原发性肺型卵巢小细胞癌6例分析及31例文献复习

Q2 Medicine Chinese Medical Sciences Journal Pub Date : 2023-06-01 DOI:10.24920/004183

Xu Chen , Hong-Ling Liu , Jin-Sui Wang , Feng-Hui Zhao

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引用次数: 0

摘要

目的原发性肺型卵巢小细胞癌（SCCOPT）是一种罕见的预后不良的卵巢肿瘤。以铂为基础的化疗是标准的治疗方法。然而，由于SCCOPT的发病率较低，对其临床特征和其他治疗的潜在益处的研究很少。本研究旨在探讨SCCOPT的临床病理特征和治疗方法。方法总结37例SCCOPT病例的临床、影像学、实验室和病理学特征，其中甘肃省医院2008年至2022年收治6例，17篇英文文献和3篇中文文献报道31例。结果研究的SCCOPT病例（n=37）的中位年龄为56.00岁（范围为22-80岁）。几乎80%的患者患有III期或IV期肿瘤。所有患者均接受了手术和术后化疗。然而，所有病例的预后都很差，中位总生存时间为12个月。免疫组化显示，所有患者的SCCOPT均显示上皮标志物阳性表达，如CD56和Y染色体相关高迁移率组box 2（SOX-2）的性别决定区，雌激素受体、孕激素受体、波形蛋白、Leu-7和生长抑素受体2阴性表达。80%以上的肿瘤表达突触素。只有少数病例表达神经元特异性烯醇化酶、嗜铬粒蛋白a和甲状腺转录因子-1。结论SCCOPT预后不良。SOX-2可作为诊断SCCOPT的生物标志物。

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Primary Ovarian Small Cell Carcinoma of Pulmonary Type: Analysis of 6 Cases and Review of 31 Cases in the Literatures

Objective

Primary ovarian small cell carcinoma of pulmonary type (SCCOPT) is a rare ovarian tumor with a poor prognosis. The platinum-based chemotherapy is the standard treatment. However, there is little research on the clinical characteristics of SCCOPT and the potential benefits of other treatments due to its low incidence. The study aims to investigate clinicopathological characteristics and treatment of SCCOPT.

Methods

We summarized the clinical, imaging, laboratorical and pathological characteristics of 37 SCCOPT cases, in which 6 cases were admitted to the Gansu Provincial Hospital from the year of 2008 to 2022 and 31 cases reported in 17 English and 3 Chinese literatures.

Results

The median age of the studied SCCOPT cases (n=37) was 56.00 (range, 22–80) years. Almost 80% of them had a stage III or IV tumor. All patients underwent an operation and postoperative chemotherapy. Nevertheless, all cases had a poor prognosis, with a median overall survival time of 12 months. Immunohistochemically, the SCCOPT of all patients showed positive expressions of epithelial markers, such as CD56 and sex-determining region of Y chromosome-related high-mobility-group box 2 (SOX-2), and negative expressions of estrogen receptor, progesterone receptor, vimentin, Leu-7, and somatostatin receptor 2. The tumor of above 80% cases expressed synaptophysin. Only a few cases expressed neuron-specific enolase, chromogranin A, and thyroid transcription factor-1.