Treatment Options for Stages I and II
Hepatoblastoma of pure fetal histology: For tumors of pure fetal histology, complete surgical resection followed by watchful waiting or single-agent doxorubicin.In the Children's Oncology Group (COG) study COG-P9645, stage I pure fetal histology hepatoblastomas with two or fewer mitoses per 10 high power fields were not treated with chemotherapy. Completely excised tumor of purely fetal and favorable histology may be carefully followed without...
Updated statistics with estimated new cases and deaths for 2013 (cited American Cancer Society as reference 1).
Added text to state that even in Lynch syndrome (LS), a hereditary form of colon cancer, cigarette smoking has been identified as a risk factor for the development of colorectal adenomas (cited Winkels et al. as reference 66).
Revised text to state that a variety of LS-associated mutations in MSH2 and MLH1 have been identified; these include founder mutations in the Ashkenazi Jewish, Finnish, Portuguese, and German American populations (cited Pinheiro et al. and Tomsic et al. as references 31 and 32, respectively).
Added De novo mutation rate as a new subsection.
Major Genetic Syndromes
Added Khan et al. as reference 251.
Added text to state that one study of two families with the same EPCAM deletion found few extracolonic cancers and no endometrial cancers (cited Lynch et al. as reference 275).
Added text about a more efficient screening strategy that has been suggested in which staining for only PMS2 and MSH6 are performed (cited Hall et al. as reference 285), although staining for all four mismatch repair proteins remains the current standard of care.
Added text to state that even in centers that rely exclusively on immunohistochemistry (IHC) testing, there may be a role for subsequent microsatellite instability (MSI) testing in cases where the clinical picture suggests LS, notwithstanding the results of IHC.
The Surgical management in LS subsection was renamed from Risk-reducing surgery in LS.
Added text about a quality-of-life and functional outcome survey of LS patients in which global quality-of-life outcomes were comparable among patients with more extensive and less extensive resections (cited Haanstra et al. as reference 350); this parallels the experience in familial adenomatous polyposis (FAP) patients (cited Church et al., Hassan et al., Aziz et al. as references 351, 352, and 353, respectively).
Added Chromoendoscopy as a new subsection.
Added Small Bowel Imaging as a new subsection.
Psychosocial Issues in Hereditary Colon Cancer Syndromes
This section was renamed from Psychosocial Issues in Hereditary Colon Cancer Syndromes: Lynch Syndrome and FAP.
Revised text to state that from the limited studies published to date, there appears to be interest in the use of assisted reproductive technology (ART) for FAP, LS, and Peutz-Jeghers syndrome (PJS) (cited Dewanwala et al. and van Lier et al. as references 29 and 30, respectively).
Revised Table 15, Summary of Studies Evaluating Attitudes Toward, Interest in, or Intention to Use ART for FAP, LS, and PJS, to include a study in LS patients and a study in PJS patients.
Added Issues With Informed Consent for MSI and IHC Tumor Testing as a new subsection.
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