Veras Emanuela, Mao Tsui-Lien, Ayhan Ayse, Ueda Stefanie, Lai Hong, Hayran Mutlu, Shih Ie-Ming, Kurman Robert J
Department of Pathology, The Johns Hopkins University School of Medicine and Hospital, Baltimore, MD, USA.
Am J Surg Pathol. 2009 Jun;33(6):844-53. doi: 10.1097/PAS.0b013e31819c4271.
Ovarian clear cell carcinomas (CCC) typically present as large adnexal, stage I tumors and are generally considered highly malignant. They are frequently associated with endometriosis and, less often with clear cell adenofibromas. We hypothesized that CCCs are a heterogeneous group of tumors, some arising from a cyst and others from an adenofibroma. To test this hypothesis, 122 cases of CCC were retrieved from the surgical pathology files of National Taiwan University Hospital (74), The Johns Hopkins Hospital (23), and Serei Mikatahara General Hospital (23) (1985 to 2006). Cases were divided into 3 subgroups: (1) cystic, (2) adenofibromatous, and (3) indeterminate. Various features were analyzed including: age, race, laterality, tumor size, architectural pattern (papillary, tubulo-cystic, solid, mixed patterns), grade, mitotic index, association with endometriosis including atypical endometriosis/intraepithelial carcinoma, stage and survival. Nearly 70% of all the patients were diagnosed as stage I. The 2-year and 5-year survival (all stages) was 78% and 68%, respectively. Striking clinicopathologic differences were observed between cystic and adenofibromatous CCCs. Cystic CCC was more frequently diagnosed as stage I compared with adenofibromatous CCC (75% vs. 44%). Conversely, adenofibromatous CCCs were diagnosed more often in advanced stages (stages II-IV) compared with cystic CCCs (56% vs. 18%). Both the cystic and adenofibromatous CCC forms were associated with endometriosis and atypical endometriosis/intraepithelial carcinoma, but the frequency was much higher in the cystic group. Specifically, endometriosis was found in 91% of cystic CCCs and atypical endometriosis/intraepithelial carcinoma in 62% of these cases, whereas endometriosis was found in 44% of adenofibromatous CCCs and atypical endometriosis/intraepithelial carcinoma in 11% of cases. A predominantly papillary pattern was seen in 47% of cystic CCCs, whereas none of the adenofibromatous carcinomas displayed a predominantly papillary pattern. A more favorable outcome was observed for cystic CCCs compared with adenofibromatous CCCs (all stages) which was accounted for by the high proportion of stage I tumors. The 2-year and 5-year survival for the cystic CCCs was 82% and 77% and for the adenofibromatous CCCs (all stages), 62% and 37%, respectively. In summary, subdividing ovarian CCCs into cystic and adenofibromatous CCC reveals differences in a number of clinicopathologic features including their association with endometriosis, histologic patterns, stage distribution, and clinical behavior. Because there were a relatively small number of adenofibromatous CCCs in this series, additional cases must be studied to confirm these findings.
卵巢透明细胞癌（CCC）通常表现为较大的附件区I期肿瘤，一般被认为具有高度恶性。它们常与子宫内膜异位症相关，较少与透明细胞腺纤维瘤相关。我们推测CCC是一组异质性肿瘤，一些起源于囊肿，另一些起源于腺纤维瘤。为验证这一假设，我们从台湾大学医院（74例）、约翰霍普金斯医院（23例）和三河御殿场综合医院（23例）（1985年至2006年）的外科病理档案中检索了122例CCC病例。病例被分为3个亚组：（1）囊性，（2）腺纤维瘤样，（3）不确定型。分析了各种特征，包括：年龄、种族、肿瘤侧别、肿瘤大小、组织学模式（乳头状、管状囊性、实性、混合性模式）、分级、有丝分裂指数、与子宫内膜异位症的关联（包括非典型子宫内膜异位症/上皮内癌）、分期和生存率。几乎所有患者中有近70%被诊断为I期。2年和5年生存率（所有分期）分别为78%和68%。在囊性和腺纤维瘤样CCC之间观察到显著的临床病理差异。与腺纤维瘤样CCC相比，囊性CCC更常被诊断为I期（75%对44%）。相反，腺纤维瘤样CCC在晚期（II-IV期）的诊断率高于囊性CCC（56%对18%）。囊性和腺纤维瘤样CCC两种类型均与子宫内膜异位症和非典型子宫内膜异位症/上皮内癌相关，但在囊性组中的发生率要高得多。具体而言，91%的囊性CCC中发现有子宫内膜异位症，其中62%的病例发现有非典型子宫内膜异位症/上皮内癌，而44%的腺纤维瘤样CCC中发现有子宫内膜异位症，11%的病例发现有非典型子宫内膜异位症/上皮内癌。47%的囊性CCC呈现主要为乳头状模式，而腺纤维瘤样癌均未呈现主要为乳头状模式。与腺纤维瘤样CCC（所有分期）相比，囊性CCC观察到更有利的预后，这是由于I期肿瘤的比例较高。囊性CCC的2年和5年生存率分别为82%和77%，腺纤维瘤样CCC（所有分期）的2年和5年生存率分别为62%和37%。总之，将卵巢CCC分为囊性和腺纤维瘤样CCC揭示了许多临床病理特征的差异，包括它们与子宫内膜异位症的关联、组织学模式、分期分布和临床行为。由于本系列中腺纤维瘤样CCC的数量相对较少，必须研究更多病例以证实这些发现。
