Goto T, Aoyama K
Department of Pediatric Surgery, National Okayama Hospital, Children's Medical Center.
Nihon Rinsho. 1995 Nov;53(11):2779-85.
Familial sacrococcygeal teratomas are very rare. Up to now, only 8 kindreds (31 patients, including 1 Japanese kindreds) have been reported. All cases are Altman type IV variety. The tumor complex consists of a presacral teratoma and sacrococcygeal bony anomalies. The most common symptoms are constipation and anorectal stenosis. Two patients died. One died of meningitis following removal of a large benign tumor because of communication with a spinal arachinoid space. The other, with a large benign tumor, died as a result of malignant change, 10 years later after the first operation. The incidence of malignant change of familiar sacrococcygeal teratoma is rare in comparison with that of non-familiar sacrococcygeal teratomas.
家族性骶尾部畸胎瘤非常罕见。截至目前，仅报道了8个家系（31例患者，包括1个日本家系）。所有病例均为阿尔特曼IV型。肿瘤复合体由骶前畸胎瘤和骶尾骨异常组成。最常见的症状是便秘和肛门直肠狭窄。有2例患者死亡。1例因与脊髓蛛网膜下腔相通，在切除一个大的良性肿瘤后死于脑膜炎。另1例有一个大的良性肿瘤，在首次手术后10年因恶变死亡。与非家族性骶尾部畸胎瘤相比，家族性骶尾部畸胎瘤恶变的发生率较低。
