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Primary intracranial soft tissue sarcomas in children, adolescents, and young adults: single institution experience and review of the literature

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Abstract

There is a paucity of literature reporting the outcome of intracranial sarcomas (IS) in children, adolescents, and young adults (CAYA). A multimodal therapeutic approach is commonly used, with no well-established treatment consensus. We conducted a retrospective review of CAYA with IS, treated at our institution, to determine their clinical findings, treatments, and outcomes. Immunohistochemistry (PDGFRA and EGFR) and DNA sequencing were performed on 5 tumor samples. A literature review of IS was also conducted. We reviewed 13 patients (median age, 7 years) with a primary diagnosis of IS between 1990 and 2015. Diagnoses included unclassified sarcoma (n = 9), chondrosarcoma (n = 2), and rhabdomyosarcoma (n = 2). Five patients underwent upfront gross total resection (GTR) of the tumor. The 5-drug regimen (vincristine, doxorubicin, cyclophosphamide, etoposide, and ifosfamide) was the most common treatment used. Nine patients died due to progression or recurrence (n = 8) or secondary malignancy (n = 1). The median follow-up period of the 4 surviving patients was 1.69 years (range 1.44–5.17 years). The 5-year progression-free survival and overall survival rates were 21 and 44 %, respectively. BRAF, TP53, KRAS, KIT, ERBB2, MET, RET, ATM, and EGFR mutations were detected in 4 of the 5 tissue samples. All 5 samples were immunopositive for PDGFRA, and only 2 were positive for EGFR. IS remain a therapeutic challenge due to high progression and recurrence rates. Collaborative multi-institutional studies are warranted to delineate a treatment consensus and investigate tumor biology to improve the disease outcome.

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Acknowledgments

We appreciate Dr. Adriana Olar’s help with the pathology review. She is currently supported by NIH/NCI training grant no. 5T32CA163185. Dr. Ossama Maher is also currently affiliated with the National Cancer Institute, Cairo University, Cairo, Egypt.

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Authors and Affiliations

  1. Department of Pediatrics, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd., Unit 87, Houston, TX, 77030, USA

    Ossama M. Maher, Soumen Khatua, Devashis Mukherjee & Wafik Zaky

  2. Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Adriana Olar & Alexander Lazar

  3. Department of Hematopathology, Molecular Diagnostic Laboratory, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Raja Luthra

  4. Department of Biostatistics, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Diane Liu & Jimin Wu

  5. Department of Radiology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA

    Leena Ketonen

Authors
  1. Ossama M. Maher
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  2. Soumen Khatua
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  4. Adriana Olar
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  5. Alexander Lazar
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  7. Diane Liu
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  8. Jimin Wu
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  10. Wafik Zaky
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Correspondence to Ossama M. Maher or Wafik Zaky.

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11060_2015_2027_MOESM1_ESM.tif

Supplementary Figure 1 Axial T1-weighted post-contrast image (A) from patient #9 shows a falx-based, homogeneously enhancing mass involving the adjacent brain, with falx thickening and a falx tail (arrows). Coronal T2-weighted image (B) from patient #11 shows a dura-based tumor in the skull base (arrow) with a large surrounding hematoma (dark area). Axial T1-weighted postcontrast image (C) from patient #13, obtained at initial diagnosis, shows an enhancing dura-based mass in the right temporal area with dural thickening and enhancement that was suspicious for vascular malformation. Axial T-weighted image (D) of the same patient 6 weeks later shows an enhancing dura-based tumor with intratumoral bleeding and fluid-fluid levels in the enlarged tumor. Supplementary material 1 (TIFF 395 kb)

Supplementary material 2 (DOCX 16 kb)

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Maher, O.M., Khatua, S., Mukherjee, D. et al. Primary intracranial soft tissue sarcomas in children, adolescents, and young adults: single institution experience and review of the literature. J Neurooncol 127, 155–163 (2016). https://doi.org/10.1007/s11060-015-2027-3

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  • DOI: https://doi.org/10.1007/s11060-015-2027-3

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