Department of Pediatrics, The First Affiliated Hospital of Anhui Medical University, 218 Jixi Road, Hefei, 230022, Anhui Province, China.
Childs Nerv Syst. 2024 Jul;40(7):2245-2250. doi: 10.1007/s00381-024-06406-8. Epub 2024 Apr 18.
Craniopharyngioma is a common intracranial tumour in children. Clinical manifestations are related to hypothalamic/pituitary deficiencies, visual impairment, and increased intracranial pressure. Defects in pituitary function cause shortages of growth hormone, gonadotropin, corticotropin, thyrotropin, and vasopressin, resulting in short stature, delayed puberty, feebleness, lethargy, polyuria, etc. However, manifestations involving precocious puberty (PP) are rare.
In both patients, surgical resection was performed after the diagnosis of craniopharyngioma, and breast development occurred postoperatively at one month in one patient and at one year and three months in the other patient. Central precocious puberty (CPP) was diagnosed via relevant examinations. Leuprorelin was injected subcutaneously every 28 days, and changes in height, weight, bone age, gonadal ultrasound and sex hormones were recorded. During the follow-up of the two children, the sex hormone levels were significantly reduced, and significant acceleration in bone age was not observed.
CPP was induced by craniopharyngioma surgery, and treatment with gonadotropin-releasing hormone analogues (GnRHa) inhibited sexual development and bone age progression. More attention should be given to monitoring for CPP during long-term follow-up of craniopharyngiomas in the clinic.
颅咽管瘤是儿童常见的颅内肿瘤。临床表现与下丘脑/垂体功能减退、视力损害和颅内压增高有关。垂体功能缺陷导致生长激素、促性腺激素、促皮质激素、促甲状腺激素和血管加压素缺乏,导致身材矮小、青春期延迟、乏力、嗜睡、多尿等。然而,涉及性早熟(PP)的表现较为罕见。
两名患者均在诊断为颅咽管瘤后进行了手术切除,一名患者术后一个月出现乳房发育,另一名患者术后一年三个月出现乳房发育。通过相关检查诊断为中枢性性早熟(CPP)。皮下注射亮丙瑞林,每 28 天一次,并记录身高、体重、骨龄、性腺超声和性激素的变化。在两名儿童的随访过程中,性激素水平显著降低,骨龄无明显加速。
颅咽管瘤手术可诱发 CPP,促性腺激素释放激素类似物(GnRHa)治疗可抑制性发育和骨龄进展。在临床长期随访颅咽管瘤时,应更加关注 CPP 的监测。