Response of brain tumours (BTs) to chemotherapy in CMMR-D
| Author /year | Brain tumour (gene defect) | Age (years) | Surgery | Chemotherapy | Radiotherapy | Response | Outcome | Status |
|---|---|---|---|---|---|---|---|---|
| Menko/2004 | Oligodendroma (MSH6) | 10 | Partial resection | Not specified | Yes | Partial remission | Recurrence of BT | Died at 12 years from BT |
| Gurugangan/2007 | Astrocytoma grade III (PMS2) | 19 | Subtotal resection | Temozolonide after RT | Yes | Resistant | New BT | |
| 19 | Carmustine and irinotecan | “Very good” partial remission | 18 months later minimal uptake scan | Died 22 months later from progression BT | ||||
| Scott/2007 | Medulloblastoma (MSH6) | 7 | Not specified | SIOP PNET 3: vincristine, cyclophosphamide etoposide, carboplatin | Yes spinal cord | Complete response | Alive at 13 years | |
| Tan/2008 | Glioblastoma (PMS2) | 8 | No | Temozolomide later: temozolomide and lomustin | Yes | Resistant | Leptomeningeal disease | Died 10 months after diagnosis |
| Etzler/2008 | Medulloblastoma (MSH6) | 6 | Gross total resection | Cisplatin CCNU vincristine | Yes spinal cord | Not reported | MDS/AML at 9 | Died 36 months later |
| Kruger/2008 | Glioblastoma (PMS2) | 6 | Surgical resection | Yes not specified | Yes | Complete remission | Recurrent BT 10 years later | Died at age 16 years from relapse of BT |
| Kruger/2008 | Glioblastoma (PMS2) | 6 | Surgical resection | Yes not specified | Yes | Resistant | Unresectable tumour | Died at age 7 years |
| Ilencikova/2011 | Fibrillar astrocytoma (MSH6) | 11 | No | POG 9233/34 | Yes | Resistant | <1 year new tumour, anaplastic astrocytoma III | |
| 11 | HIT-AGG-2007 plus temozolomide | Yes | Resistant | Died 3 months later | ||||
| Ilencikova/2011 | Glioblastoma (MSH6) | 10 | Surgical resection | Temozolomide | Yes | Resistant | Progression | Died at age 11 years |
| Johannes-ma/2011 | Cystic glioma (PMS2) | 11 | Resection | No | No | Recurrence after 7 months | ||
| 11 | Debulking | Vincristine, carboplatin, VP16, etoposide | Yes | Partial response | New tumour after 2 years, 8 months, high grade glioma | |||
| 14 | Carboplatin, melfalan, bone marrow transplantation | Yes | Partial response | Recurrence 3 years, 4 months later | ||||
| 18 | temozolomide | Yes | Resistant | Died at age 19 years | ||||
| Baas/2013 | Glioblastoma (PMS2) | 3 | Surgical resection | Temozolomide | Yes | Partial response; “26 months healthy” | Died at 5 years 6 months due to sepsis during chemotherapy for NHL | |
| Baas/2013 | Anaplastic astrocytoma/spin al cord (MLH1) | 2 years 10 months | Surgical resection | HIT-GBM-D protocol | Yes | Partial response | Died at 6 years and 3 months due to sepsis during chemotherapy for NHL | |
| Lindsay/2013 | Medulloblastoma and metastatic CRC (PMS2) | 12 | Complete resection | Folinic acid, 5FU, oxaliplatin for CRC | Yes craniospinal | Complete response | Died 2 years later from liver metastasis | |
| Yeung/2013 | Optic pathway glioma (PMS2) | 3 | No | Carboplatin, vincristine, | Partial response | GH-excess | Stable disease during 4½ years | |
| 8 | Vinblastine followed by temozolomide | Resistant | Increased 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.