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Comparison of hypothalamopituitary axis dysfunction of intrasellar and third ventricular craniopharyngiomas in
Yi-Yen Lee1, Tai-Tong Wong, Yi-Ting Fang
1Department of Pediatrics, Taipei City Hospital, Renai Branch, NO.10, Sec. 4, Renai Road, Taipei 106, Taiwan, ROC.
Insights
Tumor location in childhood craniopharyngiomas impacts hypothalamopituitary axis function. Intrasellar tumors cause more pituitary issues, while third ventricle tumors lead to greater long-term weight gain and hypothalamic dysfunction.
Area of Science:
- Pediatric Endocrinology
- Neuro-oncology
- Clinical Outcomes Research
Background:
- Craniopharyngiomas are pediatric brain tumors affecting the hypothalamopituitary axis.
- Tumor location is a critical factor influencing clinical presentation and endocrine outcomes.
Purpose of the Study:
- To investigate the influence of tumor location (intrasellar vs. third ventricle floor) on hypothalamopituitary axis function and outcomes in children with craniopharyngiomas.
- To compare the initial symptoms and long-term endocrinological disturbances based on tumor site.
Main Methods:
- Retrospective study of 66 children diagnosed with craniopharyngioma.
- Patients were stratified into two groups based on tumor location: intrasellar and third ventricle floor.
- Analysis of preoperative, postoperative, and long-term follow-up endocrinological data.
Main Results:
- Intrasellar tumors were associated with more frequent vision problems (55.6%) as an initial symptom compared to third ventricle tumors (15.4%).
- Increased intracranial pressure was more common in third ventricle tumors (51.3%) than intrasellar tumors (37%).
- While most patients required pituitary hormone supplements, intrasellar tumors led to a higher need for cortisone (79.2% vs. 45.9%).
- Third ventricle tumors were linked to increased weight gain (27.0% vs. 4.2%) and hypothalamic dysfunction at long-term follow-up.
Conclusions:
- Tumor location significantly impacts the initial presentation and long-term endocrinological outcomes in pediatric craniopharyngiomas.
- Intrasellar craniopharyngiomas are associated with more pronounced pituitary hormone disturbances.
- Third ventricle floor craniopharyngiomas present with distinct symptoms and are linked to hypothalamic dysfunction and weight gain in the long term.
Abstract:
In this study, we attempted to determine if different locations of a tumor influence the hypothalamopituitary axis function and outcomes with childhood craniopharyngiomas. The preoperative, postoperative, and long-term follow-up endocrinological disturbances of 66 children with a craniopharyngioma were retrospectively studied. The patients were divided into two subgroups according to the location of the tumor (intrasellar and third ventricle floor). The mean age at onset was 8.02 (range, 1.42-17.58)years. These patients were followed-up for a median duration of 7.2 (range, 2-22)years. Vision problems as the first symptom were more common in Group One (with intrasellar tumors) compared to Group Two (55.6% vs 15.4%; p=0.001; Fisher's exact test). Increased intracranial pressure was the most common initial symptom in patients in Group Two (51.3%) and the second most common symptom in Group One (37%). The majority of patients in both Group One and Group Two required some forms of pituitary hormone supplements (96% vs 84%). At the last follow-up, more patients with intrasellar craniopharyngiomas needed cortisone supplements (79.2% in Group One vs 45.9% in Group Two; p=0.016; Fisher's exact test); however, children with third ventricle floor tumors had more prevalent weight gain (4.2% in Group One vs 27.0% in Group Two; p=0.038; Fisher's exact test). There were different initial presentations and endocrinological outcomes between children with intrasellar and third ventricle floor craniopharyngiomas. The intrasellar tumors had greater pituitary hormone disturbance. However, at the long-term follow-up, children with third ventricle floor tumors had a greater prevalence of being overweight and obese, which was associated with hypothalamic dysfunction.
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