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胎儿水肿时先天性膈肌横纹肌肉瘤的产前处理。

Prenatal management of diaphragmatic rhabdomyosarcoma presenting with fetal hydrops.

机构信息

Obstetric and Gynaecology Department, University Hospital Frankfurt, Frankfurt, Germany.

出版信息

Ultrasound Obstet Gynecol. 2012 Aug;40(2):235-7. doi: 10.1002/uog.10113. Epub 2012 Jul 9.

DOI:10.1002/uog.10113
PMID:21997954
Abstract

Fetal malignant tumors are rare. We present a case of intrauterine diagnosis of a diaphragmatic tumor presenting with fetal hydrops at 32 weeks' gestation. The sonographic findings were bilateral pleural effusion, ascites and skin edema. A large right-sided diaphragmatic tumor was identified. Owing to the findings on ultrasound and magnetic resonance imaging a solid malignant tumor was suspected. The pleural effusions were drained and malignant cells identified. Because of rapid tumor progression Cesarean section was performed and a hydropic female newborn was delivered at 34 + 0 weeks' gestation. There was no sign of metastatic disease. Postnatally tumor biopsy revealed an alveolar rhabdomyosarcoma. Therapy included chemotherapy and secondary surgical intervention. After a good primary response with complete remission after 6 months, the rhabdomyosarcoma relapsed at 12 months with cerebral metastasis. The prognosis was poor.

摘要

胎儿恶性肿瘤较为罕见。我们报告一例胎儿水肿的 32 周宫内诊断为膈肌肿瘤的病例。超声表现为双侧胸腔积液、腹水和皮肤水肿。发现右侧膈肌有一个大肿瘤。由于超声和磁共振成像的结果,怀疑为实性恶性肿瘤。胸腔积液被引流,发现恶性细胞。由于肿瘤快速进展,行剖宫产术,34+0 周时分娩出一名患有水肿的女婴。没有转移疾病的迹象。产后肿瘤活检显示肺泡横纹肌肉瘤。治疗包括化疗和二次手术干预。在 6 个月后完全缓解的原发性反应良好后,横纹肌肉瘤在 12 个月时复发并伴有脑转移。预后较差。

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