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Surg Oncol Clin N Am 2021 04 27;30(2):355-371. Epub 2021 Jan 27.

Department of Surgery, Brody School of Medicine, East Carolina University, 600 Moye Boulevard, Greenville, NC 27834, USA.

Pediatric nonrhabdomyosarcoma soft tissue sarcomas (NRSTSs) encompass a heterogeneous group of mesenchymal tumors with more than 50 histologic variants. The incidence of NRSTS is greater than rhabdomyosarcoma; however, each histologic type is rare. The treatment schema for all NRSTSs is largely surgical. Read More

Int J Radiat Oncol Biol Phys 2021 07 3;110(3):821-830. Epub 2021 Feb 3.

Department of Orthopedics, University of California Davis, Sacramento, California.

Purpose: The ARST0332 trial for pediatric and young adults with nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) used risk-based treatment including primary resection with lower-than-standard radiation doses to optimize local control (LC) while minimizing long-term toxicity in those requiring radiation therapy (RT). RT for high-grade NRSTS was based on extent of resection (R0: negative margins, R1: microscopic margins, R2/U: gross disease/unresectable); those with >5 cm tumors received chemotherapy (CT; ifosfamide/doxorubicin). This analysis evaluates LC for patients assigned to RT and prognostic factors associated with local recurrence (LR). Read More

Arch Argent Pediatr 2020 12;118(6):e549-e553

Servicio de Hematología y Oncología, Hospital de Pediatría SAMIC "Prof. Dr. Juan P. Garrahan", Ciudad Autónoma de Buenos Aires.

Infantile fibrosarcoma is a rare tumor, belonging to the nonrhabdomyosarcoma, soft tissue sarcoma. It is mostly presented in infants, most commonly involving the extremities. We report a 5-month-old boy, presenting with digestive bleeding since the age of 3 months, initially diagnosed as cow's milk allergy, with a torpid evolution and anemia. Read More

Cancer 2021 03 10;127(5):739-747. Epub 2020 Nov 10.

Department of Radiation Oncology and Molecular Radiation Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland.

Background: Metastasectomy is standard of care for pediatric patients with metastatic sarcoma with limited disease. For patients with unresectable disease, stereotactic body radiotherapy (SBRT) may serve as an alternative. Herein, the authors report the results of a prospective, multi-institutional phase 2 trial of SBRT in children and young adults with metastatic sarcoma. Read More

Sarcoma 2020 4;2020:6312480. Epub 2020 May 4.

Children's Cancer Therapy Development Institute, Beaverton, OR 97005, USA.

Nonrhabdomyosarcoma soft-tissue sarcomas (STSs) are a class of 50+ cancers arising in muscle and soft tissues of children, adolescents, and adults. Rarity of each subtype often precludes subtype-specific preclinical research, leaving many STS patients with limited treatment options should frontline therapy be insufficient. When clinical options are exhausted, personalized therapy assignment approaches may help direct patient care. Read More

J Pediatr Surg 2020 Aug 14;55(8):1673-1676. Epub 2020 Apr 14.

Homi Bhabha National Institute (HBNI), Mumbai, India; Department of Radiology, Tata Memorial Hospital, Tata Memorial Centre, Mumbai, India.

Surgery continues to remain an integral component of treatment, especially for nonrhabdomyosarcoma soft tissue sarcoma as compared to rhabdomyosarcoma owing to their general insensitivity to chemotherapy. A key determinant of outcomes, particularly for extremity tumors includes complete tumor resection with negative margins; however, a significant limitation for limb salvage surgery is the adherence of sarcomas to vital vascular structures. Hitherto, vascular involvement constituted an adequate reason for amputation. Read More

Curr Oncol 2020 02 1;27(Suppl 1):6-16. Epub 2020 Feb 1.

Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, Toronto, ON.

Soft-tissue sarcoma (sts) is rare and represents approximately 7% of cancers in children and in adolescents less than 20 years of age. Rhabdomyosarcoma (rms) is most prevalent in children less than 10 years of age and peaks again during adolescence (16-19 years of age). Multi-agent chemotherapy constitutes the mainstay of treatment for rms. Read More

J Pediatr Hematol Oncol 2020 04;42(3):175-180

Departments of Otorhinolaryngology, Head and Neck Surgery and Audiology.

Aim: The aim of this study was to estimate nationally the survival of children, adolescents, and young adults with head and neck soft tissue sarcomas.

Materials And Methods: The authors included patients 0 to 21 years of age and diagnosed with rhabdomyosarcoma (RMS) or nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) located in the head and neck between 1980 and 2014. Survival probabilities were estimated using the Kaplan-Meier method. Read More

Curr Opin Pediatr 2019 06;31(3):368-377

Department of Surgery, Division of Pediatric Surgery, University of North Carolina Children's Hospital, Chapel Hill, North Carolina, USA.

Purpose Of Review: Nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) is a rare subgroup of malignancy in childhood that is composed of a variety of soft tissue and bony tumors. Prognosis for resectable localized disease is usually good and improved with systemic treatment. However, survival from locally advanced and metastatic disease remains poor. Read More

J Surg Res 2019 09 24;241:205-214. Epub 2019 Apr 24.

Division of Pediatric Surgery, DeWitt-Daughtry Department of Surgery, Leonard M. Miller School of Medicine, University of Miami, Miami, Florida. Electronic address:

Background: The significance of lymph node sampling (LNS) on disease-specific survival (DSS) of extremity soft tissue sarcomas (STS) is unknown. We investigated the effect of LNS on DSS in child and adolescent extremity STS.

Materials And Methods: The Surveillance, Epidemiology, and End Results registry was queried for patients aged <20 y with extremity STS who underwent surgery. Read More

Int J Radiat Oncol Biol Phys 2019 01 10;103(1):38-44. Epub 2018 Sep 10.

Department of Radiation Oncology and Molecular Radiation Sciences, Johns Hopkins University School of Medicine, Baltimore, Maryland. Electronic address:

Purpose: For pediatric patients with large, high-grade, extremity nonrhabdomyosarcoma soft-tissue sarcomas, preoperative radiation therapy (RT) provides the opportunity for smaller radiation fields and tumor shrinkage resulting in less extensive surgery. The potential disadvantage is an increased risk of wound complications after surgery compared with rates after postoperative chemoradiation. We assessed the impact of preoperative RT technique on target coverage in relationship to dose to skin and adjacent joints to determine whether acute wound complications and late musculoskeletal injury might be influenced by treatment technique. Read More

Pediatr Blood Cancer 2018 04 29;65(4). Epub 2017 Dec 29.

Fondazione IRCCS Istituto Nazionale Tumori Milano, Milan, Italy.

Background: As alveolar soft part sarcomas (ASPS) are rare with no prospective series within pediatric sarcoma trials, the European Paediatric Soft Tissue Sarcoma Study Group (EpSSG) examined the clinical data and outcomes of ASPS enrolled in a multinational study of nonrhabdomyosarcoma soft tissue sarcomas (NRSTS).

Patients And Methods: Twenty-two patients with ASPS were enrolled into the EpSSG NRSTS 2005 study. After surgical resection, subsequent treatment depended on the stratification of patients for completeness of resection and Intergroup Rhabdomyosarcoma Study (IRS) stage, size, and French Federation of Cancer Centres Sarcoma Group (FNCLCC) grade. Read More

Pediatr Blood Cancer 2018 Feb 19;65(2). Epub 2017 Sep 19.

Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Milano, Italy.

Background: Malignant peripheral nerve sheath tumor (MPNST) is one of the most common nonrhabdomyosarcoma soft tissue sarcomas encountered in pediatric age, and it is generally characterized by poor outcome, particularly for relapsing patients.

Materials And Methods: This study considered 73 patients <21 years of age with relapsing MPNST observed among 120 patients enrolled in Italian pediatric protocols from 1979 to 2004. With the aim of possibly establishing a risk-adapted stratification, patients' outcome was examined using univariate and multivariate analysis based on clinical features at onset, first-line treatments, clinical findings at the time of first relapse, and second-line treatments. Read More

Cancer 2017 Nov 31;123(22):4419-4429. Epub 2017 Jul 31.

Department of Radiation Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee.

Background: Indications for and delivery of adjuvant therapies for pediatric nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) have been derived largely from adult studies; therefore, significant concern remains regarding radiation exposure to normal tissue. The authors report long-term treatment outcomes and toxicities for pediatric and young adult patients with high-grade NRSTS who were treated on a prospective trial using limited-margin radiotherapy.

Methods: Sixty-two patients (ages 3-22 years) with predominantly high-grade NRSTS requiring radiation were treated on a phase 2 institutional study of conformal external-beam radiotherapy and/or brachytherapy using a 1. Read More

Pract Radiat Oncol 2017 Nov - Dec;7(6):411-417. Epub 2017 Apr 26.

Department of Radiation Oncology, St. Jude Children's Research Hospital Memphis, Tennessee.

Purpose: To investigate the contribution of radiation therapy to acute and late toxicity in pediatric chest wall sarcoma patients and evaluate dosimetric correlates of higher incidence toxicities such as scoliosis and pneumonitis.

Methods And Materials: The data from 23 consecutively treated pediatric patients with chest wall sarcomas of various histologies (desmoid, Ewing, rhabdomyosarcoma, nonrhabdomyosarcoma-soft tissue sarcomas) were reviewed to evaluate the relationship between end-organ radiation dose, clinical factors, and the risk of subsequent late effects (scoliosis, pneumonitis). Cobb angles were used to quantify the extent of scoliosis. Read More

Semin Nucl Med 2017 05 18;47(3):229-241. Epub 2017 Jan 18.

Diagnostic Imaging Department, Saint Jude Children's Research Hospital, Memphis, TN.

Considerable debate remains regarding how best to incorporate F-FDG-PET/CT into clinical practice for pediatric sarcomas. Although there is a clear role for F-FDG-PET/CT in staging pediatric sarcoma, the value of F-FDG-PET/CT in prognostication for pediatric sarcomas remains unclear. In osteosarcoma, Ewing sarcoma, and rhabdomyosarcoma, F-FDG-PET/CT may be most useful in the identification of skeletal metastases, where the literature consistently suggests that it has improved sensitivity and specificity as compared to bone scintigraphy. Read More

Cochrane Database Syst Rev 2017 04 13;4:CD008216. Epub 2017 Apr 13.

Department of Psychology, Social Work and Public Health, Oxford Brookes University, Jack Straws Lane, Marston, Oxford, UK, OX3 0FL.

Background: Soft tissue sarcomas (STS) are a highly heterogeneous group of rare malignant solid tumors. Nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) comprise all STS except rhabdomyosarcoma. In people with advanced local or metastatic disease, autologous hematopoietic stem cell transplantation (HSCT) applied after high-dose chemotherapy (HDCT) is a planned rescue therapy for HDCT-related severe hematologic toxicity. Read More

Pediatr Blood Cancer 2017 Sep 24;64(9). Epub 2017 Feb 24.

Pediatric Hematology and Oncology Division, Padova University, Padova, Italy.

Background: Pediatric nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) may rarely occur in visceral tissues, and little is known about their clinical history. The present study retrospectively analyzed a group of patients prospectively registered in Italian pediatric protocols conducted between 1979 and 2004.

Methods: Inclusion criteria for the study were as follows: a pathological diagnosis of "adult-type NRSTS," arising at visceral sites (lung-pleurae, liver, kidney, and mesentery-bowel); age under 18 years; no previous treatment except for primary surgery; available clinical data; and written consent. Read More

J Pediatr Surg 2017 Aug 21;52(8):1340-1343. Epub 2017 Jan 21.

Department of Biostatistics, Tata Memorial Centre, Bombay, India.

Background: It is not exceptional to come by children with initial unplanned surgical intervention for nonrhabdomyosarcoma soft tissue sarcomas (NRSTS). The aim of this study was to evaluate the presence of residual disease in these patients after re-excision and compare the treatment outcomes with patients who had planned upfront excision.

Methods: The data of patients with primary nonmetastatic NRSTS with initially unplanned excision who underwent re-excision between March 2006 and December 2014 were analyzed and the results compared with patients having planned upfront excision in the similar period. Read More

J Pediatr Hematol Oncol 2016 11;38(8):615-621

Departments of *Pediatric Oncology §Surgical Oncology, National Cancer Institute, Cairo University †Department of Pediatric Oncology, Children Cancer Hospital ‡Department of Pediatrics, National Research Center ∥Department of Pediatrics, Misr Insurance Hospital, Cairo, Egypt.

Background: Nearly half of soft tissue sarcomas are nonrhabdomyosarcomas (NRSTSs). The low-grade (LG) form comprises a heterogenous group of diseases that rarely metastasize but are known for local recurrence.

Aim Of The Study: The aim of the study was to retrospectively evaluate pediatric LG-NRSTS with regard to demography, survival, and factors affecting outcome in Egyptian patients. Read More

Surg Clin North Am 2016 Oct;96(5):1107-25

Department of Surgery, University of Tennessee Health Science Center, 49 North Dunlap Avenue, Second Floor, Memphis, TN 38105, USA.

Pediatric sarcomas are a heterogeneous group of tumors accounting for approximately 10% of childhood solid tumors. Treatment is focused on multimodality therapy, which has improved the prognosis over the past two decades. Current regimens focus on decreasing treatment for low-risk patients to decrease the long-term side effects while maximizing therapy for patients with metastatic disease to improve survival. Read More

Am J Clin Pathol 2016 Sep 10;146(3):328-38. Epub 2016 Aug 10.

Department of Pediatrics, Stanford University School of Medicine, Palo Alto, CA.

Objectives: Treatment of soft tissue tumors in young patients relies on the diagnostic information conveyed in the pathology report. We examined pathology reports from Children's Oncology Group ARST0332 for inclusion of data elements required in published guidelines.

Methods: Pathology reports for 551 eligible patients were examined for required data elements defined by the College of American Pathologists, including tissue type, procedure, tumor site, tumor maximum diameter, macroscopic extent of tumor, histologic type, mitotic rate, extent of necrosis, tumor grade, margin status, use of ancillary studies, and pathologic stage. Read More

Ophthalmic Plast Reconstr Surg 2017 May/Jun;33(3):e57-e60

*John A. Moran Eye Center, Department of Ophthalmology and Visual Sciences, The University of Utah School of Medicine, Salt Lake City, Utah, †Ruiz Department of Ophthalmology and Visual Science and Department of Neurology, McGovern Medical School at The University of Health Science Center at Houston (UTHealth), Houston, Texas, ‡Robert Cizik Eye Clinic, Houston, Texas, and §Department of Pathology, University of Utah, Salt Lake City, Utah, U.S.A.Supported in part by National Eye Institute Vision Core Grant P30EY010608 (UT), a Challenge Grant to the McGovern Medical School from Research to Prevent Blindness, New York, NY (UT), an Unrestricted Grant from Research to Prevent Blindness, Inc., New York, NY, to the Department of Ophthalmology & Visual Sciences, University of Utah, and the Hermann Eye Fund, Houston, TX (UT).

A 6-month-old boy presented with recurrent, poorly differentiated orbital sarcoma diagnosed as nonrhabdomyosarcoma soft tissue sarcoma, as pathologic and immunohistochemical evaluation was inconsistent with rhabdomyosarcoma or other specific sarcoma subtypes. He responded favorably to a treatment regimen for poorly differentiated sarcomas. Read More

Int J Radiat Oncol Biol Phys 2016 May 17;95(1):267-278. Epub 2015 Oct 17.

Department of Radiation Oncology, University of Leuven, Leuven, Belgium.

Because it spares many normal tissues and reduces the integral dose, proton therapy (PT) is the preferred tumor irradiation technique for treating childhood cancer. However, to the best of our knowledge, no systematic review of the clinical effectiveness of PT in children has been reported in the scientific literature. A systematic search for clinical outcome studies on PT published between 2007 and 2015 was performed in Medline (through OVID), EMBASE, and the Cochrane Library. Read More

AJR Am J Roentgenol 2015 Aug;205(2):414-20

7 Department of Radiological Sciences, St. Jude Children's Research Hospital, Memphis, TN.

Objective: The purpose of this article is to determine the MRI and CT features of low-grade fibromyxoid sarcoma in children.

Materials And Methods: We retrospectively analyzed images of 11 pediatric patients with low-grade fibromyxoid sarcoma from a phase 3 clinical trial of nonrhabdomyosarcoma soft-tissue sarcoma (Children's Oncology Group Protocol ARST0332). MRI and CT were performed in 10 and four patients, respectively. Read More

Pediatr Clin North Am 2015 Feb;62(1):179-200

Division of Hematology, Oncology, and Blood & Marrow Transplantation, Department of Pediatrics, Children's Center for Cancer and Blood Diseases, Children's Hospital Los Angeles, University of Southern California Keck School of Medicine, 4650 Sunset Boulevard, MS 54, Los Angeles, CA 90027, USA. Electronic address:

Malignant bone tumors (osteosarcoma, Ewing sarcoma) and soft-tissue sarcomas (rhabdomyosarcoma, nonrhabdomyosarcoma) account for approximately 14% of childhood malignancies. Successful treatment of patients with sarcoma depends on a multidisciplinary approach to therapy, including oncology, surgery, radiation oncology, radiology, pathology, and physiatry. By combining systemic treatment with chemotherapy and primary tumor control using surgery and/or radiation, survival rates for localized disease range from 70% to 75%. Read More

AJR Am J Roentgenol 2014 Dec;203(6):1345-52

1 Department of Radiological Sciences, St. Jude Children's Research Hospital, 262 Danny Thomas Pl, MS 220, Memphis, TN 38105.

Objective: There are few studies in the literature regarding the imaging features of alveolar soft-part sarcoma (ASPS). We performed a comprehensive assessment of the imaging characteristics of this rare tumor to determine whether there are features that suggest the diagnosis.

Materials And Methods: Twenty-two subjects with ASPS underwent pretherapy imaging as part of enrollment in Children's Oncology Group protocol ARST0332 for the treatment of nonrhabdomyosarcoma soft-tissue sarcomas: 16 patients underwent MRI; three, CT; and three, both MRI and CT. Read More

Curr Oncol Rep 2014 ;16(8):395

Division of Pediatric Hematology-Oncology, Maine Medical Center, 22 Bramhall Street, Portland, ME, 04102, USA,

Pediatric sarcomas are relatively rare malignancies individually. As a group they are typically approached with combination chemotherapies in addition to local control. Fortunately, these malignancies have been approached through careful clinical trial collaboration to define risk groups and appropriately deliver local control measures and systemic therapies. Read More

J Pediatr Hematol Oncol 2014 May;36(4):257-62

*Pediatric, Adolescent and Young Adult Department Departments of †Radiology ‡Medical Oncology #Pathology §Radiotherapy Department, Institut Curie ∥Pediatric Orthopedic Department, Necker Hospital-Assistance Publique, Université Paris Descartes-Sorbonne Paris ¶Pediatric Orthopedic Department, Armand Trousseau Hospital-Assistance Publique, Paris, France.

Synovial sarcoma (SS) is a high-grade soft tissue sarcoma characterized by local invasiveness and a propensity to metastasize, affecting pediatric, adolescent, and adult populations. The peak incidence is observed in the third decade of life and SS is the most common nonrhabdomyosarcoma soft tissue sarcoma in childhood and adolescence. Although pediatric and adult SS appear clinically and radiologically identical, treatment modalities may differ according to the patient's age. Read More

Case Rep Oncol Med 2013 10;2013:627472. Epub 2013 Dec 10.

Pediatric Pathology Research Center, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran 15468-15514, Iran.

Malignant peripheral nerve sheath tumors (MPNSTs) are nonrhabdomyosarcoma soft tissue sarcomas with rare occurrence in children specially in the retroperitoneum. We describe a young child who presented with an abdominal mass. Both ultrasound and computed tomography revealed a large right-sided abdominal mass in the anatomic place of right kidney, while no kidney or ureter was observed at that side. Read More