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Desmoplastic small round cell tumor of the central nervous system: report of two cases and review of the literature

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Abstract

Desmoplastic small round cell tumor (DSRCT) is a malignant tumor often involving the abdominal and/or pelvic peritoneum. Only one fully documented example has arisen in the central nervous system (CNS). Herein, we describe two additional examples, fulfilling the morphologic, immunohistochemical, and molecular criteria (EWS/WT1 translocation) of DSRCT. Both arose in the cerebellopontine angle (CPA) and underwent spinal dissemination. Patient 1, a 37-year-old male, underwent a subtotal resection, and 2 years later died of recurrent disease with spinal dissemination. Patient 2, a 39-year-old man, presented with cerebellar and CPA lesions as well as spinal leptomeningeal deposits. After 27 months of adjuvant therapy, he is alive with progressive disease. In conclusion, CNS DSRCT follows a similar aggressive course as do peritoneal examples. Although rare, DSRCT warrants consideration in the differential diagnosis of “malignant small blue cell tumors” of the CNS.

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Acknowledgments

The authors thank Dr. Ricardo V. Lloyd for his critical review of the manuscript, Mrs. Julie Majerus for technical assistance, Mr. James Hopfenspirger for photographic expertise, and Mrs. Denise Chase for excellent secretarial support.

Conflict of interest statement

We declare that we have no conflict of interest.

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Authors and Affiliations

  1. Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street, SW, Rochester, MN, 55905, USA

    Luciano Neder, Bernd W. Scheithauer, Rhett P. Ketterling, Long Jin & Caterina Giannini

  2. Department of Neurosurgery, Bombay Hospital Institute of Medical Sciences, Mumbai, India

    Keki E. Turel

  3. Department of Laboratory Medicine, Abbott Northwestern Hospital, Minneapolis, MN, USA

    Mark A. Arnesen

  4. Department of Medical Oncology, Mayo Clinic, 200 First Street, SW, Rochester, MN, 55905, USA

    Timothy J. Moynihan

  5. Department of Neurosurgery, Mayo Clinic, 200 First Street, SW, Rochester, MN, 55905, USA

    Fredric B. Meyer

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  1. Luciano Neder
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Correspondence to Bernd W. Scheithauer.

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Neder, L., Scheithauer, B.W., Turel, K.E. et al. Desmoplastic small round cell tumor of the central nervous system: report of two cases and review of the literature. Virchows Arch 454, 431–439 (2009). https://doi.org/10.1007/s00428-009-0750-x

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  • DOI: https://doi.org/10.1007/s00428-009-0750-x

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