纵隔生殖细胞肿瘤：7例儿童的临床情况及预后

De Backer A, Madern G C, Hakvoort-Cammel F G A J, Oosterhuis J W, Hazebroek F W J

Section of Pediatric Surgery, Academic Hospital, Free University of Brussels, Laarbeeklaan 101, 1090 Brussels, Belgium.

Eur J Pediatr Surg. 2006 Oct;16(5):318-22. doi: 10.1055/s-2006-924647.

BACKGROUND

Mediastinal germ cell tumors presenting during childhood are extremely rare. Publications on this entity are very scarce. This paper reports on the clinical presentations, method(s) of treatment, complications, results and outcomes in a series of children with mediastinal germ cell tumors.

METHODS

A retrospective chart review of 7 children treated between 1971 and 2001 for mediastinal germ cell tumor was carried out. Age at diagnosis and symptoms were recorded. Each patient's surgical treatment, peri- and postoperative complications, histological staging and final outcome were analysed.

RESULTS

The median age of the 4 boys and 3 girls was 3 years (range 21 months-15 years). The most frequent symptoms were respiratory distress, persistent coughing, thoracic pain and anorexia/weight loss. Four patients had histologically benign tumors (mature teratoma). Their sole treatment consisted of complete surgical excision of the tumor and (part of) the thymus using either median sternotomy or left-sided thoracotomy. Recovery was uneventful. No recurrences have been observed. All four are alive with no evidence of disease, between 2.5 and 29 years after treatment. Malignant tumors were observed in three patients (1 yolk sac tumor, 1 choriocarcinoma and 1 malignant teratoma). Treatment consisted of either biopsy or debulking followed by chemotherapy (and radiotherapy in 1 case). Two of them died from uncontrollable metastatic disease. The patient with yolk sac tumor survived; he is now in remission, 4 years after diagnosis.

CONCLUSIONS

Both this study and the literature review testify to the extreme rarity of mediastinal germ cell tumors in childhood. Children with this type of tumor usually are severely symptomatic. Histologically benign tumors carry an excellent prognosis provided surgical excision is complete. Histologically malignant tumors, on the other hand, have a worse prognosis. However, the use of platinum-based combination chemotherapy has considerably increased the survival rates.

背景

儿童期出现的纵隔生殖细胞肿瘤极为罕见。关于这一实体的出版物非常稀少。本文报告了一系列纵隔生殖细胞肿瘤患儿的临床表现、治疗方法、并发症、结果及转归。

方法

对1971年至2001年间接受纵隔生殖细胞肿瘤治疗的7名儿童进行回顾性病历审查。记录诊断时的年龄和症状。分析每位患者的手术治疗、围手术期及术后并发症、组织学分期和最终结局。

结果

4名男孩和3名女孩的中位年龄为3岁（范围21个月至15岁）。最常见的症状是呼吸窘迫、持续咳嗽、胸痛和厌食/体重减轻。4名患者组织学上为良性肿瘤（成熟畸胎瘤）。他们唯一的治疗方法是通过正中胸骨切开术或左侧开胸术完整切除肿瘤及（部分）胸腺。恢复过程顺利。未观察到复发。所有4名患者均存活，治疗后2.5至29年无疾病证据。3名患者观察到恶性肿瘤（1例卵黄囊瘤、1例绒毛膜癌和1例恶性畸胎瘤）。治疗包括活检或肿瘤减积术，随后进行化疗（1例患者接受放疗）。其中2例死于无法控制的转移性疾病。卵黄囊瘤患者存活；他目前处于缓解期，诊断后4年。