原发性纵隔卵黄囊瘤7例

doi:10.19723/j.issn.1671-167X.2019.06.020

摘要/Abstract

摘要：

目的总结原发性纵隔卵黄囊瘤诊治经验。方法选择北京大学第一医院胸外科2014年8月至2018年8月收治的7例原发性纵隔卵黄囊瘤患者临床及病理资料,进行回顾性分析。结果 7例患者胸部CT显示前纵隔肿物,血清甲胎蛋白(alphafetoprotein, AFP) 水平均明显升高。5例术前接受穿刺活检,诊断为卵黄囊瘤,接受含铂双药或三药方案化疗后行肿瘤扩大切除,其中4例术后继续进行化疗;另2例患者术前未接受穿刺活检,术后病理诊断为卵黄囊瘤,并接受含铂方案化疗。7例患者均顺利完成手术,6例达到R0切除,1例为R1切除。2例出现术后并发症(1例肺炎和1例肺不张)。3例术后1年内出现肺转移,4例未出现复发和转移。结论原发性纵隔卵黄囊瘤临床罕见,恶性程度较高,经合理诊治后部分患者可获得长期生存。

关键词: 原发性纵隔卵黄囊瘤, 化疗, 扩大切除

Abstract:

Objective: Primary mediastinal yolk sac tumor, which is also known as endodermal sinus tumor, is a rare but lethal neoplasm and its prognosis is very dismal. The current treatment for this tumor is controversial, and chemotherapy combined with resection of residual lesions is adopted sometimes. We summarized the experience of seven primary mediastinal yolk sac tumors treated with platinum-based chemotherapy and extended resection in Peking University First Hospital.Methods: Clinicopathological data of the patients with primary mediastinal yolk sac tumor who received operation in Peking University First Hospital between August 2014 and August 2018 were collected and analyzed retrospectively.Results: We experienced seven primary mediastinal yolk sac tumors during this period. Computed tomography scan revealed an anterior mediastinal tumor in all the patients and all of them had markedly raised alphafetoprotein (AFP) and normal β-human chorion gonadotropin (β-HCG). Five patients underwent needle core biopsy before treatment, which showed a mediastinal yolk sac tumor. All of these patients received preoperative platinum-based chemotherapy and they all presented partial response according to computed tomography. Two other patients did not receive preoperative biopsy, so they directly underwent extended resection. All of the seven patients underwent operation successfully and two of them expe-rienced postoperative complications, including one with pneumonia and the other with atelectasis. R0 resection was achieved in six patients and R1 resection was achieved in the other patient. According to postoperative pathology, there were one microcyst subtype, one adenoid subtye, one giant capsule subtype and two hybrid subtypes. Surprisingly, there were no yolk sac tumor tissue in the other two patients after preoperative chemotherapy. All the patients received postoperative chemotherapy, excluded one patient who was unable to tolerate chemotherapy after operation. Three patients experienced postoperative pulmonary metastases within one year and two of them died soon. The other patient received chemotherapy and immunotherapy after recurrence and he was alive at the time of writing. Four other patients were alive without recurrence and metastasis.Conclusion: Primary mediastinal yolk sac tumor is rare and its prognosis is poor. A multimodality approach including adjuvant chemotherapy and resection of residual lesions is the optimal treatment and it may lead to long-term survival.

Key words: Primary mediastinal yolk sac tumor, Chemotherapy, Extended resection

中图分类号:

R736

马义祥,刘敬伟,齐康,张继新,林钢,刘海波,商学谦,李简. 原发性纵隔卵黄囊瘤7例[J]. 北京大学学报（医学版）, 2019, 51(6): 1091-1095.

Yi-xiang MA,Jing-wei LIU,Kang QI,Ji-xin ZHANG,Gang LIN,Hai-bo LIU,Xue-qian SHANG,Jian LI. Diagnosis and treatment of seven primary mediastinal yolk sac tumors[J]. Journal of Peking University(Health Sciences), 2019, 51(6): 1091-1095.

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参考文献 19

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Case	Age/years	Symptom	AFP before chemotherapy/ (μg/L)	Preoperative chemotherapy (cycle, drug)	AFP after chemotherapy/ (μg/L)	Surgical approach	Excision extension	Surgical margin	Subtype	Postoperative chemotherapy (cycle, drug)	Recurrence location	Status^*	Survival/ months
1	16	Chest distress fever	9 545.00	4×PEB	4.84	LMSAT	Tumor+partial pericardium+ phrenic nerve	R0	Not clear	2×PEB	No	Alive	48
2	17	Chest distress chest pain	285.50	4×EP	13.31	MS	Tumor+partial pericardium+ partial pulmonary	R0	Microcyst	2×PEB	Pulmonary	Alive	39
3	32	No	2 697.00	-	-	RMSAT	Tumor+partial pericardium	R0	Adenoid	4×PEB	Pulmonary	Dead	11
4	35	Chest distress SVCS	12 045.00	6×PEB	1 117.00	MS	Tumor+partial pericardium and pulmonary+superior vena cava	R1	Hybird	No	Pulmonary	Dead	5
5	14	Cough chest pain	32 105.00	6×PEB	10.91	RMSAT	Tumor+thymoma	R0	Giant capsule	2×PEB	No	Alive	23
6	22	Cough	1 210.00	-	-	LMSAT	Tumor+ phrenic nerve	R0	Hybird	4×EP	No	Alive	15
7	22	Fever chest pain	42 034.00	2×PEB	261.60	MS	Tumor+partial pericardium and pulmonary+superior vena cava	R0	Not clear	4×PEB	No	Alive	13

Case	Vimentin	CK(AE1/AE3)	AFP	SALL4	PLAP	CD117	CK7	OCT4	Ki-67/%
1	-	+	+	-	-	+	-	-	70
2	-	+	+	-	+	-	-	-	50
3	-	+	+	+	+	+	-	-	70
4	-	+	+	+	+	-	-	-	70
5	-	+	+	+	-	+	+	-	60
6	-	+	+	+	-	+	-	-	60
7	-	+	+	-	-	-	-	-	60