Table 4

Response of brain tumours (BTs) to chemotherapy in CMMR-D

Author /yearBrain tumour (gene defect)Age (years)SurgeryChemotherapyRadiotherapyResponseOutcomeStatus
Menko/2004Oligodendroma (MSH6)10Partial resectionNot specifiedYesPartial remissionRecurrence of BTDied at 12 years from BT
Gurugangan/2007Astrocytoma grade III (PMS2)19Subtotal resectionTemozolonide after RTYesResistantNew BT
19Carmustine and irinotecan“Very good” partial remission18 months later minimal uptake scanDied 22 months later from progression BT
Scott/2007Medulloblastoma (MSH6)7Not specifiedSIOP PNET 3: vincristine, cyclophosphamide etoposide, carboplatinYes spinal cordComplete responseAlive at 13 years
Tan/2008Glioblastoma (PMS2)8NoTemozolomide later: temozolomide and lomustinYesResistantLeptomeningeal diseaseDied 10 months after diagnosis
Etzler/2008Medulloblastoma (MSH6)6Gross total resectionCisplatin CCNU vincristineYes spinal cordNot reportedMDS/AML at 9Died 36 months later
Kruger/2008Glioblastoma (PMS2)6Surgical resectionYes not specifiedYesComplete remissionRecurrent BT 10 years laterDied at age 16 years from relapse of BT
Kruger/2008Glioblastoma (PMS2)6Surgical resectionYes not specifiedYesResistantUnresectable tumourDied at age 7 years
Ilencikova/2011Fibrillar astrocytoma (MSH6)11NoPOG 9233/34YesResistant<1 year new tumour, anaplastic astrocytoma III
11HIT-AGG-2007 plus temozolomideYesResistantDied 3 months later
Ilencikova/2011Glioblastoma (MSH6)10Surgical resectionTemozolomideYesResistantProgressionDied at age 11 years
Johannes-ma/2011Cystic glioma (PMS2)11ResectionNoNoRecurrence after 7 months
11DebulkingVincristine, carboplatin, VP16, etoposideYesPartial responseNew tumour after 2 years, 8 months, high grade glioma
14Carboplatin, melfalan, bone marrow transplantationYesPartial responseRecurrence 3 years, 4 months later
18temozolomideYesResistantDied at age 19 years
Baas/2013Glioblastoma (PMS2)3Surgical resectionTemozolomideYesPartial response; “26 months healthy”Died at 5 years 6 months due to sepsis during chemotherapy for NHL
Baas/2013Anaplastic astrocytoma/spin al cord (MLH1)2 years 10 monthsSurgical resectionHIT-GBM-D protocolYesPartial responseDied at 6 years and 3 months due to sepsis during chemotherapy for NHL
Lindsay/2013Medulloblastoma and metastatic CRC (PMS2)12Complete resectionFolinic acid, 5FU, oxaliplatin for CRCYes craniospinalComplete responseDied 2 years later from liver metastasis
Yeung/2013Optic pathway glioma (PMS2)3NoCarboplatin, vincristine,Partial responseGH-excessStable disease during 4½ years
8Vinblastine followed by temozolomideResistantIncreased activity scan, alive
  • 5FU, fluorouracil; AML, acute myeloid leukemia; CCNU, Lomustine; CMMR-D, constitutional mismatch repair-deficiency; CRC, colorectal cancer; GH, growth hormone; NHL, non-Hodgkin lymphoma, PNET, primitive neuroectodermal tumours.