Cheng W Y, Chang C S, Shen C C, Wang Y C, Sun M H, Hsieh P P
Department of Neurosurgery, Taichung Veterans General Hospital, Taichung, Taiwan.
Pediatr Neurosurg. 2001 May;34(5):247-51. doi: 10.1159/000056032.
Being the most common childhood tumor to involve the hypothalamus and pituitary gland, craniopharyngioma, a histologically benign tumor, is usually related to growth retardation and hypogonadism. The presentation of precocious puberty is very rare for a hypothalamic craniopharyngioma. Here, we report such a case. The female patient had presented with symptoms of menarche and breast development since she was 6 years old. Hormonal therapy with a gonadotropin-releasing hormone analogue was instituted to cease precocious puberty but was unsuccessful. Magnetic resonance imaging of the sella showed a 1-cm tumor in the hypothalamic area. Through a pterional approach, the tumor was removed en bloc with endoscope-assisted microsurgery. After the operation, our patient's symptoms improved, so the hormone therapy was discontinued. This extremely unique association of craniopharyngioma and precocious puberty, the causes and mechanisms involved along with the advantages of endoscope-assisted microsurgery are discussed.
颅咽管瘤是累及下丘脑和垂体的最常见儿童肿瘤,是一种组织学上的良性肿瘤,通常与生长发育迟缓及性腺功能减退有关。下丘脑颅咽管瘤出现性早熟的情况非常罕见。在此,我们报告这样一例病例。该女性患者自6岁起就出现了月经初潮和乳房发育的症状。采用促性腺激素释放激素类似物进行激素治疗以停止性早熟,但未成功。蝶鞍的磁共振成像显示下丘脑区域有一个1厘米的肿瘤。通过翼点入路,在内镜辅助显微手术下将肿瘤整块切除。术后,我们这位患者的症状有所改善,因此停止了激素治疗。本文讨论了颅咽管瘤与性早熟这种极其独特的关联、其中涉及的原因和机制以及内镜辅助显微手术的优势。