Pediatric clinical case of parameningeal undifferentiated pleomorphic sarcoma treated with chemoradiotherapy

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Abstract

Malignant fibrous histiocytoma (MFH) is a rare “NON-RMS-LIKE” pediatric tumor with highly malignant clinical behavior, high metastatic potential and overall 5-year survival around 65-70%. Soft tissue  sarcoma diagnostic work-up and optimal combination of systemic therapy and local therapy approaches are one of  the most challenging issues in modern pediatric oncology. To present a clinical case of parameningeal  undifferentiated pleomorphic sarcoma in 7-year old child treated with adaptive conformal radiotherapy  aimed at local tumor control. We describe a method of combined treatment of patient with parameningeal  UPS consisting of the intensity-modulated radiotherapy (IMRT) in the form of volumetrically-modulated arc- therapy (VMAT) aimed at local tumor control. The radiotherapy course was completed at the planned total  dose of 54 Gy. Acute skin and mucosal toxicity not more than Grade 2 (RTOG scale) was noted during the  treatment. A good partial response was recorded after chemoradiotherapy course had been finished.  Histological verification of UPS diagnosis is complicated as substantial amount of tumor tissue material is  required for immunohistochemical and cytogenetical studies. An aggressive surgical approach is a method  of choice for local control in parameningeal soft-tissue sarcomas. However the adaptive radiotherapy was  the only possible treatment method of local tumor control with significant decrease of high doses to adjacent normal organs and tissues.

About the authors

A. Y. Usychkina

National Scientific and Practical Center of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev

Author for correspondence.
Email: usychkina@gmail.com
ORCID iD: 0000-0001-8313-9056

MD, pediatric oncologist Department of Radiation Therapy

Russia 117997, Moscow, Samory Mashela st., 1 Tel.: +7 (916)-695-0460

Russian Federation

D. A. Kobyzeva

National Scientific and Practical Center of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev

Russian Federation

V. V. Gornostaev

National Scientific and Practical Center of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev

Russian Federation

A. A. Loginova

National Scientific and Practical Center of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev

Russian Federation

D. Y. Kachanov

National Scientific and Practical Center of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev

ORCID iD: 0000-0002-3704-8783
Russian Federation

S. R. Varfolomeeva

National Scientific and Practical Center of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev

Russian Federation

V. Y. Roshin

National Scientific and Practical Center of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev

Russian Federation

A. V. Nechesnyuk

National Scientific and Practical Center of Pediatric Hematology, Oncology and Immunology named after Dmitry Rogachev

Russian Federation

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Copyright (c) 2017 Usychkina A.Y., Kobyzeva D.A., Gornostaev V.V., Loginova A.A., Kachanov D.Y., Varfolomeeva S.R., Roshin V.Y., Nechesnyuk A.V.

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