Synchronous glioblastoma and medulloblastoma in a child with mismatch repair mutation. Childs Nerv Syst 2016 Mar;32(3):553-7
Date
08/22/2015Pubmed ID
26293676DOI
10.1007/s00381-015-2883-3Scopus ID
2-s2.0-84959363942 (requires institutional sign-in at Scopus site) 14 CitationsAbstract
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.
Author List
Amayiri N, Al-Hussaini M, Swaidan M, Jaradat I, Qandeel M, Tabori U, Hawkins C, Musharbash A, Alsaad K, Bouffet EAuthor
Monther Qandeel MBChB Assistant Professor in the Radiology department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
Antineoplastic Combined Chemotherapy ProtocolsBrain Neoplasms
Child, Preschool
Colorectal Neoplasms
Combined Modality Therapy
Cranial Irradiation
Fatal Outcome
Germ-Line Mutation
Glioblastoma
Humans
Male
Medulloblastoma
Mismatch Repair Endonuclease PMS2
MutL Protein Homolog 1
Neoplasms, Multiple Primary
Neoplastic Syndromes, Hereditary
Tumor Suppressor Protein p53
