Nonrhabdomyosarcoma Soft Tissue Sarcomas Clinical Presentation

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

History

Patients with nonrhabdomyosarcoma soft tissue sarcomas (NRSTSs) usually present with painless, asymptomatic masses. The tumors may come to attention because of an episode of trauma in the affected area. Mass effect due to the tumor may cause specific signs or symptoms depending on the location of the mass. For instance, invasion of local neurovascular bundles in an involved extremity may lead to pain, swelling, numbness, or loss of function. Large masses in the chest wall may cause pulmonary dysfunction.

If advanced metastatic disease is present, systemic symptoms with fever, sweats, and weight loss may be observed.

Hemangiopericytomas have been associated with hypoglycemia and hypophosphatemic rickets. Hyperglycemia has been observed with fibrosarcoma of the lung.

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Physical

Physical findings depend on the location of the mass. A mass is palpable in many, if not most, patients.

Specific tumors may be associated with specific findings. Malignant peripheral nerve sheath tumors may be associated with neurofibromatosis type 1 (NF1), which is characterized by café au lait spots, axillary freckling, neurofibromas, skeletal dysplasias, learning disabilities, and various neoplasms. CNS tumors may cause an abnormal neurologic findings depending on the location of the mass and the structures affected.

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Causes

Genetic conditions such as Li-Fraumeni syndrome associated with P53 mutations, NF1, and germline mutation of the retinoblastoma susceptibility gene, RB, are known genetic risk factors for NRSTS. Gorlin syndrome has been associated with an increased risk of development of fibrosarcoma and leiomyosarcoma. NF1 is strongly associated with the development of malignant peripheral nerve sheath tumors.

Other factors with an association with the development of NRSTs include exposure to previous radiation, childhood cancer survival, and infection with retroviruses in immunocompromised children (eg, those with HIV or Epstein-Barr virus [EBV] infection). Individuals with HIV have an increased risk of developing leiomyosarcoma related to EBV infection.

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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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