and determine treatment algorithm
- diagnostic MRI
  - MRI is typically diagnostic for the following benign lesions
    - lipoma
    - neurilemoma (schwanoma)
    - intramuscular myxoma
  - if MRI is diagnostic and the mass is benign and symptomatic, then it can be removed without a biopsy
- indeterminate MRI
  - if MRI is indeterminate or suggestive of sarcoma, then a core needle or open biopsy must be obtained before further treatment is initiated
  - soft tissue sarcomas can look similiar to hematomas so be cautious of a "hematoma" which occurs without trauma

Evaluation

Histology
- detemined by type of sarcoma
  - synovial sarcoma
  - liposarcoma
  - rhabdomyosarcoma
  - fibrosarcoma

Treatment

Treatment overview
- treatment must be based on a tissue diagnosis unless images are diagnostic (e.g. lipoma)
Operative
- radiation therapy & wide surgical resection
  - indications
    - standard of care in most cases
  - radiation therapy
    - an important adjunct to surgery decreasing local recurrence
    - 50-60 Gy is the standard dose for soft-tissue sarcomas
    - radiotherapy may be given pre- or post-operatively
      - pre-operative radiotherapy is associated with a 30% risk for wound complications
      - post-operative radiotherapy is associated with greater radiation induced morbidity and an increased risk for radiation induced sarcoma
  - chemotherapy
    - is controversial for soft tissue sarcomas
  - surgical resection
    - must confirm border free of disease with histology

Complications

Recurrence
- following resection the most common location for recurrence of a low grade, soft tissue sarcoma is locally

Please Rate Educational Value!

4.0

Average 4.0 of 4 Ratings

Qbank (9 Questions)

TAG

(OBQ11.234) A 56-year-old male undergoes resection of a mass that was suspected to be a simple lipoma. The mass was contained within his sartorius muscle and shown in Figure A. The final pathology came back as a high grade soft tissue sarcoma. The treating surgeon operated completely within the sartorius compartment and did not violate any other compartments, however the final pathology showed positive margins. Staging studies show isolated disease in his right leg. What is the most appropriate treatment for this patient? Topic

Review Topic

FIGURES: A

1. Amputation
2. Systemic chemotherapy
3. Radiation therapy
4. Wide excision of the tumor bed
5. Radiation therapy and wide excision of the tumor bed

PREFERRED RESPONSE ▶

DISCUSSION: Incompletely excised soft tissue sarcomas present special treatment dilemmas for the orthopaedic oncologist. As such, any DEEP lesion requires advanced imaging prior to surgical excision. Because of the complex muscle planes and anatomy, deep lesions which are incompletely excised can make limb-salvage very difficult . Superficial lesions may be removed based on the treating surgeon's judgement, however they need to be removed without violation of the superficial fascia.

Potter et al review the rates of local recurrence after unplanned incomplete excision of high-grade soft tissue sarcomas. With appropriate radiation therapy and negative margin tumor bed re-excision, these patients have nearly the same rates of local recurrence as all other negative margin patients. They do however require more morbid surgical excision to achieve these negative margins.

Incorrect answers:
1) Amputation - without contamination of the femoral vessels, the fascia deep to sartorius will serve as a good boundary for the secondary tumor bed excision
2) Systemic chemotherapy isn't indicated for local control in soft tissue sarcoma
3&4) Given the positive margins of the initial excision and the close proximity to the femoral vessels, radiation therapy can be used, but only in combination with tumor bed re-excision.

REFERENCES:

1. Potter BK, Adams SC, Pitcher JD Jr, Temple HT. Local recurrence of disease after unplanned excisions of high-grade soft tissue sarcomas. Clin Orthop Relat Res. 2008 Dec;466(12):3093-100. Epub 2008 Sep 26. PMID:18818981 (Link to Abstract)

Question Authors:

Patrick O'Donnell MD/PhD, John Badylak MD, Ben Taylor MD,

Please Rate Educational Value!

5.0

Average 5.0 of 7 Ratings

TAG

(OBQ09.69) A 24-year-old male presents to your office with ecchymosis and a mass in the posterolateral aspect of his thigh. He states he noticed the mass approximately 6-months ago and that it has slowly been growing. He denies constitutional symptoms, is otherwise healthy, and denies any associated trauma. MRI report suggests the mass may be an organizing hematoma. Core needle biopsy in the office yields only blood. What is the next appropriate treatment option? Topic

Review Topic

1. Conservative treatment - rest, ice, compression, elevation
2. Repeat core needle biopsy
3. Consult interventional radiography to place a drain under image guidance
4. Irrigation and debridement with initiation of postoperative antibiotics
5. Open surgical biopsy

PREFERRED RESPONSE ▶

DISCUSSION: The history is concerning for a soft tissue sarcoma. Many sarcomas can bleed into the surrounding tissues or pseudocapsule making diagnosis difficult. Without a history of injury, trauma, or bleeding diathesis, the diagnosis of a hematoma must be entertained only after ruling out malignant causes for the symptoms. Given this patient's presentation with a slow growing mass, a negative core needle biopsy does not exclude a sarcoma. As such, open surgical biopsy is the treatment of choice. In a retrospective review, Ward et al showed a 7 month average delay in the 33 patients where a soft tissue sarcoma was misdiagnosed as a hematoma. These authors argue that the presence of sub-cutaneous ecchymosis is a finding which may help treating physicians make the difference between hematomas and soft tissue sarcomas, however, this point has not been supported in the literature.

REFERENCES:

1. Ward WG Sr, Rougraff B, Quinn R, Damron T, O'Connor MI, Turcotte RE, Cline M. Tumors masquerading as hematomas. Clin Orthop Relat Res. 2007 Dec;465:232-40 PMID:17828027 (Link to Abstract)

Question Authors:

Patrick O'Donnell MD/PhD,

Please Rate Educational Value!

2.0

Average 2.0 of 14 Ratings

TAG

(OBQ06.225) A surgeon resects a small, superficial, mobile lesion from the thigh. Pathological review of the specimen suggests it is a low-grade soft tissue sarcoma with positive surgical margins. After staging the patient, no other sites of disease are identified. What is the next most appropriate treatment for this patient? Topic

Review Topic

1. Observation
2. Systemic chemotherapy
3. Radiotherapy
4. Tumor bed re-excision
5. Amputation

PREFERRED RESPONSE ▶

DISCUSSION: Accidental excision of soft tissue sarcomas is a common reason for referral to a musculoskeletal oncologist. If such accidental excisions are accompanied with negative surgical margins, observation vs. adjuvant radiotherapy is the appropriate therapy. As positive margin resections in this instance leave residual disease within the patient, tumor bed re-excision to attain negative surgical margins is necessary. Amputation for superfical incompletely excised sarcomas usually isn't necessary.

Rougraff et al performed a retrospective review of 46 of their patients with high-grade, nonmetastatic soft tissue sarcomas. Treatment with local wide-margin excision and adjuvant radiotherapy resulted in approximately a 60% survival rate at a mean of 50 months. Surgical resection was accoplished using limb-salvage in 91% of cases.

REFERENCES:

1. Rougraff B, Sandler A. Karnofsky performance score as a predictor of survival in soft tissue sarcoma. Clin Orthop Relat Res. 2002 Apr;(397):196-203. PMID:11953611 (Link to Abstract)

Question Authors:

Patrick O'Donnell MD/PhD, Patrick McCulloch MD, John Badylak MD,

Please Rate Educational Value!

3.0

Average 3.0 of 14 Ratings

TAG

(OBQ06.248) Which treatment factor has shown to be most important for five-year disease-free survival in patients with subcutaneous sarcomas? Topic

Review Topic

1. dose of chemotherapy
2. addition of adjuvant radiation
3. size of operative margin
4. length of chemotherapy course
5. addition of radiofrequency ablation

PREFERRED RESPONSE ▶

DISCUSSION: Basically, there are studies which show microscopic residual tumor when a marginal resection is done. The size of the operative margin is the most important treatment-related factor. If the proper treatment was not done initially for a subcutaneous high-grade sarcoma the patient should be taken back to provide adequate margins. The authors also concluded that size is the most important tumor-related factor.

REFERENCES:

1. Gibbs CP, Peabody TD, Mundt AJ, Montag AG, Simon MA. Oncological outcomes of operative treatment of subcutaneous soft-tissue sarcomas of the extremities. J Bone Joint Surg Am. 1997 Jun;79(6):888-97 PMID:9199387 (Link to Abstract)

Question Authors:

Greg Galano MD,

Please Rate Educational Value!

4.0

Average 4.0 of 4 Ratings

TAG

(OBQ05.231) Sixty Gy is a typical radiation dose for which of the following indications? Topic

Review Topic

1. Prevention of post-operative heterotopic ossification
2. Neoadjuvant radiation therapy for a high-grade chondrosarcoma
3. Adjuvant radiation therapy for a high-grade osteosarcoma
4. Adjuvant radiation therapy for a soft-tissue sarcoma
5. Palliative therapy for a painful adenocarcinoma metastasis in the thoracic spine

PREFERRED RESPONSE ▶

DISCUSSION: External beam radiotherapy plays a major role in the treatment of soft-tissue sarcomas. Radiotherapy dose depends on the indication for its use. Prevention of heterotopic ossification typically requires 600cGy. Treatment of metastatic lesions requires 25-30 Gy. As a general rule, radiation therapy does not have an indication for osteosarcoma or chondrosarcoma. Finally, 60 Gy is a general dose given for the treatment of soft-tissue sarcoma.

REFERENCES:

1. Orthopaedic Knowledge Update. Musculoskeletal Oncology. Schwartz, H (ed). American Academy of Orthopaedic Surgeons. Rosemont, IL. 2005.

Question Authors:

Patrick O'Donnell MD/PhD, John Badylak MD,

Please Rate Educational Value!

2.0

Average 2.0 of 30 Ratings

TAG

(OBQ04.106) A 52-year-old male presents with 6 months of swelling and pain in his leg. He states the lesion has not changed in size for several months and doesn't bother him. He is otherwise healthy and has no other complaints. Representative photograph and MRI are shown in Figures A through C. What is the best initial step in his management? Topic

Review Topic

FIGURES: A

1. Follow-up in 6 months with repeat radiographs
2. Biopsy
3. Marginal excision
4. Radiation therapy
5. Neoadjuvant chemotherapy and wide excision

PREFERRED RESPONSE ▶

DISCUSSION: The initial step in management of a patient presenting with a large soft tissue mass deep to the fascia is to obtain a tissue diagnosis, via biopsy. Initiation of treatment prior to tissue diagnosis is inappropriate and can result in significant patient morbidity and potential mortality. While some soft tissue sarcomas may benefit from chemotherapy, this cannot be initiated prior to diagnosis. Radiation therapy is used in the treatment of soft tissue sarcoma, but again, only after tissue diagnosis. Peabody et al review the appropriate evaluation and staging for musculoskeletal neoplasms and present flow-charts useful in the work-up of bone (Illustration A) and soft tissue (Illustration B) neoplasms. Skrzynski et al performed a comparison of outpatient core-needle biopsy with open surgical biopsy for musculoskeletal tumors. They found the diagnostic accuracy of core-needle biopsy was only 84% with many samples yielding no or different histological samples when compared to final resected specimens. They conclude that while core-needle biopsy is significantly less expensive than surgical biopsy ($1106 vs. $7234), there is higher concern for sampling error or general diagnostic inaccuracy associated with a core-needle biopsy compared to open biopsy, the "gold standard".

Illustrations: A

REFERENCES:

1. Peabody TD, Gibbs CP Jr, Simon MA. Evaluation and staging of musculoskeletal neoplasms. J Bone Joint Surg Am. 1998 Aug;80(8):1204-18. Review. PMID:9730132 (Link to Abstract)

2. Skrzynski MC, Biermann JS, Montag A, Simon MA. Diagnostic accuracy and charge-savings of outpatient core needle biopsy compared with open biopsy of musculoskeletal tumors. J Bone Joint Surg Am. 1996 May;78(5):644-9. PMID:8642019 (Link to Abstract)

Question Authors:

Patrick O'Donnell MD/PhD,

Please Rate Educational Value!

3.0

Average 3.0 of 7 Ratings

TAG

(OBQ04.184) What is the correct treatment for an incompletely excised high grade sarcoma on the volar aspect of the forearm? Topic

Review Topic

1. Chemotherapy
2. Radiation therapy
3. Marginal resection of the tumor bed to spare the neurovascular bundles
4. Wide resection of the tumor bed and coverage as necessary
5. Palliative care

PREFERRED RESPONSE ▶

DISCUSSION: The correct treatment for an incompletely excised high grade sarcoma on the volar aspect of the forearm is wide resection of the tumor bed and coverage as needed. It is always important to gain appropriate imaging, biopsy, and staging of a patient with a mass to correctly excise the tumor during the index surgery. Incomplete excision requiring tumor bed wide excision is a morbid procedure that potentially may affect the patients survival. Davis et al review 239 patient with "unplanned excision" of high-grade sarcomas and showed that positive tumor at the index surgery correlated with increased risk of local recurrence. Noria et al retrospectively reviewed the charts of 65 patients to determine the prevalence of residual tumor after unplanned excision and to identify factors that predict which patients will have residual tumor following such an excision. All sixty-five patients had a repeat resection of the wound bed and histological evidence of sarcoma was identified in a total of twenty-three (35 percent) patients. Even after the second excision, the rate of local recurrence was significantly higher than other patients treated at this cancer center. These data indicate that it is not possible to predict which patients will have residual tumor at the site of the operative wound and therefore repeat excision is advised.

REFERENCES:

1. Davis AM, Kandel RA, Wunder JS, Unger R, Meer J, O'Sullivan B, Catton CN, Bell RS. The impact of residual disease on local recurrence in patients treated by initial unplanned resection for soft tissue sarcoma of the extremity. J Surg Oncol. 1997 Oct;66(2):81-7. PMID:9354161 (Link to Abstract)

2. Noria S, Davis A, Kandel R, Levesque J, O'Sullivan B, Wunder J, Bell R. Residual disease following unplanned excision of soft-tissue sarcoma of an extremity. J Bone Joint Surg Am. 1996 May;78(5):650-5. PMID:8642020 (Link to Abstract)

Question Authors:

Patrick O'Donnell MD/PhD,

Please Rate Educational Value!

3.0

Average 3.0 of 11 Ratings

TAG

(OBQ04.260) A 39-year-old male is diagnosed with myxoid liposarcoma of the thigh. Radiation therapy is being considered in adjunct to surgical excision. Which of the following is true regarding radiation therapy in soft tissue sarcoma? Topic

Review Topic

1. Compared to post-operative radiation, pre-operative radiation is associated with a reduced rate of wound dehiscence
2. Radiation therapy reduces the risk of local recurrence
3. Radiation therapy improves overall survival
4. Compared to post-operative radiation, pre-operative radiation is associated with increased rates of radiation induced morbidity such as post-radiation fibrosis
5. Radiation therapy decreases the chances of successful limb-salvage

PREFERRED RESPONSE ▶

DISCUSSION: Adjuvant radiation therapy in extremity soft tissue sarcomas decreases the rate of local recurrence. In a level 1 study by Yang et al, patients with soft tissue sarcomas of the extremity were randomized to receive or not receive radiation therapy following excision. No difference in overall survival rate was reported. The group receiving radiation had a lower rate of local recurrence, but also had transient loss of strength and range of motion. In another level 1 study by O'Sullivan et al, patients with soft tissue sarcomas of the extremity were randomized to pre-operative or post-operative radiation therapy. While the rate of local recurrence was not significantly different between those patients receiving pre-operative vs post-operative radiotherapy, 30% of patients who received pre-operative radiotherapy experienced significant wound complications. The incidence of radiation induced morbidity and the concern for radiation induced sarcoma was significantly greater in the post-operative radiotherapy group.

REFERENCES:

1. Yang JC, Chang AE, Baker AR, Sindelar WF, Danforth DN, Topalian SL, DeLaney T, Glatstein E, Steinberg SM, Merino MJ, Rosenberg SA. Randomized prospective study of the benefit of adjuvant radiation therapy in the treatment of soft tissue sarcomas of the extremity. J Clin Oncol. 1998 Jan;16(1):197-203. PMID:9440743 (Link to Abstract)

2. O'Sullivan B, Davis AM, Turcotte R, Bell R, Catton C, Chabot P, Wunder J, Kandel R, Goddard K, Sadura A, Pater J, Zee B. Preoperative versus postoperative radiotherapy in soft-tissue sarcoma of the limbs: a randomised trial. Lancet. 2002 Jun 29;359(9325):2235-41. PMID:12103287 (Link to Abstract)

Question Authors:

John Badylak MD,

Please Rate Educational Value!

3.0

Average 3.0 of 9 Ratings

Cases

	Soft tissue sarcoma encroaching on brachial artery and radial/ulnar/median nerves! (C1163) Pathology - - Soft Tissue Sarcoma HPI - 72-year-old male presented to clinic with a 3 month history of arm swelling. No... What treatment do you propose?	3/6/2012 41 responses
	Shoulder Tumor (C1282) Pathology - Treatment Consult - Soft Tissue Sarcoma HPI - 67 yr-old lady with presents with 7 month history of pain and swelling over the... How would you treat this patient?	10/4/2012 90 responses

See More Cases

Posts

Preoperative versus postoperative radiotherapy in soft-tissue sarcoma of the limbs: a randomised trial. Includes Poll

O'Sullivan B, Davis AM, Turcotte R, Bell R, Catton C, Chabot P, Wunder J, Kandel R, Goddard K, Sadura A, Pater J, Zee B
Lancet. 2002 Jun;359(9325):2235-41. PMID: 12103287 (Link to Pubmed)

Pathology - Journal Club - Soft Tissue Sarcoma

5/13/2013

16 responses

See More Posts

Groups

ABOS II: Sarcoma

General - Oral Boards Review

7/29/2011

0 responses

See More Groups

Evidence & References Show References

Level of Evidence 1 (Randomized Control Trial)

O'Sullivan B, Davis AM, Turcotte R, Bell R, Catton C, Chabot P, Wunder J, Kandel R, Goddard K, Sadura A, Pater J, Zee B. Preoperative versus postoperative radiotherapy in soft-tissue sarcoma of the limbs: a randomised trial. Lancet. 2002 Jun 29;359(9325):2235-41. PMID:12103287 (Link to Abstract)
Yang JC, Chang AE, Baker AR, Sindelar WF, Danforth DN, Topalian SL, DeLaney T, Glatstein E, Steinberg SM, Merino MJ, Rosenberg SA. Randomized prospective study of the benefit of adjuvant radiation therapy in the treatment of soft tissue sarcomas of the extremity. J Clin Oncol. 1998 Jan;16(1):197-203. PMID:9440743 (Link to Abstract)

Level of Evidence 3 (Retrospective Cohort Study or Case-Control Study)

Potter BK, Adams SC, Pitcher JD Jr, Temple HT. Local recurrence of disease after unplanned excisions of high-grade soft tissue sarcomas. Clin Orthop Relat Res. 2008 Dec;466(12):3093-100. Epub 2008 Sep 26. PMID:18818981 (Link to Abstract)
Skrzynski MC, Biermann JS, Montag A, Simon MA. Diagnostic accuracy and charge-savings of outpatient core needle biopsy compared with open biopsy of musculoskeletal tumors. J Bone Joint Surg Am. 1996 May;78(5):644-9. PMID:8642019 (Link to Abstract)

Level of Evidence 4 (Case Series)

Ward WG Sr, Rougraff B, Quinn R, Damron T, O'Connor MI, Turcotte RE, Cline M. Tumors masquerading as hematomas. Clin Orthop Relat Res. 2007 Dec;465:232-40 PMID:17828027 (Link to Abstract)
Davis AM, Kandel RA, Wunder JS, Unger R, Meer J, O'Sullivan B, Catton CN, Bell RS. The impact of residual disease on local recurrence in patients treated by initial unplanned resection for soft tissue sarcoma of the extremity. J Surg Oncol. 1997 Oct;66(2):81-7. PMID:9354161 (Link to Abstract)
Noria S, Davis A, Kandel R, Levesque J, O'Sullivan B, Wunder J, Bell R. Residual disease following unplanned excision of soft-tissue sarcoma of an extremity. J Bone Joint Surg Am. 1996 May;78(5):650-5. PMID:8642020 (Link to Abstract)

Level of Evidence 5 and Other Journal Articles (includes Case Reports, Expert Opinions, Personal Observations, and Biomechanic Studies)

Peabody TD, Gibbs CP Jr, Simon MA. Evaluation and staging of musculoskeletal neoplasms. J Bone Joint Surg Am. 1998 Aug;80(8):1204-18. Review. PMID:9730132 (Link to Abstract)

Textbooks

Review of Orthopaedics, 6th Edition, Mark D. Miller MD, Stephen R. Thompson MBBS MEd FRCSC, Jennifer Hart MPAS PA-C ATC, an imprint of Elsevier, Philadelphia, Copyright 2012
AAOS Comprehensive Orthopaedic Review, Jay R. Leiberman. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2009
Orthopaedic Knowledge Update 10, John M Flyn. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2011
Hoppenfeld SP. Surgical Exposures in Orthopaedics: The Anatomic Approach. Lipponcott, Williams, and Wilkins, Philadelphia, PA, Copyright 2009
Orthopaedic In-training Examination (OITE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
Self-Assessment Examination (SAE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
Orthopaedic Knowledge Update. Musculoskeletal Oncology. Schwartz, H (ed). American Academy of Orthopaedic Surgeons. Rosemont, IL. 2005.

Undefined

Demas BE, Heelan RT, Lane J, Marcove R, Hajdu S, Brennan MF. Soft-tissue sarcomas of the extremities: comparison of MR and CT in determining the extent of disease. AJR Am J Roentgenol. 1988 Mar;150(3):615-20. PMID:3257620 (Link to Abstract)
Gibbs CP, Peabody TD, Mundt AJ, Montag AG, Simon MA. Oncological outcomes of operative treatment of subcutaneous soft-tissue sarcomas of the extremities. J Bone Joint Surg Am. 1997 Jun;79(6):888-97 PMID:9199387 (Link to Abstract)
Gilbert NF, Cannon CP, Lin PP, Lewis VO. Soft-tissue sarcoma. J Am Acad Orthop Surg. 2009 Jan;17(1):40-7. Review. PMID:19136426 (Link to Abstract)
Rougraff B, Sandler A. Karnofsky performance score as a predictor of survival in soft tissue sarcoma. Clin Orthop Relat Res. 2002 Apr;(397):196-203. PMID:11953611 (Link to Abstract)

Topic Comments

Subscribe status: On Off

Recent on Bottom | Recent on Top

Page:1

Soft Tissue Sarcoma

Qbank (9 Questions)

Question Comments

Question Comments

Question Comments

Question Comments

Question Comments

Question Comments

Question Comments

Question Comments

Cases

Posts

Groups

Evidence & References Show References

Topic Comments