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Int J Radiat Oncol Biol Phys 2019 Jan 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 Apr 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):267-78. 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

Pathol Res Pract 2013 May 15;209(5):309-13. Epub 2013 Mar 15.

Division of Pathology, Kanagawa Children's Medical Center, Yokohama, Japan.

Synovial sarcoma, which is difficult to diagnose precisely, is one of the most common childhood nonrhabdomyosarcoma soft-tissue sarcomas. The purpose of this study is to develop new molecular cytogenetic assay. We used two sets of two-color break-apart FISH probes, flanking either the SSX1/SSX4 or SSX2 locus. Read More

Clin Orthop Relat Res 2013 Mar;471(3):926-31

Department of Orthopaedic Surgery, Tokyo Medical and Dental University, 1-5-45, Yushima, Bunkyo-ku, Tokyo 113-8510, Japan.

Background: Lymph node metastases in patients with soft tissue sarcomas are rare and these metastases are frequently associated with certain histologic subtypes. The survival is believed to be poor if lymph node metastases occur and the potential benefit of lymphadenectomy is unclear.

Questions/purposes: We determined whether lymph node metastases affect overall survival with regard to the status of lymphadenectomy, histologic subtypes, isolated or systemic metastasis, and the timing of presentation of lymph node metastases. Read More

Sarcoma 2012 4;2012:350806. Epub 2012 Apr 4.

Department of Oncology and Hematology, Russian State Medical University, 1 Ostrovityanova Street, Moscow 117997, Russia.

The aim of the study was to assess the incidence and survival rates of soft tissue sarcomas (STSs) in children 0-14 years of age in Moscow Region, Russian Federation. The database of childhood population-based cancer registry of Moscow Region was used as a data source. Tumors were stratified according to International Classification of Childhood Cancer, 3d ed. Read More

ISRN Oncol 2012 15;2012:341408. Epub 2012 Mar 15.

Department of Radiation Oncology, Stanford Cancer Center, 875 Blake Wilbur Drive, Stanford, CA 94305, USA.

It remains unclear which children and adolescents with resected nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) benefit from radiation therapy, as well as the optimal dose, volume, and timing of radiotherapy when used with primary surgical resection. This paper reviews the sparse literature from clinical trials and retrospective studies of resected pediatric NRSTS to discern local recurrence rates in relationship to the use of radiation therapy. Read More

Semin Pediatr Surg 2012 Feb;21(1):61-7

University of Texas, MD Anderson Cancer Center, Houston, Texas, USA.

Nonrhabdomyosarcoma soft-tissue sarcomas (NRSTS) represent a subgroup of sarcomas that encompass more than 50 distinct histologies. All are rare, but some are more common in patients younger than 20 years of age. The management of patients with many histologies overlap. Read More

Pediatr Blood Cancer 2011 Dec 2;57(7):1244-6. Epub 2011 Sep 2.

Department of Pediatrics, Tokushima University Hospital, Tokushima, Japan.

A 3-month-old male with a mass on the right side of his back was admitted to our hospital. The tumor was a pathologically high-grade nonrhabdomyosarcoma soft tissue sarcoma (NRSTS). Treatment included subtotal tumor resection followed by chemotherapy. Read More

Pediatr Blood Cancer 2011 Aug 16;57(2):247-51. Epub 2011 Jan 16.

Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, Florida, USA.

Background: Few published articles describe outcomes following definitive radiation for unresectable pediatric and young adult nonrhabdomyosarcoma soft tissue sarcoma (NRSTS). The purpose of this study is to evaluate the prognostic factors, outcomes, and complications in patients age 30 years or younger with NRSTS treated at the University of Florida from 1973 to 2002.

Procedure: Nineteen pediatric and young adult patients with NRSTS were treated with radiotherapy after biopsy. Read More

Int J Radiat Oncol Biol Phys 2011 Sep 6;81(1):150-7. Epub 2011 May 6.

Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, FL, USA.

Purpose: To evaluate the prognostic factors, outcomes, and complications in patients aged ≤30 years with resectable nonrhabdomyosarcoma soft-tissue sarcoma treated at the University of Florida with radiotherapy (RT) during a 34-year period.

Methods And Materials: A total of 95 pediatric or young adult patients with nonrhabdomyosarcoma soft-tissue sarcoma were treated with curative intent with surgery and RT at the University of Florida between 1973 and 2007. The most common histologic tumor subtypes were synovial sarcoma in 22 patients, malignant fibrous histiocytoma in 19, and malignant peripheral nerve sheath tumor in 11 patients. Read More

Pediatr Dev Pathol 2011 Jul-Aug;14(4):339-44. Epub 2011 Mar 21.

Department of Pathology, Seattle Children's Hospital, Seattle, WA 98105, USA.

Adult-type sarcomas are, as the name indicates, rare tumors in the pediatric population. Although soft tissue sarcomas as a group are not uncommon diagnoses, nonrhabdomyosarcoma soft tissue sarcomas are much rarer and encompass a wide range of diagnoses. A few of these tumors are commonly found in the adult population and are thus referred to as adult-type sarcomas. Read More

AJR Am J Roentgenol 2010 Oct;195(4):1026-32

Department of Radiology, Children's Hospital Boston and Harvard Medical School, Boston, MA 02115, USA.

Objective: Synovial sarcoma is the most common malignant nonrhabdomyosarcoma soft-tissue sarcoma in children. This article shows examples of synovial sarcoma in children and corresponding examples of benign mimics.

Conclusion: It is important for radiologists to recognize the often nonaggressive appearance of synovial sarcoma in the pediatric population to guide surgical resection and expedite diagnosis before the development of locoregional spread or metastatic disease. Read More

Cancer 2010 May;116(9):2266-74

Department of Pathology, St. Jude Children's Research Hospital, Memphis, Tennessee, USA.

Background: Two systems for grading soft tissue sarcoma are widely used currently: the National Cancer Institute (NCI) and the Fédération Nationale des Centers de Lutte Contre le Cancer (FNCLCC) systems. Both were developed using cohorts of predominantly adult patients. The Pediatric Oncology Group (POG) system, based on the NCI system, was adapted for grading pediatric nonrhabdomyosarcoma soft tissue sarcoma (NRSTS). Read More

Cancer 2010 Jan;116(1):177-83

Pediatric Oncology Unit, National Tumor Institute, Milan, Italy.

Background: In a series of 575 patients < or =21 years of age with soft tissue sarcomas (STSs), the authors investigated the association patterns between symptom interval (ie, the period between the onset of the first symptoms or signs of the disease and its definitive diagnosis) and patient/tumor characteristics or disease outcome (in terms of survival).

Methods: The analysis was based on multivariate models (linear for associations with patient/tumor characteristics and Cox's for survival).

Results: The symptom interval ranged between 1 week and 60 months (median, 2 months) and tended to be longer the older the patient (ie, the interval was longer in adolescents than in children) and the larger the tumor's size, and for tumors located at the extremities and for nonrhabdomyosarcoma STSs (as opposed to rhabdomyosarcomas). Read More

J Pediatr Surg 2009 Oct;44(10):1972-6

Department of Surgery, St Jude Children's Research Hospital, Memphis, TN 38105, USA.

Purpose: The incidence of pediatric nonrhabdomyosarcoma soft tissue sarcomas (NRSTSs) of the groin and axilla is unknown, and the optimal surgical approach to these patients is unclear.

Methods: We conducted a retrospective study of patients treated at St Jude Children's Research Hospital from January 1962 to March 2007 for NRSTSs of the groin and axilla. Demographic variables, tumor pathology, clinical management, and outcome were reviewed. Read More

J Pediatr Surg 2009 Oct;44(10):1965-71

Department of Oncology, St Jude Children's Research Hospital, Memphis, TN 38105-2794, USA.

Background: Predictors of outcome have not been established for pediatric visceral and body wall nonrhabdomyosarcoma soft tissue sarcomas (NRSTS).

Methods: The study used a retrospective review of clinical features and outcome of 61 patients with visceral and body wall NRSTS evaluated at our institution between March 1962 and December 1999.

Results: Median age at diagnosis was 9. Read More

Int J Radiat Oncol Biol Phys 2010 Mar 20;76(3):874-8. Epub 2009 Jul 20.

Department of Radiological Sciences, St Jude Children's Research Hospital, Memphis, TN 38105, USA.

Purpose: To demonstrate the safety and efficacy of limited margin radiotherapy in the local control of pediatric and young adult patients with high-grade nonrhabdomyosarcoma soft tissue sarcoma (NRSTS).

Methods And Materials: Pediatric patients with high-grade NRSTS requiring radiation were treated on an institutional review board approved prospective institutional study of conformal/intensity-modulated/interstitial brachytherapy using a 2-cm anatomically constrained margin.

Results: A total of 32 patients (median age, 15. Read More

Am J Clin Oncol 2009 Feb;32(1):27-9

Department of Radiation Oncology, University of Miami, Miami, FL 33136, USA.

Objective: We report the management and outcome of prostate sarcoma at 1 institution and analyze factors that may determine prognosis.

Materials And Methods: The medical records of 10 patients with prostate sarcoma were reviewed to identify symptoms at presentation, diagnostic procedures, and staging methods. Histology, grade, tumor size, stage, and treatment modality were analyzed. Read More

J Egypt Natl Canc Inst 2008 Dec;20(4):395-402

The Departments of Pediatric Oncology, NCI, Cairo University.

Purpose: Evaluation of demographic, pathological, and clinical patterns in addition to treatment outcome of pediatric NRSTS patients treated at the NCI, Egypt.

Procedure: 21 pediatric patients of NRSTS between 2001 and 2006 were included. Clinical and pathological diagnosis and subtyping verification were done. Read More

Oncologist 2008 Jun;13(6):668-78

Department of Oncology, St. Jude Children's Research Hospital, Department of Oncology, 332 N. Lauderdale Street, ALSAC-6032, Memphis, TN 38105-2794, USA.

The nonrhabdomyosarcoma soft tissue sarcomas (NRSTSs) are a heterogeneous group of mesenchymal cell neoplasms that account for about 4% of childhood cancers. Because each histologic subtype of NRSTS is rare, they have been poorly studied and little is known about their biology, natural history, or optimal treatment. Data from adults with soft tissue sarcomas provide some helpful insight, but adult and childhood NRSTSs differ considerably in the distribution of their histologic subtypes, and certain entities are known to behave differently in young children. Read More

Expert Rev Anticancer Ther 2008 Jun;8(6):929-38

Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale Tumori, Via G Venezian, 1 20133 Milano MI, Italy.

The definition of nonrhabdomyosarcoma soft-tissue sarcomas includes a varied group of malignant soft part tumors that can occur in childhood, but the majority are entities typically observed in adult age. Similar to their adult counterparts, pediatric nonrhabdomyosarcoma soft-tissue sarcomas are usually considered scarcely sensitive to chemotherapy, but treatment strategies for these tumors have changed to some degree in recent years, and multiple-modality treatments that also include chemotherapy have increasingly been attempted. Subsets of patients with specific histological subtypes and prognostic variables have been thought likely to benefit from chemotherapy. Read More

Cancer 2008 May;112(9):2052-9

Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.

Background: Few data exist regarding techniques, indications, and outcomes for sentinel lymph node biopsy in pediatric patients with sarcomas and carcinomas.

Methods: A retrospective 10-year review was conducted, with Institutional Review Board waiver, of the pathology, lymphoscintigraphy, and clinical records for all pediatric patients selected to undergo sentinel lymph node biopsy at a major cancer center.

Results: Thirty-one sentinel lymph node biopsies were performed in 30 pediatric patients (median age, 12 years; range, 2-21 years). Read More

Surg Oncol 2007 Nov 17;16(3):187-93. Epub 2007 Aug 17.

Department of Pediatric Surgery, KK Women's and Children's Hospital, 100 Bukit Timah Road, Singapore 229 899, Singapore.

Nonrhabdmyosarcoma soft tissue sarcoma (NRSTS) is a heterogenous group of tumors analyzed as a unique group because of the rarity of each histopathological subtype. Initial information available has been obtained from adult series or pediatric case studies. Recent reports by several multi-center groups have been published that evaluated prognostic factors and treatment protocols. Read More

Future Oncol 2006 Oct;2(5):667-73

Medical University of Gdansk, Department of Surgery & Urology for Children & Adolescents, Ul. Nowe Ogrody 1-6, 80-803 Gdansk, Poland.

Pediatric soft tissue sarcomas (STSs), of which rhabdomyosarcoma is the most common, constitute approximately 5-6% of all cancers in children. Biopsy, being incisional or rarely excisional, is an essential primary diagnostic step, while in some cases it may be followed by primary re-excision to establish a correct diagnosis in which molecular analysis can be helpful. Most European STS treatment groups favor preoperative chemotherapy, sometimes combined with preoperative irradiation. Read More

J Clin Oncol 2006 Apr;24(12):1958-9; author reply 1959-60

Expert Rev Anticancer Ther 2005 Apr;5(2):307-18

Istituto Nazionale Tumori, Via G.Venezian, 1-20133 Milan, Italy.

Nonrhabdomyosarcoma soft tissue sarcomas form a group of rare tumors with a different biology and clinical behavior. The recently established European Pediatric Soft Tissue Sarcoma Study Group is organizing a new study devoted specifically to these tumors that were formerly treated according to the principles derived from experience with rhabdomyosarcoma, which is a clearly distinct entity. The new study includes two prospective trials, one for synovial sarcoma and the other for adult-type nonrhabdomyosarcoma soft tissue sarcomas. Read More

AJR Am J Roentgenol 2005 Apr;184(4):1293-304

Department of Radiological Sciences, St. Jude Children's Research Hospital, 332 N Lauderdale St., Memphis, TN 38105, USA.

Objective: Our objective was to review our preliminary experience with PET/CT in evaluating childhood sarcomas including rhabdomyosarcoma (n = 28), the Ewing's sarcoma family of tumors (n = 14), nonrhabdomyosarcoma soft-tissue sarcoma (n = 9), osteosarcoma (n = 8), chondrosarcoma (n = 1), and embryonal sarcoma (n = 1).

Conclusion: We found PET/CT useful in depicting an unknown primary rhabdomyosarcoma and detecting unsuspected and unusual metastatic sites of childhood sarcomas. It was useful in monitoring response to chemotherapy, radiation therapy, and radiofrequency ablation and aided the postoperative evaluation of tumor resection sites. Read More

J Clin Oncol 2005 Jun 14;23(18):4021-30. Epub 2005 Mar 14.

Pediatric Oncology Unit, Department of Pathology, Istituto Nazionale Tumori, Via G. Venezian, 1 20133 Milano, Italy.

Purpose: Nonrhabdomyosarcoma soft tissue sarcomas are a heterogeneous group of tumors for which optimal treatment remains controversial. We report on a large group of 182 patients younger than 18 years old treated at a single institution over a 25-year period.

Patients And Methods: In this relatively homogeneous subgroup of adult-type histotypes, surgery was the mainstay of treatment; radiotherapy was administered to 73 patients, and chemotherapy was administered to 114 patients (70 received chemotherapy as adjuvant therapy). Read More

Pediatr Blood Cancer 2005 May;44(5):449-54

National Cancer Institute, Pediatric Oncology Branch, Bethesda, MD 20892, USA.

Background: The survival of children and adolescents with advanced (unresectable or metastatic) nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) is poor. In order to clarify the role of combining chemotherapy with aggressive local control using surgery and/or radiation, we reviewed our institutional experience with the treatment of advanced pediatric NRSTS.

Procedure: We reviewed the charts of all patients less than 21 years treated for an advanced NRSTS at the National Cancer Institute (NCI) between 1983 and 2003. Read More

Pediatr Blood Cancer 2004 Oct;43(5):587-93

Department of Radiation Oncology, Emory University, Atlanta, Georgia, USA.

Objective: To determine the value of postoperative radiotherapy (RT) in the management of nonrhabdomyosarcoma soft tissue sarcoma (NRSTS) of childhood.

Procedure: From 1964 to 2000, 62 children with a median age of 14 years were seen at the University of Iowa and underwent a wide local excision for non-metastatic NRSTS. Tumors were high grade in 36 (58%) and >5 cm in 24 (39%). Read More

Expert Rev Anticancer Ther 2004 Apr;4(2):247-56

Department of Radiation Oncology, Emory Clinic, 1365 Clifton Road, NE, A1300, Atlanta, GA 30322, USA.

Nonrhabdomyosarcoma soft tissue sarcomas account for approximately 40% of all soft tissue sarcomas in children. Although these tumors are typically grouped together, individual tumor types with different biological characteristics have been found that may impact on the optimal therapy for each type of sarcoma in the future. Most of the current information regarding this tumor comes from the adult literature. Read More

Int J Radiat Oncol Biol Phys 2003 Sep;57(1):177-83

Department of Radiation Oncology, Emory University, Atlanta, Georgia, USA.

Purpose: To determine the incidence and prognosis of children who develop brain metastasis.

Materials And Methods: The medical and tumor registry records of 611 children treated at the University of Iowa Hospitals and Clinics between 1965 and 2000 for a sarcoma, neuroblastoma, or Wilms' tumor were reviewed.

Results: Thirty children (4. Read More