Lécuru F, Ansquer Y, Bats A-S, Olschwang S, Laurent-Puig P, Eisinger F
Service de chirurgie gynécologique et cancérologique, hôpital européen Georges-Pompidou, AP-HP, Paris, France.
J Gynecol Obstet Biol Reprod (Paris). 2008 Oct;37(6):547-53. doi: 10.1016/j.jgyn.2008.05.010. Epub 2008 Jul 22.
The Hereditary Non-Polyposis Colorectal Cancer syndrome (HNPCC) has initially been described as a predisposition to colorectal cancers (CRC). Subsequently, other cancers, such as endometrial cancers (EC), have been added. The objective of this review was to update data on endometrial cancers of HNPCC syndrome. Endometrial cancers of the HNPCC syndrome are characterized by a younger age at diagnosis (46-48 year old), and a higher cumulative risk along life (30% at 70 years). Complex atypical hyperplasia seems to occur before the cancer, but the transition between precursors and cancer seems to be short. Histology of endometrial cancers of the HNPCC syndrome appears quite similar to that of sporadic cases, except for non-endometrioid lesions which seem more frequent and could occur in younger women. Screening of endometrial cancer in predisposed women should associate annual clinical examination, transvaginal sonography and endometrial sampling. Unfortunately, available data on screening by sonography show that this test seems poorly accurate, with no asymptomatic cancer or hyperplasia recognized and interval cancers between screenings. Endometrial biopsy appears as the most interesting method, since 11 asymptomatic cancers and 14 hyperplasia have been diagnosed in 175 mutation carriers. Diagnostic hysteroscopy seems also interesting, but requires further evaluation. Prophylactic hysterectomy confers a complete protection against endometrial cancer. However, perioperative morbidity (especially in women with history of colorectal surgery) and long-term effects of ovarian suppression should also be considered. Screening of endometrial cancer remains the main objective of the management of those patients. Endometrial biopsy should have a larger place.
遗传性非息肉病性结直肠癌综合征（HNPCC）最初被描述为易患结直肠癌（CRC）。随后，其他癌症，如子宫内膜癌（EC），也被纳入其中。本综述的目的是更新关于HNPCC综合征子宫内膜癌的数据。HNPCC综合征的子宫内膜癌具有诊断时年龄较轻（46 - 48岁）以及一生中累积风险较高（70岁时为30%）的特点。复杂的非典型增生似乎在癌症发生之前出现，但前驱病变与癌症之间的转变似乎很短暂。HNPCC综合征子宫内膜癌的组织学表现与散发性病例相当相似，除了非子宫内膜样病变似乎更常见且可能发生在年轻女性中。对易感女性进行子宫内膜癌筛查应结合每年的临床检查、经阴道超声检查和子宫内膜取样。不幸的是，关于超声筛查的现有数据表明，这项检查的准确性似乎很差，未发现无症状癌症或增生，且筛查期间出现间隔癌。子宫内膜活检似乎是最有效的方法，因为在175名突变携带者中已诊断出11例无症状癌症和14例增生。诊断性宫腔镜检查似乎也很有效，但需要进一步评估。预防性子宫切除术可完全预防子宫内膜癌。然而，围手术期发病率（尤其是有结直肠手术史的女性）以及卵巢抑制的长期影响也应予以考虑。子宫内膜癌筛查仍然是这些患者管理的主要目标。子宫内膜活检应发挥更大的作用。
