Mulvany Nicholas J, Allen David G
Department of Anatomical Pathology, Austin Hospital, Heidelberg, Victoria, Australia.
Int J Gynecol Pathol. 2008 Jan;27(1):49-57. doi: 10.1097/pgp.0b013e31806219c5.
We present the surgical and pathological findings and follow-up of 5 women diagnosed with combined endometrioid and high-grade neuroendocrine carcinoma of large cell type (LCNEC) arising in the endometrium. The mean age of the women was 75 years (range, 50-88 years). Of the 5 tumors, 4 formed polypoid endometrial masses associated with extensive lymphovascular involvement of the myometrium by neoplastic cells. A single endometrial tumor was formed by LCNEC alone, and 4 tumors were composite with varying proportions formed by endometrioid (4/5) and small cell neuroendocrine carcinoma (1/5). In all 5 LCNEC tumor components, an insular growth pattern was noted, whereas a diffuse (solid) pattern was found in 4 tumors, a trabecular in 2, and rosettes/pseudorosettes in another 2. In all 5 tumors, the LCNEC tumor components were labeled with neuron-specific enolase (NSE). Four tumors were reactive for chromogranin A, CAM 5.2, and p53. Three tumors were labeled for AE1/AE3, CD56 (NCAM), p16, and cytokeratin 7. Synaptophysin was reactive in 2 tumors, and CD117 was found in only a single tumor. Of the 3 endometrioid tumor components examined, all were reactive for NSE. Two tumors were reactive for p16 and p53, 1 for CD56, but none for synaptophysin orchromogranin A. We conclude that LCNEC of the endometrium is a distinct clinicopathological entity with a poor prognosis irrespective of stage. The gross and histomorphological features are often suggestive, but confirmation requires immunoperoxidases, including NSE, synaptophysin, chromogranin A, p16, and p53. Combined endometrioid and high-grade LCNEC possess more characteristics of a type II than a type I endometrial carcinoma.
我们报告了5例诊断为子宫内膜样腺癌合并大细胞型高级别神经内分泌癌（LCNEC）的女性患者的手术、病理结果及随访情况。这些女性的平均年龄为75岁（范围50 - 88岁）。5例肿瘤中，4例形成息肉样子宫内膜肿物，肿瘤细胞广泛累及肌层淋巴管。1例子宫内膜肿瘤仅由LCNEC构成，4例为复合性肿瘤，由不同比例的子宫内膜样腺癌（4/5）和小细胞神经内分泌癌（1/5）组成。在所有5例LCNEC肿瘤成分中，均可见岛状生长模式，而4例肿瘤呈现弥漫（实性）模式，2例为小梁状模式，另2例为菊形团/假菊形团模式。所有5例肿瘤的LCNEC肿瘤成分均表达神经元特异性烯醇化酶（NSE）。4例肿瘤对嗜铬粒蛋白A、CAM 5.2和p53呈阳性反应。3例肿瘤表达AE1/AE3、CD56（NCAM）、p16和细胞角蛋白7。2例肿瘤突触素呈阳性反应，仅1例肿瘤检测到CD117。在检测的3例子宫内膜样肿瘤成分中，均表达NSE。2例肿瘤对p16和p53呈阳性反应，1例对CD56呈阳性反应，但均不表达突触素或嗜铬粒蛋白A。我们得出结论，子宫内膜LCNEC是一种独特的临床病理实体，无论分期如何，预后均较差。大体及组织形态学特征常具有提示意义，但确诊需要免疫过氧化物酶检测，包括NSE、突触素、嗜铬粒蛋白A、p16和p53。子宫内膜样腺癌合并高级别LCNEC具有更多II型而非I型子宫内膜癌的特征。
