Groves A M, Fagg N L, Cook A C, Allan L D
Department of Fetal Cardiology, Guy's Hospital, London.
Arch Dis Child. 1992 Oct;67(10 Spec No):1189-92. doi: 10.1136/adc.67.10_spec_no.1189.
Since 1980, 11 examples of cardiac tumour have been detected in the fetus out of a total of 794 congenital cardiac malformations. Patients were referred because of fetal hydrops in two, a family history of tuberous sclerosis in two, and because of the detection of a tumour mass during a scan at the local hospital in seven. The gestational age range at presentation was from 20-34 weeks. Of eight fetuses where death occurred, the histological type was rhabdomyoma in seven and teratoma in one. In seven cases, the lesion appeared single and in four there were multiple tumours. In two of the cases of rhabdomyoma, other family members had evidence of tuberous sclerosis. Termination of pregnancy took place in four cases; of seven continuing pregnancies, spontaneous intrauterine death occurred in four, and three children are still alive. Two of the three survivors has the clinical picture of tuberous sclerosis. The last case is as yet only 1 month old. In summary, even where the lesion is single, the most likely diagnosis in fetal cardiac tumour is rhabdomyoma, with associated tuberous sclerosis. However, the characteristic features of this latter condition may not become evident until some months after birth.
自1980年以来,在总共794例先天性心脏畸形胎儿中,检测出11例心脏肿瘤。其中2例因胎儿水肿转诊,2例有结节性硬化症家族史,7例因当地医院扫描时发现肿瘤块而转诊。就诊时的孕周范围为20至34周。在8例死亡胎儿中,组织学类型7例为横纹肌瘤,1例为畸胎瘤。7例病变为单发,4例有多发性肿瘤。在2例横纹肌瘤病例中,其他家庭成员有结节性硬化症的证据。4例终止妊娠;在7例继续妊娠中,4例发生自然宫内死亡,3名儿童仍存活。3名幸存者中有2名有结节性硬化症的临床表现。最后1例患儿目前仅1个月大。总之,即使病变为单发,胎儿心脏肿瘤最可能的诊断也是横纹肌瘤,并伴有结节性硬化症。然而,后一种疾病的特征可能在出生后几个月才会显现出来。