title: Synchronous glioblastoma and medulloblastoma in a child with mismatch repair mutation.
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/m(2)/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.
authors: Department of Pediatrics, Hematology/Oncology, King Hussein Cancer Center, Queen Rania Al Abdullah Street, P.O. Box 1269, Al-Jubeiha, Amman, 11941, Jordan. namayiri@khcc.jo.
authors: Nisreen
authors: N
authors: Amayiri
authors: Department of Pathology, King Hussein Cancer Center, Amman, Jordan.
authors: Maysa
authors: M
authors: Al-Hussaini
authors: Department of Radiology, King Hussein Cancer Center, Amman, Jordan.
authors: Maisa
authors: M
authors: Swaidan
authors: Department of Radiation Oncology, King Hussein Cancer Center, Amman, Jordan.
authors: Imad
authors: I
authors: Jaradat
authors: Department of Radiology, King Hussein Cancer Center, Amman, Jordan.
authors: Monther
authors: M
authors: Qandeel
authors: Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Onatrio, Jordan.
authors: Uri
authors: U
authors: Tabori
authors: The Arthur and Sonia Labatt Brain Tumour Research Centre, Division of Pathology, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors: Cynthia
authors: C
authors: Hawkins
authors: Department of Surgery/Neurosurgery, King Hussein Cancer Center, Amman, Jordan.
authors: Awni
authors: A
authors: Musharbash
authors: Department of Pediatrics, Salmanyia Medical Complex, Manama, Kingdom of Bahrain.
authors: Khulood
authors: K
authors: Alsaad
authors: Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, Toronto, Onatrio, Jordan.
authors: Eric
authors: E
authors: Bouffet
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001932:Brain Neoplasms / Q000150:complications / Q000235:genetics* / Q000628:therapy; D002675:Child, Preschool; D015179:Colorectal Neoplasms / Q000150:complications* / Q000235:genetics; D003131:Combined Modality Therapy; D016371:Cranial Irradiation; D017809:Fatal Outcome; D018095:Germ-Line Mutation; D005909:Glioblastoma / Q000235:genetics* / Q000628:therapy; D006801:Humans; D008297:Male; D008527:Medulloblastoma / Q000235:genetics* / Q000628:therapy; D000070976:Mismatch Repair Endonuclease PMS2 / Q000235:genetics; D000070957:MutL Protein Homolog 1 / Q000235:genetics; D009378:Neoplasms, Multiple Primary / Q000235:genetics* / Q000628:therapy; D009386:Neoplastic Syndromes, Hereditary / Q000150:complications* / Q000235:genetics; D016159:Tumor Suppressor Protein p53 / Q000235:genetics
chemical_list: C501486:MLH1 protein, human; C495901:TP53 protein, human; D016159:Tumor Suppressor Protein p53; C094934:PMS2 protein, human; D000070976:Mismatch Repair Endonuclease PMS2; D000070957:MutL Protein Homolog 1
keywords: Biallelic mismatch repair syndrome; Cis-retinoic acid; Glioblastoma; Synchronous tumors