Author/year | Type of tumour | Gene defect | Age (years) | Surgery | Chemotherapy | Radio therapy | Response | Status |
---|---|---|---|---|---|---|---|---|
Gallinger/2004 | 3× CRC | MLH1 | 9 | Subtotal colectomy (3 polyps) | Irinotecan, 5FU, leucovorin | No | No evidence recurrence | After 11 months alive |
Gururangan/2007 | CRC sigmoid | PMS2 | 14 | Pancolectomy | 5FU, leucovorin | Yes | No evidence recurrence | Died 7 years later from brain tumour |
Tan/2008 | CRC | PMS2 | 15 | Low anterior resection | FOLFOX4/12× | No | ||
Panproctocolectomy (3× CRC, >10 polyps in specimen) | Not reported | Not reported | ||||||
Jackson/2008 | CRC sigmoid liver metastases | PMS2 | 14 | Resection sigmoid, partial liver resection; colectomy | Oxaliplatin, capacitabine. | 5 months later recurrence rectal cancer | ||
14 | Resection rectal cancer, RFA liver metastases, | Irinotecan, 5FU, bevacizumab | Not reported | Alive | ||||
Kruger/2008 | Sigmoid CRC | PMS2 | 13 | Sigmoid resection, later panproctocolectomy (multiple polyps, CRC right colon) | FOLFOX/10 months | No | No evidence recurrence | Alive |
Rahner/2008 | 4× CRC | MSH6 | 17 | Colectomy | Cetuximab | No | Not reported | |
Kratz/2009 | CRC | PMS2 | 8 | Transversectomy | FOLFOX | Alive | ||
Toledano/2009 | Rectal cancer, 20 polyps | MSH2 | 14 | Panproctocolectomy (100 polyps) | FOLFOX | Yes | Relapse after 2 months | |
FOLFIRI/avastin | Died 12 months after diagnosis from brain tumour | |||||||
Vasovcak/2012 | Rectal cancer | PMS2 | 13 | Resection | Capecitabine, oxaliplatin, followed by cetuximab, irinotecan | Resistant after 4 cycles | ||
Yes | Died after short remission 17 months later | |||||||
Lindsay/2013 | CRC, liver metastases | PMS2 | 12 | Left hemicolectomy | Folinic acid, 5FU, oxaliplatin; later irinotecan, 5FU, Leucovorin | No | Partial remission | 2 years later new liver metastases; died from progressive leukoencephalopathy |
5FU, fluorouracil; CMMR-D, constitutional mismatch repair-deficiency; CRC, colorectal cancer; FOLFIRI, folinic acid, fluorouracil and irinotecan; FOLFOX, folinic acid, fluorouracil and oxaliplatin; RFA, radio frequency ablation.