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Non-Rhabdomyosarcoma (Non-RMS)

 

 

Surgery

 

 

Surgery is the most important modality for local control in non-RMS. The primary aim of surgery should be to excise the tumor with a rim of surrounding normal tissue.

Unplanned surgical excisions can lead to very poor outcomes with tumor spread and should never be performed.

Surgery should be performed by an appropriately trained surgeon. The tumor should be removed en bloc, with at least a 0.5 cm margin of uninvolved tissue or an intact fascial boundary surrounding the tumor if possible. Previous biopsy needle tracts and incisions should be included in the en bloc resection specimen.

There is some controversy about the width of surrounding normal tissue that is adequate.

  • 1 cm generally considered to be adequate for adult patients.
  • For pediatric patients, a margin of 0.5 cm is usually considered adequate.
  • When the tumor abuts fascia or periosteum and the fascia or periosteum is removed in continuity with the tumor specimen, this margin is considered negative.

When a drain is required, it should exit via the incision or very close to the incision. 

Radiation therapy (RT), if required, will have to encompass the drain tract. Care should be taken not to contaminate normal tissue using instruments that have come in contact with tumor.

Sometimes because of tumor location (e.g. involvement of neurovascular bundle) it is not possible to achieve wide margins.

If surgery is not possible alone to resect the primary tumor with negative margins, then a planned marginal excision is performed.  Radiation therapy (RT) can be used either pre- or post-operatively to help ensure local control.

RT in children may be associated with significant morbidity such as very reduced growth within the area treated with RT and an increased risk of a second malignant neoplasm within the RT field many years after treatment and therefore should be avoided if possible.

In the great majority of cases arising in the extremities, limb-sparing surgery should be the objective possible and amputation can be avoided if possible.

Patients with lymph node involvement by tumor should have lymph node dissection at the time of definitive tumor resection. Prior to dissection of the involved lymph nodes, more proximal nodes should be biopsied to identify occult involvement that would mandate a more extensive lymph node dissection.

After an unplanned surgical excision it may be difficult or impossible to perform a re-excision with negative margins.

 

 

Four categories of surgical margins have been identified:

Intralesional
  • Margin runs through the tumor and therefore gross tumor remains in place
  • Tumor resection leaves macroscopic tumor (even if only
    minimal) in situ.

 
Marginal
  • Gross total resection with positive microscopic margins
  • Surgical plane runs through the pseudocapsule (reactive zone).
  • Tumor resection leaves no residual macroscopic tumor behind, but there are minimal margins (< 0.5 cm without any
    fascia)
  • Tumor is present at the margins of the specimen, or biopsies of grossly uninvolved areas of the tumor bed contain tumor cells microscopically.
  • This category includes excisional biopsies.
  • The local recurrence rate is extremely high because of tumor satellites in the reactive tissue

 
Wide
  • Gross total resection with negative microscopic margins
  • Removal of tumor entirely surrounded by either a cuff of normal tissue at least 0.5 cm thick or intact fascia.
  • If the pathologist finds that the margins are inadequate (less than 0.5 cm of normal tissue or intact fascia surrounding the
    specimen), the resection should be reclassified as a marginal resection (gross total resection with positive
    microscopic margins).
  • The surgical plane is in normal tissue, but in the same compartment as the  tumor.
  • The recurrence rate is low and related only to skip lesions in the affected compartment

 
Radical
  • The tumor is removed completely including the affected compartments
  • The risk of local recurrence is extremely small

 

 

 

 

Limb Sparing Surgery:  

Goal
  • Remove the entire tumor while preserving margins of normal tissue all around the tumor

 

Timing of Surgery

  • If wide margins are not possible, may be performed after pre-operative radiation therapy/chemotherapy
  • Imaging studies must be repeated just prior to surgery to look at extent of disease

 

Disadvantages
  • May not achieve complete local control

 

Indications
  • Tumor is relatively small, a wide rim of normal tissue can be taken around the tumor without endangering the neurovascular bundle

 

 

 

Amputation:

Goal
  • Achieve complete local control of tumor

 

Disadvantages
  • Functional Impairment

 

Indications
  • Tumor is too large and invasive for limb sparing surgery
  • RT would encompass the entire circumference of the limb and lead to significant complications (lymphoedema and vascular compromise)

 

 

 

 

 

References and Resources:

 

 

 

 

 

  

 

 

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