肺型原发性卵巢小细胞癌：6例病例分析及31例文献回顾

doi:10.24920/004183

摘要/Abstract

摘要：

目的肺型原发性卵巢小细胞癌（primary small cell ovarian cancer of pulmonary type，SCCOPT）是一种预后不良的罕见卵巢肿瘤。目前，以铂类为基础的方案是其化疗的标准方案。由于发病率低，有关SCCOPT的临床特点以及其他治疗方法的潜在益处的研究较少。本研究总结了SCCOPT的临床病理特征和治疗。

方法我们对2008年至2022年在甘肃省人民医院诊断的6例SCCOPT患者以及17篇英文文献和3篇中文文献报道的31例患者的临床、影像学、实验室和病理学特征进行了回顾性分析。

结果患者的中位年龄为56.00岁。大约80%的患者处于Ⅲ或Ⅳ期。在手术和术后化疗后，患者的预后不良，中位总生存期为12个月。瘤体为囊实性，显微镜下可见小圆形肿瘤细胞和坏死。免疫组织化学检测显示：SCCOPT均表达上皮标志物—CD56和Y染色体相关性高迁移群盒2（sex-determining region of Y chromosome-related high-mobility-group box 2，SOX-2），不表达雌激素受体、孕激素受体、波形蛋白、Leu-7和生长抑素受体2。超过80%的肿瘤表达突触素。只有少数肿瘤表达神经元特异性烯醇酶、嗜铬粒蛋白A和甲状腺转录因子-1。

结论 SCCOPT预后不良。SOX-2有可能成为诊断SCCOPT的生物标记物。

关键词: 卵巢小细胞癌, 肺型原发性卵巢小细胞癌, Y染色体相关性高迁移群盒2, 辅助化疗。

Abstract:

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.

Conclusions SCCOPT had a poor prognosis. SOX-2 could be a biomarker to be used to diagnose SCCOPT.

Key words: small cell carcinoma of the ovary, primary ovarian small cell carcinoma of pulmonary type, sex-determining region of Y chromosome-related high-mobility-group box 2, adjuvant chemotherapy

Xu Chen, Hong-Ling Liu, Jin-Sui Wang, Feng-Hui Zhao. Primary Ovarian Small Cell Carcinoma of Pulmonary Type: Analysis of 6 Cases and Review of 31 Cases in the Literatures[J].Chinese Medical Sciences Journal, 2023, 38(2): 130-137.

图/表 5

References	Age (yrs)	Clinical symptoms	Tumor staging	Tumor diameter (cm)	Operation	Chemotherapy	Overall survival time (months)
Eichhorn et al.^[3]	62	pelvic mass	ⅠA (no metastasis )	21.5	RSO	NR	4, die from disease
Eichhorn et al.^[3]	59	pelvic mass	ⅠA (no metastasis)	17.0	TAH, RSO	NR	NR
Eichhorn et al.^[3]	55	pelvic mass	ⅠA (no metastasis)	26.0	TAH, BSO	NR	NR
Eichhorn et al.^[3]	30	abdominal mass	ⅠC (no metastasis)	20.0	BSO	NR	6, die from disease
Eichhorn et al.^[3]	84	abdominal mass	ⅡB (peritoneal metastasis)	13.5	BSO	NR	1, die from disease
Eichhorn et al.^[3]	76	vaginal bleeding and pelvic mass	ⅢB (peritoneal metastasis)	NR	TAH, BSO	NR	12, die from disease
Eichhorn et al.^[3]	50	vaginal bleeding and abdominal mass	ⅢB (peritoneal metastasis)	NR	TAH, BSO	NR	NR
Eichhorn et al.^[3]	72	pelvic mass	ⅢB (peritoneal metastasis)	4.5	TAH, BSO	cisplatin, cyclophosphamide	NR
Eichhorn et al.^[3]	64	pelvic mass	ⅢB (peritoneal metastasis)	5.5	TAH, BSO	cisplatin, cyclophosphamide	12, die from disease
Eichhorn et al.^[3]	49	pelvic mass and ascites	ⅢB (peritoneal metastasis)	16.0	LSO	cisplatin, cyclophosphamid, doxorubicin	8, die from disease
Eichhorn et al.^[3]	46	abdominal pain and mass	ⅢC (peritoneal metastasis)	9.5	TAH, BSO	cisplatin, cyclophosphamide, etoposide	13, die from disease
Chang et al.^[4]	22	pelvic mass	ⅢA (peritoneal metastasis )	18.0	TAH, LSO	cisplatin, cyclophosphamide	19, die from disease
Fukunaga et al.^[5]	60	abdominal mass	Ⅳ (lung metastasis)	14.0	TAH, RSO	cisplatin, cyclophosphamide	10, die from lung metastasis
Lim et al.^[6]	28	vaginal bleeding, pelvic mass, and acute abdomen	ⅢB (peritoneal metastasis )	20.0	TAH, RSO	NR	NR
Mebis et al.^[7]	54	pelvic mass	ⅢA (peritoneal metastasis	12.0	TAH, BSO	cisplatin, etoposide	22, die from disease
Wang et al.^[20]	22	abdominal mass and ascites	IV (extrapelvic nodal metastasis)	4.3	TAH, BSO, LYM, OMT	NR	NR
Rund et al.^[10]	56	vaginal bleeding and ascites	Ⅳ (liver metastasis)	12.0	TAH, RSO	cisplatin, etoposide	7, die from brain metastasis
Rund et al.^[10]	39	pelvic mass	ⅢB (peritoneal metastasis)	15.0	TAH, BSO	Cisplatin, etoposide, taxol	16, die from disease
Marie et al^.[8]	32	abdominal mass and ascites	Ⅳ (lung metastasis)	20.0	TAH, BSO	paclitaxel, carbiplatin	15, die from lung metastasis
Suzuki et al.^[12]	49	pelvic mass	Ⅳ (liver metastasis)	15.0	TAH, BSO	paclitaxel, carbiplatin	25, die from lung metastasis
Tsolakidis et al.^[13]	55	abdominal mass and pain	Ⅳ (liver metastasis)	8.0	TAH, BSO, LYM	carboplatin, etoposide	21, die from brain metastasis
Ikota et al.^[15]	65	abdominal mass and pain	Ⅳ (liver metastasis)	18.0	NR	NR	6, die from brain metastasis
Kurasaki et al.^[14]	54	pelvic mass	ⅢA (peritoneal metastasis)	14.0	TAH, LSO, OMT	paclitaxel	22, die from disease
Oyang et al.^[21]	56	abdominal mass and pain	Ⅳ (brain metastasis)	5.0	TAH, BSO, LYM, OMT	cispatin, etoposide,	NR
Rubio et al.^[11]	80	pelvic mass	Ⅳ (liver metastasis)	20.0	TAH, BSO	cisplatin, etoposide, paclitaxel	21, die from lung metastasis
Hashimoto et al.^[16]	75	abdominal pain and ascites	Ⅳ (lung metastasis)	12.5	TAH, BSO, OMT	cisplatin, etoposide	NR
Kalampokas et al.^[17]	77	pelvic mass	ⅡB (peritoneal metastasis)	11.3	TAH, BSO	cyclophosphamide, doxorubicin	NR
Oneda et al.^[18]	72	abdominal mass and vaginal bleeding	ⅢC (peritoneal metastasis)	6.0	TAH, LSO, LYM	carboplatin	10, die from disease
Ishikawa et al.^[9]	50	pelvic mass and ascites	Ⅳ (omentum and extrapelvic nodal metastasis)	12.0	TAH, LSO, OMT	cisplatin, etoposide, paclitaxel	12, die from lung metastasis
Li et al.^[22]	45	abdominal pain and mass	Ⅳ (bone metastasis)	10.0	TAH, BSO, OMT, LYM	paclitaxel, etoposide	NR
Asom et al.^[19]	80	abdominal mass, ascites, and vaginal bleeding	Ⅳ (omentum and extrapelvic nodal metastasis)	18.5	TAH, RSO, LYM	carboplatin treatment after radiotherapy	16, die from lung metastasis
Case 1	65	pelvic mass and lower abdominal pain	ⅢA (peritoneal metastasis)	10.7	TAH, BSO	cisplatin, doxorubicin	27, die from disease
Case 2	42	abdominal mass and ascites	Ⅳ (liver metastasis)	13.6	TAH, BSO, OMT, LYM, AP	cisplatin, cyclophosphamide	10, die from brain metastasis
Case 3	53	abdominal mass and lower abdominal pain	ⅢB (peritoneal metastasis)	11.2	TAH, BSO, OMT, LYM	cisplatin, paclictaxel	16, die from disease
Case 4	79	pelvic mass and vaginal bleeding	ⅢB (peritoneal metastasis)	13.4	TAH, BSO, OMT	carboplatin, paclictaxel	24, die from disease
Case 5	61	pelvic mass and ascites	Ⅵ (omentum and lung metastasis)	15.8	TAH, BSO, OMT, LYM, AP	carboplatin, etoposide	9, die from lung metastasis
Case 6	73	abdominal mass and lower abdominal pain	ⅡA (fallopian tube involvement)	9.1	TAH, BSO	cisplatin, paclictaxel	NA

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