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.

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.

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.

Differential Diagnoses

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COG-ARST0332 experimental design schema.

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Table. American Joint Commission for Cancer Staging (Tumor, Node, and Metastases [TNM] System)

	Primary Tumor	Regional Lymph Nodes	Distant Metastasis	Histologic Grade
Stage I	Any tumor size, superficial or deep	N0	M0	G1 or G2
Stage II	T1a (tumor < 5 cm, superficial)	N0	M0	G3
	T1b (tumor < 5 cm, deep)	N0	M0	G3
	T2a (tumor >5 cm, superficial)	N0	M0	G3
Stage III	T2b (tumor >5 cm, deep)	N0	M0	G3
Stage IV	Any tumor size, superficial or deep	N1	M0 or M1	G1, G2, or G3
Stage IV	Any tumor size, superficial or deep	N0 or N1	M1	G1, G2, or G3

Table, Risk Based Treatment Stratification used in COG-ARST0332 study. Adapted from Spunt et al., 2014 and Sangkhathat, 2015 ^{[50, 58]}

Risk group	Tumor characteristics				Treatment
	Tumor Grade	Tumor Size	Tumor Stage	Upfront Surgical Resectability
Low	Low	Any	Localized	Gross resection (with negative or positive microscopic margins)	Surgery + observation
Low	High	< 5 cm	Localized	Gross resection with negative microscopic margins	Surgery + observation
Low	High	< 5 cm	Localized	Gross resection with positive microscopic margins	Surgery + adjuvant radiation therapy
Intermediate	High	>5 cm	Localized	Gross resection	Surgery + adjuvant radiation therapy + adjuvant chemotherapy
Intermediate	High	Any	Localized	Unresectable or Planned delayed surgical resection due to high grade, size >5 cm, and anticipate gross resection only possible with positive microscopic margins	Neoadjuvant chemotherapy + surgery + adjuvant chemotherapy with or without radiation therapy
High	Low	Any	Metastatic	Gross resection	Surgery + observation
High	High	Any	Metastatic	Gross resection	Surgery + adjuvant radiation therapy + chemotherapy
High	High	Any	Metastatic	Unresected	Neoadjuvant chemotherapy + surgery + adjuvant chemotherapy with or without radiation therapy

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Author

Jacquelyn N Crane, MD Clinical Assistant Professor, Division of Pediatric Hematology-Oncology, Stem Cell Transplantation and Regenerative Medicine, Associate Program Director, Pediatric Hematology-Oncology Fellowship Program, Stanford University School of Medicine

Jacquelyn N Crane, MD is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, Connective Tissue Oncology Society

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, American Society of Hematology

Disclosure: Nothing to disclose.

Noah C Federman, MD Director, Pediatric Bone and Soft Tissue Sarcoma Program, Associate Clinical Professor of Pediatrics, Department of Pediatrics, Division of Pediatric Hematology/Oncology, Adjunct Associate Professor, Department of Orthopedics, Mattel Children’s Hospital, University of California, Los Angeles, David Geffen School of Medicine; Medical Director, Clinical Translational Research Center, Chair, Data Safety Monitoring Board, Vice Chair, Scientific Rationale Committee, Clinical and Translational Science Institute (CTSI) at UCLA

Noah C Federman, MD is a member of the following medical societies: American Society of Clinical Oncology, American Society of Pediatric Hematology/Oncology, Children's Oncology Group, Connective Tissue Oncology Society, International Ewing Sarcoma Research Forum, Sarcoma Alliance for Research through Collaboration, Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

Chief Editor

Vikramjit S Kanwar, MBBS, MBA, MRCP(UK) Professor Emeritus of Pediatrics, Albany Medical College; Chief of Pediatric Oncology, Homi Bhabha Cancer Hospital, Varanasi, India

Vikramjit S Kanwar, MBBS, MBA, MRCP(UK) is a member of the following medical societies: Children's Oncology Group, Indian Academy of Pediatrics, International Society of Pediatric Oncology

Disclosure: Nothing to disclose.

Additional Contributors

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, Children's Oncology Group, American Society of Clinical Oncology, International Society for Experimental Hematology, American Society of Hematology, American Society of Pediatric Hematology/Oncology

Disclosure: Nothing to disclose.

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 Academy of Pediatrics, American Society of Hematology

Disclosure: Nothing to disclose.

Acknowledgements

Samuel Gross, MD Professor Emeritus, Department of Pediatrics, University of Florida College of Medicine; Clinical Professor, Department of Pediatrics, University of North Carolina at Chapel Hill School of Medicine; Adjunct Professor, Department of Pediatrics, Duke University School of Medicine

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.

Kathleen M Sakamoto, MD, PhD Shelagh Galligan Professor, Division of Hematology/Oncology, Department of Pediatrics, Stanford University School of Medicine

Kathleen M Sakamoto, MD, PhD is a member of the following medical societies: American Society of Hematology, American Society of Pediatric Hematology/Oncology, International Society for Experimental Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.