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Synchronous glioblastoma and medulloblastoma in a child with mismatch repair mutation

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Abstract

Synchronous primary malignant brain tumors are rare. We present a 5-year-old boy with synchronous glioblastoma and medulloblastoma. Both tumor samples had positive p53 stain and loss of PMS2 and MLH1 stains. The child had multiple café au lait spots and a significant family history of cancer. After subtotal resection of both tumors, he received craniospinal radiation with concomitant temozolomide followed by chemotherapy, alternating cycles of cisplatin/lomustine/vincristine with temozolomide. Then, he started maintenance treatment with cis-retinoic acid (100 mg/m2/day for 21 days). He remained asymptomatic for 34 months despite a follow-up brain MRI consistent with glioblastoma relapse 9 months before his death. Cis-retinoic acid may have contributed to prolong survival in this child with a probable biallelic mismatch repair syndrome.

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Acknowledgments

We thank the family of this lovely child for their tremendous care and for allowing us to take care of him.

Conflict of interest

The authors declare that they have no competing interests.

Author information

Authors and Affiliations

  1. Department of Pediatrics, Hematology/Oncology, King Hussein Cancer Center, Queen Rania Al Abdullah Street, P.O. Box 1269, Al-Jubeiha, Amman, 11941, Jordan

    Nisreen Amayiri

  2. Department of Pathology, King Hussein Cancer Center, Amman, Jordan

    Maysa Al-Hussaini

  3. Department of Radiology, King Hussein Cancer Center, Amman, Jordan

    Maisa Swaidan & Monther Qandeel

  4. Department of Radiation Oncology, King Hussein Cancer Center, Amman, Jordan

    Imad Jaradat

  5. Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Onatrio, Jordan

    Uri Tabori & Eric Bouffet

  6. The Arthur and Sonia Labatt Brain Tumour Research Centre, Division of Pathology, The Hospital for Sick Children, Toronto, Ontario, Canada

    Cynthia Hawkins

  7. Department of Surgery/Neurosurgery, King Hussein Cancer Center, Amman, Jordan

    Awni Musharbash

  8. Department of Pediatrics, Salmanyia Medical Complex, Manama, Kingdom of Bahrain

    Khulood Alsaad

Authors
  1. Nisreen Amayiri
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  2. Maysa Al-Hussaini
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  3. Maisa Swaidan
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  6. Uri Tabori
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  7. Cynthia Hawkins
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  8. Awni Musharbash
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  9. Khulood Alsaad
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  10. Eric Bouffet
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Corresponding author

Correspondence to Nisreen Amayiri.

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Amayiri, N., Al-Hussaini, M., Swaidan, M. et al. Synchronous glioblastoma and medulloblastoma in a child with mismatch repair mutation. Childs Nerv Syst 32, 553–557 (2016). https://doi.org/10.1007/s00381-015-2883-3

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  • DOI: https://doi.org/10.1007/s00381-015-2883-3

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