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根治性手术治疗先天性颅咽管瘤:病例报告及文献复习

Congenital craniopharyngioma treated by radical surgery: case report and review of the literature.

作者信息

Kageji Teruyoshi, Miyamoto Takeshi, Kotani Yumiko, Kaji Tsuyoshi, Bando Yoshimi, Mizobuchi Yoshifumi, Nakajima Kohei, Nagahiro Shinji

机构信息

Department of Neurosurgery, Tokushima Prefectural Kaifu Hospital, Ooaza-nakamura, Aza-honson 75-1, Mugi-cho, Kaifu-gun, Tokushima, 775-0006, Japan.

Department of Neurosurgery, School of Medicine, The University of Tokushima, Tokushima, Japan.

出版信息

Childs Nerv Syst. 2017 Feb;33(2):357-362. doi: 10.1007/s00381-016-3249-1. Epub 2016 Sep 26.

Abstract

PURPOSE

Craniopharyngiomas are 5-10 % of all pediatric tumors, but are seldomly encountered in the perinatal period. Only seven instances of a truly antenatal diagnosis of a congenital craniopharyngioma that subsequently underwent radical surgery have been reported. We present the case of a patient who received the diagnosis of a suprasellar tumor during the prenatal period and received radical surgery.

METHODS

We report a case of a neonatal craniopharyngioma treated surgically.

RESULTS

The pregnancy progressed uneventfully until a routine ultrasound at 37 weeks of gestation showed a 15 × 15 mm high echoic mass in the center of the fetal head. Neonatal Gd-enhanced T1-weighted MRI at 5 days of life showed a homogenously enhanced mass (16×22×15 mm) in the sellar and suprasellar lesion. As the tumor showed rapid growth at the 3rd month of life, the patient underwent a surgical treatment and the mass was totally removed. Three years later, the physical and mental development of the patient was normal, and Gd-MRI studies showed no tumor recurrence.

CONCLUSION

The present case is the eighth case of a truly antenatal diagnosis of a craniopharyngioma that underwent successful radical surgery. Craniopharyngioma is a benign tumor and thought to be a slow growing tumor in childhood. The results of radical surgery were very poor, and the mortality and morbidity rates were high in the previous reports due to the huge size of tumor at operation. The present case demonstrated the rapid growth in short interval of Gd-MRI. This is the first report of tumor kinetics of congenital craniopharyngioma with previous reports. The calculated tumor doubling time in our case was 37 days.

摘要

目的

颅咽管瘤占所有儿科肿瘤的5%-10%,但在围产期很少见。仅有7例先天性颅咽管瘤在产前得到确诊并随后接受根治性手术的报道。我们报告1例在产前被诊断为鞍上肿瘤并接受根治性手术的患者。

方法

我们报告1例接受手术治疗的新生儿颅咽管瘤病例。

结果

孕期进展顺利,直到妊娠37周常规超声检查发现胎儿头部中央有一个15×15mm的高回声肿块。出生5天时的新生儿钆增强T1加权磁共振成像(MRI)显示鞍区和鞍上病变有一个均匀强化的肿块(16×22×15mm)。由于肿瘤在出生后第3个月生长迅速,患者接受了手术治疗,肿块被完全切除。3年后,患者身心发育正常,钆增强MRI检查未显示肿瘤复发。

结论

本病例是第8例真正在产前诊断出颅咽管瘤并成功接受根治性手术的病例。颅咽管瘤是一种良性肿瘤,被认为在儿童期生长缓慢。以往报道中,由于手术时肿瘤体积巨大,根治性手术的效果很差,死亡率和发病率都很高。本病例显示了钆增强MRI在短时间内的快速生长情况。这是先天性颅咽管瘤肿瘤动力学的首次报道,并与以往报道进行了对比。我们病例中计算出的肿瘤倍增时间为37天。

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