Familial Cancer Laboratory, QIMR, Herston, QLD, Australia.
Mod Pathol. 2012 May;25(5):722-30. doi: 10.1038/modpathol.2011.209. Epub 2012 Feb 10.
Debate continues as to the usefulness of assessing adenomas for loss of mismatch repair protein expression to identify individuals with suspected Lynch syndrome. We tested 109 polyps from 69 proven mutation carriers (35 females and 34 males) belonging to 49 Lynch syndrome families. All polyps were tested by immunohistochemistry for four mismatch repair proteins MLH1, MSH2, MSH6 and PMS2. Detailed pathology review was performed by specialist gastrointestinal pathologists. The majority of polyps (86%) were conventional adenomas (n=94), with 65 tubular and 28 tubulovillous adenomas and a single villous adenoma. The remaining 15 lesions (14%) were serrated polyps. Overall, loss of mismatch repair expression was noted for 78/109 (72%) of polyps. Loss of mismatch repair expression was seen in 74 of 94 (79%) conventional adenomas, and 4 of 15 (27%) serrated polyps from mismatch repair gene mutation carriers. In all instances, loss of expression was consistent with the underlying germline mutation. Mismatch repair protein expression was lost in 27 of 29 adenomas with a villous component compared with 47 of 65 adenomas without this feature (93 vs 73%; P=0.028). A strong trend was observed for high-grade dysplasia. Mismatch repair deficiency was observed in 12 of 12 conventional adenomas with high-grade dysplasia compared with 60 of 79 with low-grade dysplasia (100 vs 76%; P=0.065). We were unable to demonstrate a significant association between conventional adenoma size or site and mismatch repair deficiency. All (4/4 or 100%) of the serrated polyps demonstrating mismatch repair deficiency were traditional serrated adenomas from a single family. Diagnostic testing of adenomas in suspected Lynch syndrome families is a useful alternative in cases where cancers are unavailable. The overwhelming majority of conventional adenomas from mutation carriers show loss of mismatch repair protein expression concordant with the underlying germline mutation.
关于评估错配修复蛋白表达缺失以识别疑似林奇综合征个体的腺瘤的有用性仍存在争议。我们检测了 69 个经证实的突变携带者(35 名女性和 34 名男性)的 109 个息肉,这些携带者属于 49 个林奇综合征家族。所有息肉均通过免疫组织化学方法检测四种错配修复蛋白 MLH1、MSH2、MSH6 和 PMS2。由专门的胃肠病理学家进行详细的病理复查。大多数息肉(86%)为传统腺瘤(n=94),其中 65 个管状腺瘤和 28 个管状绒毛状腺瘤,单个绒毛状腺瘤。其余 15 个病变(14%)为锯齿状息肉。总体而言,109 个息肉中有 78/109(72%)的错配修复表达缺失。94 个传统腺瘤中有 74 个(79%)和 15 个锯齿状息肉中的 4 个(27%)来自错配修复基因突变携带者存在错配修复表达缺失。在所有情况下,表达缺失与潜在的种系突变一致。与无该特征的 65 个腺瘤相比,有绒毛成分的 29 个腺瘤中有 27 个(93%比 73%;P=0.028)的错配修复蛋白表达缺失。高级别异型增生也存在很强的趋势。在 12 个具有高级别异型增生的传统腺瘤中观察到错配修复缺陷,而在 79 个具有低级别异型增生的腺瘤中观察到 60 个(100%比 76%;P=0.065)。我们未能证明传统腺瘤大小或部位与错配修复缺陷之间存在显著关联。所有(4/4 或 100%)显示错配修复缺陷的锯齿状息肉均来自一个家族的传统锯齿状腺瘤。在无法获得癌症的疑似林奇综合征家族中,腺瘤的诊断性检测是一种有用的替代方法。突变携带者的绝大多数传统腺瘤均显示错配修复蛋白表达缺失,与潜在的种系突变一致。