SMALL-CELL CARCINOMA OF THE OVARY, HYPERCALCEMIC TYPE - A CLINICOPATHOLOGICAL ANALYSIS OF 150 CASES
SMALL-CELL CARCINOMA OF THE OVARY, HYPERCALCEMIC TYPE - A CLINICOPATHOLOGICAL ANALYSIS OF 150 CASES
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DOI：
10.1097/00000478-199411000-00004
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发表时间：
1994-11-01
影响因子：
5.6
通讯作者：
SCULLY, RE
中科院分区：
文献类型：
--
作者：
YOUNG, RH;OLIVA, E;SCULLY, RE
本文报告150例高钙血症型卵巢小细胞癌的临床和病理特点。患者年龄9~43岁，平均23.9岁。在79名患者中，有49名(62%)的血钙水平已知升高。其中四名患者有高钙血症症状，其中一名患者在发现卵巢肿瘤之前曾接受颈部探查，结果为阴性。开腹时肿瘤为单侧148例(99%)。大约一半的病例存在卵巢外扩散。肿瘤最大径6~26 cm，平均15.3 cm。显微镜检查显示多种形态，其中最常见的是弥漫的细胞片状，被不同数量的滤泡样腔穿透；肿瘤细胞也在巢、索、簇和单个中生长。滤泡样间隙出现在80%的病例中，含有几乎总是嗜酸性和稀疏性嗜碱性的液体。12%的肿瘤内可见粘液上皮细胞衬里的腺体或囊泡。肿瘤细胞小而圆，细胞核深染，有丝分裂活跃。然而，50%的肿瘤也有不同的细胞成分，有中等到丰富的嗜酸性细胞质，有时含有大的透明小球和大的核，通常比小细胞苍白，核仁更突出。免疫组织化学染色证实了肿瘤的上皮性，电子显微镜也证实了肿瘤的上皮性，其特征是显示大量扩张的粗面内质网。进行甲状旁腺激素相关蛋白免疫组织化学染色的7个肿瘤中，有5个肿瘤呈阳性结果。流式细胞术检查的23个肿瘤均为二倍体。在42例有随访信息的IA期患者中，14例(33%)术后1-13年(平均5.7年)仍然完好无病；23例死于疾病，通常在2年内；5例复发，但最后一次随访仍存活。肿瘤分期高于IA的患者几乎全部死于疾病，但1例IIB期患者接受了强化化疗和放射治疗，7年后存活并明显无病。IA期肿瘤的特征似乎与更有利的结果相关，包括年龄30岁，术前钙测定正常，肿瘤大小10厘米，以及没有大细胞。本系列中的肿瘤最初常常被误解为各种其他卵巢肿瘤，最常见的是成人或幼年颗粒细胞瘤或原始生殖细胞瘤，但小细胞癌的特征有助于将其与这些肿瘤和其他可能被混淆的肿瘤区分开来。对本系列各种治疗方法的分析表明，对于IA期肿瘤患者，包括双侧输卵管卵巢切除术在内的手术可能是最理想的。辅助治疗的作用尚不清楚。化疗和放疗联合治疗高分期和复发的肿瘤通常令人失望，但偶尔也会导致长期存活和可能的治愈。
The clinical and pathological features of 150 cases of ovarian small cell carcinoma of the hypercalcemic type are described. The patients ranged from 9 to 43 (average 23.9) years of age. The serum calcium level was known to be elevated in 49 of the 79 patients (62%) whose preoperative calcium levels were measured. Four of these patients had symptoms of hypercalcemia, and one of them had undergone neck exploration with negative results before the ovarian tumor was discovered. At laparotomy the tumor was unilateral in 148 cases (99%). Extraovarian spread was present in approximately half the cases. The tumors ranged from 6 to 26 (average 15.3) cm in greatest dimension. Microscopic examination disclosed various patterns, the most common of which was diffuse sheets of cells punctured by variable numbers of follicle-like spaces; the tumor cells also grew in nests, cords, clusters, and singly. The follicle-like spaces, which were present in 80% of the cases, contained fluid that was almost always eosinophilic and rarefy basophilic. Glands or cysts lined by mucinous epithelial cells were present in 12% of the neoplasms. The neoplastic cells were typically small and round with hyperchromatic nuclei and brisk mitotic activity. Fifty percent of the tumors, however, also had a variable component of cells with moderate to abundant amounts of eosinophilic cytoplasm, which sometimes contained large hyaline globules and large nuclei that were typically paler and had more prominent nucleoli than the small cells. Immunohistochemical staining confirmed the epithelial nature of the tumors, as did electron microscopy, which characteristically showed abundant dilated rough endoplasmic reticulum. Five of seven tumors investigated by immunohistochemical staining for parathyroid hormone-related protein showed positive results. All 23 tumors examined by flow cytometry with interpretable results were diploid. Fourteen of 42 patients (33%) with stage IA disease for whom follow-up information is available remained well and free of disease 1-13 (average 5.7) years postsurgery; 23 died of their disease, usually within 2 years; and five had recurrences but were alive at last follow-up. Almost all the patients with tumors of a stage higher than IA died of disease, but one patient with stage IIB disease who received intensive chemotherapy and radiation therapy is alive and apparently free of disease at 7 years. Features in stage IA tumors that appeared to be associated with a more favorable outcome included an age > 30 years, a normal preoperative calcium determination, a tumor size < 10 cm, and an absence of large cells. The tumors in this series were frequently misinterpreted initially as a variety of other ovarian neoplasms, most commonly adult or juvenile granulosa cell tumors or a primitive germ cell tumor, but the characteristic microscopic features of the small cell carcinoma facilitate its distinction from those tumors and others with which it may be confused. Analysis of the various types of therapy in the present series suggests that a procedure that includes bilateral salpingo-oophorectomy may be optimal for patients with stage IA tumors. The role of adjuvant therapy is unclear. Combination chemotherapy and radiation therapy for high-stage and recurrent tumors has been generally disappointing, but it has occasionally resulted in long-term survival and possible cure.