Clinical and endocrinological manifestations of childhood-onset craniopharyngioma before surgical removal: A report from one medical center in Taiwan
Background: Craniopharyngiomas are benign tumors of embryologic origin located in the sellar region. Patients have both neurological and endocrinological symptoms. Symptoms may be subtle in the early clinical course, which leads to delayed diagnosis. This study evaluated the clinical and endocrinolo...
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Elsevier
2021-03-01
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| Series: | Pediatrics and Neonatology |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S1875957220301947 |
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| author | Ching-Chih Huang Kuang-Lin Lin Chieh-Tsai Wu Shih-Ming Jung Chao-Jan Wang Yi-Ching Chen Fu-Sung Lo |
| author_facet | Ching-Chih Huang Kuang-Lin Lin Chieh-Tsai Wu Shih-Ming Jung Chao-Jan Wang Yi-Ching Chen Fu-Sung Lo |
| author_sort | Ching-Chih Huang |
| collection | DOAJ |
| description | Background: Craniopharyngiomas are benign tumors of embryologic origin located in the sellar region. Patients have both neurological and endocrinological symptoms. Symptoms may be subtle in the early clinical course, which leads to delayed diagnosis. This study evaluated the clinical and endocrinological manifestations of childhood-onset craniopharyngioma. Methods: We retrospectively reviewed medical records of 45 children diagnosed as having craniopharyngioma between 1995 and 2019. We collected data on clinical symptoms and signs, height, weight, biochemical and hormone data, images, operation records, and pathology reports. A three-graded classification system was applied to define the degree of hypothalamic damage (HD). We analyzed clinical and endocrinological manifestations among patients with and without obesity, with short and normal stature, and with differing degrees of HD. Results: Clinical endocrinologic manifestations included adrenocortical insufficiency (42%), central hypothyroidism (37%), short stature (31%), obesity (20%), weight < third percentile (19%), and polyuria or polydipsia (11%). The distribution of height and body mass index (BMI) revealed that a relatively large proportion of patients had short stature and obesity compared to the general population. Patients with grade 2 HD were significantly taller (height median SDS −0.07 vs. −2.05, P = 0.032), and had higher BMI (BMI median standard deviation scores [SDS] 1.14 vs. −0.54, P = 0.039) and shorter time to diagnosis (0.27 vs. 8.29 months, P = 0.007) than were those in the grade 0–1 HD. Delayed diagnosis was associated with short stature (6/7 vs. 4/26, P = 0.001) and no initial neurological symptoms (4/7 vs. 2/28, P = 0.009). Conclusion: Growth patterns may change variously depend on the tumor location and the severity of hypothalamic damage. Therefore, monitoring possible neurological symptoms and evaluating the growth patterns of patients during regular outpatient clinical visits are paramount. |
| format | Article |
| id | doaj-art-8155cdf562c5428f922bae2d120a20f6 |
| institution | Kabale University |
| issn | 1875-9572 |
| language | English |
| publishDate | 2021-03-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Pediatrics and Neonatology |
| spelling | doaj-art-8155cdf562c5428f922bae2d120a20f62025-08-20T03:49:03ZengElsevierPediatrics and Neonatology1875-95722021-03-0162218118610.1016/j.pedneo.2020.08.014Clinical and endocrinological manifestations of childhood-onset craniopharyngioma before surgical removal: A report from one medical center in TaiwanChing-Chih Huang0Kuang-Lin Lin1Chieh-Tsai Wu2Shih-Ming Jung3Chao-Jan Wang4Yi-Ching Chen5Fu-Sung Lo6Department of Pediatrics, Chang Gung Memorial Hospital, Linkou Medical Center, Taoyuan, TaiwanDivision of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, TaiwanDepartment of Neurosurgery, Chang Gung Memorial Hospital, Linkou Medical Center, Taoyuan, TaiwanDepartment of Pathology, Chang Gung Memorial Hospital, Linkou Medical Center, Taoyuan, TaiwanDepartment of Radiology, Chang Gung Memorial Hospital, Linkou Medical Center, Taoyuan, TaiwanDepartment of Pediatrics, Chang Gung Memorial Hospital, Linkou Medical Center, Taoyuan, TaiwanDivision of Pediatric Endocrinology and Genetics, Department of Pediatrics, Chang Gung Memorial Hospital, Linkou Medical Center, Taoyuan, Taiwan; Corresponding author. Division of Pediatric Endocrinology and Genetics, Department of Pediatrics, Chang Gung Memorial Hospital, 5 Fu-Hsin Street, Kweishan, Taoyuan, 333, Taiwan. Fax: +886 3 3288957.Background: Craniopharyngiomas are benign tumors of embryologic origin located in the sellar region. Patients have both neurological and endocrinological symptoms. Symptoms may be subtle in the early clinical course, which leads to delayed diagnosis. This study evaluated the clinical and endocrinological manifestations of childhood-onset craniopharyngioma. Methods: We retrospectively reviewed medical records of 45 children diagnosed as having craniopharyngioma between 1995 and 2019. We collected data on clinical symptoms and signs, height, weight, biochemical and hormone data, images, operation records, and pathology reports. A three-graded classification system was applied to define the degree of hypothalamic damage (HD). We analyzed clinical and endocrinological manifestations among patients with and without obesity, with short and normal stature, and with differing degrees of HD. Results: Clinical endocrinologic manifestations included adrenocortical insufficiency (42%), central hypothyroidism (37%), short stature (31%), obesity (20%), weight < third percentile (19%), and polyuria or polydipsia (11%). The distribution of height and body mass index (BMI) revealed that a relatively large proportion of patients had short stature and obesity compared to the general population. Patients with grade 2 HD were significantly taller (height median SDS −0.07 vs. −2.05, P = 0.032), and had higher BMI (BMI median standard deviation scores [SDS] 1.14 vs. −0.54, P = 0.039) and shorter time to diagnosis (0.27 vs. 8.29 months, P = 0.007) than were those in the grade 0–1 HD. Delayed diagnosis was associated with short stature (6/7 vs. 4/26, P = 0.001) and no initial neurological symptoms (4/7 vs. 2/28, P = 0.009). Conclusion: Growth patterns may change variously depend on the tumor location and the severity of hypothalamic damage. Therefore, monitoring possible neurological symptoms and evaluating the growth patterns of patients during regular outpatient clinical visits are paramount.http://www.sciencedirect.com/science/article/pii/S1875957220301947Craniopharyngiomaendocrinologyhypopituitarismpediatric obesitypediatrics |
| spellingShingle | Ching-Chih Huang Kuang-Lin Lin Chieh-Tsai Wu Shih-Ming Jung Chao-Jan Wang Yi-Ching Chen Fu-Sung Lo Clinical and endocrinological manifestations of childhood-onset craniopharyngioma before surgical removal: A report from one medical center in Taiwan Pediatrics and Neonatology Craniopharyngioma endocrinology hypopituitarism pediatric obesity pediatrics |
| title | Clinical and endocrinological manifestations of childhood-onset craniopharyngioma before surgical removal: A report from one medical center in Taiwan |
| title_full | Clinical and endocrinological manifestations of childhood-onset craniopharyngioma before surgical removal: A report from one medical center in Taiwan |
| title_fullStr | Clinical and endocrinological manifestations of childhood-onset craniopharyngioma before surgical removal: A report from one medical center in Taiwan |
| title_full_unstemmed | Clinical and endocrinological manifestations of childhood-onset craniopharyngioma before surgical removal: A report from one medical center in Taiwan |
| title_short | Clinical and endocrinological manifestations of childhood-onset craniopharyngioma before surgical removal: A report from one medical center in Taiwan |
| title_sort | clinical and endocrinological manifestations of childhood onset craniopharyngioma before surgical removal a report from one medical center in taiwan |
| topic | Craniopharyngioma endocrinology hypopituitarism pediatric obesity pediatrics |
| url | http://www.sciencedirect.com/science/article/pii/S1875957220301947 |
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