Arginine Vasopressin Deficiency in Children with Craniopharyngioma and Cerebral Germ Cell Tumour: Two Sides of the Same Coin. Clinical and Radiological features.

IF 2.6 3区 医学 Q3 ENDOCRINOLOGY & METABOLISM
Sabrina Criscuolo, Cristina Partenope, Mario Tortora, Ved Bhushan Arya, Assunta Albanese
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引用次数: 0

Abstract

Introduction: Paediatric brain tumours in the sellar-suprasellar region (SSR) are often associated with arginine vasopressin peptide deficiency (AVPD), either at diagnosis caused by the tumour itself or during follow-up as consequence of treatments. The purpose of this research is to retrospectively describe the neuroradiological characteristics and the timing of AVPD development in a cohort of paediatric patients with craniopharyngioma (CP) or germ cell tumours (GCT).

Methods: We evaluated brain MRI at tumour diagnosis and at the onset of AVPD, as well as recorded clinical, endocrinological and histopathological data, treatments, and outcome.

Results: Seventy-two patients with AVPD were included: 46 CP (M: F=25:21) and 26 GCT (M: F=18:8). CPs were suprasellar (63%), sellar (4%) or both (33%). GCTs were suprasellar (65%), pineal (24%) or bifocal (11%). No statistically significant differences were noted in tumour size between CP and GCT. Posterior pituitary bright spot absence was reported at diagnosis or at follow-up (as surgery consequence) in all patients with AVPD, indicating that the absence of hyperintensity is a cardinal feature of AVPD. When measurable, pituitary stalk was thickened in most GCT patients (61.5%). At AVPD diagnosis in GCT, the mean age was 11.9 years; 18 (69%) patients had AVPD at the time of tumour diagnosis, 5 (19.3%) before the diagnosis with a latency of 24.4 months (range 4-48), and 3 (11.5%) during follow-up (mean 24 months, range 4-60) due to tumour recurrence. GCT patients presented with severe endocrinological manifestations (18/26), headache and vomiting (10/26), visual impairment (5/26) and behavioural changes with fatigue (1/26). In CP, the mean age at AVPD diagnosis was 10.3 years; 7 (15.2%) patients had AVPD at time of tumour diagnosis, 37 (80.5%) developed it shortly after neurosurgery and 2 patients (4.3%) after 2 and 4 months from surgery, respectively. Clinically, headache and visual abnormalities were the most frequent clinical symptoms at diagnosis of CP (39/46, 84.8%), with hydrocephalus (16/46, 35%) and displacement of optic chiasm (29/46, 63%) at the initial MRI. While the vast majority of CP patients (93%) received only surgery, all GCT patients received radiation therapy in addition to or instead of surgery.

Conclusion: An early differential diagnosis in children with AVPD and brain tumours is supported by a good understanding of the clinical features and imaging findings. Expert follow-up is necessary.

颅咽管瘤和脑生殖细胞瘤患儿精氨酸加压素缺乏症:一枚硬币的两面。临床和放射学特征。
简介:髓窦-髌上区(SSR)的小儿脑肿瘤通常伴有精氨酸加压素肽缺乏症(AVPD),这可能是在诊断时由肿瘤本身引起的,也可能是在随访过程中治疗的结果。本研究的目的是回顾性描述一组颅咽管瘤(CP)或生殖细胞瘤(GCT)儿科患者的神经放射学特征和 AVPD 发生的时间:方法:我们对肿瘤诊断时和 AVPD 发病时的脑 MRI 进行了评估,并记录了临床、内分泌学和组织病理学数据、治疗方法和结果:共纳入72例AVPD患者:其中 CP 46 例(男:女=25:21),GCT 26 例(男:女=18:8)。CP为鞍上型(63%)、蝶鞍型(4%)或两者皆有(33%)。GCT为星状上皮(65%)、松果体(24%)或双灶(11%)。CP和GCT的肿瘤大小无明显统计学差异。所有 AVPD 患者在诊断时或随访时(作为手术后果)均报告垂体后叶亮点缺失,这表明高密度缺失是 AVPD 的主要特征。如果可以测量,大多数 GCT 患者(61.5%)的垂体柄增粗。GCT患者确诊AVPD时的平均年龄为11.9岁;18名(69%)患者在肿瘤确诊时出现AVPD,5名(19.3%)患者在确诊前出现AVPD,潜伏期为24.4个月(4-48个月),3名(11.5%)患者在随访期间(平均24个月,4-60个月)因肿瘤复发而出现AVPD。GCT患者表现为严重的内分泌症状(18/26)、头痛和呕吐(10/26)、视力障碍(5/26)以及伴有疲劳的行为改变(1/26)。在 CP 中,确诊 AVPD 的平均年龄为 10.3 岁;7 名患者(15.2%)在肿瘤确诊时患有 AVPD,37 名患者(80.5%)在神经外科手术后不久出现 AVPD,2 名患者(4.3%)分别在手术后 2 个月和 4 个月后出现 AVPD。临床上,头痛和视力异常是确诊为 CP 时最常见的临床症状(39/46,84.8%),初次 MRI 检查发现脑积水(16/46,35%)和视交叉移位(29/46,63%)。绝大多数CP患者(93%)只接受了手术治疗,而所有GCT患者除手术治疗外,还接受了放射治疗:结论:充分了解临床特征和影像学检查结果有助于对患有 AVPD 和脑肿瘤的儿童进行早期鉴别诊断。专家随访是必要的。
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来源期刊
Hormone Research in Paediatrics
Hormone Research in Paediatrics ENDOCRINOLOGY & METABOLISM-PEDIATRICS
CiteScore
4.90
自引率
6.20%
发文量
88
审稿时长
4-8 weeks
期刊介绍: The mission of ''Hormone Research in Paediatrics'' is to improve the care of children with endocrine disorders by promoting basic and clinical knowledge. The journal facilitates the dissemination of information through original papers, mini reviews, clinical guidelines and papers on novel insights from clinical practice. Periodic editorials from outstanding paediatric endocrinologists address the main published novelties by critically reviewing the major strengths and weaknesses of the studies.
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