Nonrhabdomyosarcoma Soft Tissue Sarcomas Workup

  • Author: Justine K Walker, MD; Chief Editor: Robert J Arceci, MD, PhD   more...
 
Updated: Aug 6, 2010
 

Laboratory Studies

In patients with nonrhabdomyosarcoma soft tissue sarcoma (NRSTS), a baseline CBC count with differential provides parameters before therapy and is useful in evaluating for involvement of the bone marrow.

Chemical tests to assess renal function and creatinine clearance provide baseline parameters before chemotherapy is given and further testing is performed.

Liver function testing provides baseline parameters before chemotherapy and is helpful in evaluating for hepatic involvement.

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Imaging Studies

Chest radiography and chest CT are useful for evaluating for lung involvement. Perform these studies before the use of general anesthesia, which can cause pulmonary changes that might make the interpretation of images difficult.

CT and MRI are used to determine the size of the mass and the extent of local involvement and impingement on adjacent structures. CT and MRI also help in defining options for surgical resection. Contrast-enhanced studies are most helpful. Abdominal CT scanning is important for assessing abdominal primary lesions and to determine hepatic involvement.

Radionucleotide bone scanning is necessary to rule out bony involvement.

Plain radiography of the involved areas may be useful in the initial evaluation of a mass, depending on its location and suspected involvement of bony structures.

The roles of positive emission tomography (PET)-CT and of18 F-fluorodeoxyglucose (FDG) PET to image NRSTS in children have not been well established. However, PET-CT may prove beneficial in distinguishing normal from pathologic processes, in the initial staging of a sarcoma, in monitoring responses to therapy, and in detecting recurrences.[10, 11]

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Other Tests

A cardiologist may need to be consulted to perform cardiac ECG and echocardiography in patients who will receive anthracycline-based chemotherapy or radiation therapy to the chest.

Testing of renal glomerular filtration or creatinine clearance may be necessary before renal-toxic chemotherapy agents (eg, cisplatin, ifosfamide) are administered.

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Procedures

Carefully planned and executed biopsy of the mass lesion is required for diagnosis.

Whatever technique is used, adequate tissue must be obtained to allow for histology, immunostaining, and other studies including cytogenetics, fluorescent in situ hybridization (FISH), and molecular pathology. If possible, surgery should be accomplished in a manner that does not compromise the possibility for later local surgical control of the tumor.

A surgeon with expertise in oncologic surgery should perform this procedure. The surgeon's specific discipline depends on the location of the mass.

The best approach for small lesions in accessible areas may be excisional biopsy.

Large masses involving critical organs or structures may require incisional biopsy for diagnosis.

Delay the definitive surgical procedure until after adjuvant chemotherapy, radiation therapy, or both are given to shrink the tumor.

Fine-needle aspiration biopsy may be possible in certain cases. However, an open procedure is required if this technique yields nondiagnostic pathologic material.

Minimally invasive surgical techniques using fiberoptic surgical procedures may be appropriate in certain biopsy situations.

Consider long-term venous access. In most cases, placement of an implanted or externalized central venous catheter is useful for monitoring laboratory results and for delivering chemotherapy and supportive care.

Bilateral bone marrow aspirates and biopsy samples should be obtained in most cases to rule out tumoral involvement of the bone marrow. Strongly consider examining the bone marrow in all patients with NRSTS.

In children, these procedures are best performed in the posterior iliac crests.

These tests should be accomplished in conjunction with another procedure requiring sedation, if possible.

If patients have parameningeal tumors or tumors involving the CNS, lumbar puncture may be necessary to rule out contamination of the cerebrospinal fluid (CSF) with tumor cells.

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Histologic Findings

Diagnosis of an NRSTS can be confirmed only with biopsy of the mass. Diagnosis of a specific tumor depends on the mesenchymal and/or support tissue it most closely represents. Immunostaining, electron microscopy, cytogenetic analysis, and tests for molecular markers of genetic rearrangements may all be used for final diagnosis, depending on the differentiation of the specific tumor. Many of the NRSTSs are characterized by chromosomal translocations that lead to a fusion of two genes. The resultant fusion gene can be detected by polymerase chain reaction (PCR)-based techniques. NRSTSs possess a wide range of histologic features.

A tumor is classified as low-grade or high-grade on the basis of its potential to metastasize. Low-grade lesions are unlikely to metastasize. Certain types of tumors are arbitrarily considered high grade; examples are synovial cell sarcomas and malignant peripheral nerve sheath tumors. Malignant and metastatic potential are based on the degree of anaplasia and mitotic activity the tumor specimen exhibits.

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Staging

No staging system is validated for NRSTSs in children. Two systems are currently in use: the surgicopathologic grouping system used by the Intergroup Rhabdomyosarcoma Study (IRS), which is based on the amount of tumor remaining after initial surgery, and the American Joint Commission for Cancer (AJCC) staging system for Soft Tissue Sarcomas[12] . This is the staging system that is being used in the current COG trial for NRSTS.

Table. American Joint Commission for Cancer Staging (Tumor, Node, and Metastases [TNM] System) (Open Table in a new window)

Primary TumorRegional Lymph NodesDistant MetastasisHistologic Grade
Stage IAny tumor size, superficial or deepN0M0G1 or G2
Stage IIT1a (tumor < 5 cm, superficial)N0M0G3
T1b (tumor < 5 cm, deep)N0M0G3
T2a (tumor >5 cm, superficial)N0M0G3
Stage IIIT2b (tumor >5 cm, deep)N0M0G3
Stage IVAny tumor size, superficial or deepN1M0 or M1G1, G2, or G3
Any tumor size, superficial or deepN0 or N1M1G1, G2, or G3

G1 = well differentiated; G2 = moderately differentiated; G3 = poorly differentiated; M0 = no distant mets; M1 = distant mets; N0 = no regional LN mets; N1 = regional LN mets

Intergroup Rhabdomyosarcoma Study (IRS) groups staging is as follows:

  • Group I - Complete resection with negative margins
  • Group II - Microscopic residual disease after resection
  • Group III - Incomplete resection or biopsy with gross residual tumor
  • Group IV - Metastatic disease present at time of diagnosis
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Contributor Information and Disclosures
Author

Justine K Walker, MD  Fellow, Division of Pediatric Hematology-Oncology, University of California, Los Angeles David Geffen School of Medicine

Justine K Walker, MD, is a member of the following medical societies: American Association of Pediatrics and American Society of Hematology

Disclosure: Nothing to disclose.

Coauthor(s)

Gary D Crouch, MD  Associate Professor, Program Director of Pediatric Hematology-Oncology Fellowship, Department of Pediatrics, Uniformed Services University of the Health Sciences

Gary D Crouch, MD is a member of the following medical societies: American Academy of Pediatrics and American Society of Hematology

Disclosure: Nothing to disclose.

Noah C Federman, MD  Assistant Professor of Pediatrics, Division of Pediatric Hematology/Oncology, Mattel Children's Hospital, David Geffen School of Medicine; Director, Pediatric Bone and Soft Tissue Sarcoma Program, University of California at Los Angeles

Noah C Federman, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Hematology, and Connective Tissue Oncology Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Samuel Gross, MD  Professor Emeritus, Department of Pediatrics, University of Florida; Clinical Professor, Department of Pediatrics, University of North Carolina; Adjunct Professor, Department of Pediatrics, Duke University

Samuel Gross, MD is a member of the following medical societies: American Association for Cancer Research, American Society for Blood and Marrow Transplantation, American Society of Clinical Oncology, American Society of Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Steven K Bergstrom, MD  Department of Pediatrics, Division of Hematology-Oncology, Kaiser Permanente Medical Center of Oakland

Steven K Bergstrom, MD is a member of the following medical societies: Alpha Omega Alpha, American Society of Clinical Oncology, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, and International Society for Experimental Hematology

Disclosure: Nothing to disclose.

Paul D Petry, DO, FACOP, FAAP  Consulting Staff, Freeman Pediatric Care, Freeman Health System

Paul D Petry, DO, FACOP, FAAP is a member of the following medical societies: American Academy of Osteopathy, American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association

Disclosure: Nothing to disclose.

Chief Editor

Robert J Arceci, MD, PhD  King Fahd Professor of Pediatric Oncology, Professor of Pediatrics, Oncology and the Cellular and Molecular Medicine Graduate Program, Kimmel Comprehensive Cancer Center at Johns Hopkins University School of Medicine

Robert J Arceci, MD, PhD is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Pediatric Society, American Society of Hematology, and American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author Kathleen M Sakamoto, MD, PhD, to the original writing and development of this article.

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Table. American Joint Commission for Cancer Staging (Tumor, Node, and Metastases [TNM] System)
Primary TumorRegional Lymph NodesDistant MetastasisHistologic Grade
Stage IAny tumor size, superficial or deepN0M0G1 or G2
Stage IIT1a (tumor < 5 cm, superficial)N0M0G3
T1b (tumor < 5 cm, deep)N0M0G3
T2a (tumor >5 cm, superficial)N0M0G3
Stage IIIT2b (tumor >5 cm, deep)N0M0G3
Stage IVAny tumor size, superficial or deepN1M0 or M1G1, G2, or G3
Any tumor size, superficial or deepN0 or N1M1G1, G2, or G3
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