Bennett Julie, Nobre Liana, Bouffet Eric, Abla Oussama, Maguire Bryan, Amirabadi Afsaneh, Hawkins Cynthia, Wasserman Jonathan D, Ertl-Wagner Birgit, Bartels Ute
Division of Medical Oncology and Hematology, Princess Margaret Cancer Centre, Toronto, Ontario, Canada.
Division of Hematology/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada.
Neurooncol Adv. 2024 Dec 14;6(1):vdae214. doi: 10.1093/noajnl/vdae214. eCollection 2024 Jan-Dec.
Few studies have evaluated predictive factors of isolated pituitary stalk thickening (iPST) in children.
In this retrospective study, radiology, endocrinology, and neuro-oncology databases were interrogated to identify patients with iPST between January 2000 and June 2019. A blinded, longitudinal assessment of MRIs was performed using quantitative, semi-quantitative, and qualitative metrics. Neuroimaging parameters were correlated to clinical parameters.
Forty-seven patients were identified, with 40 meeting the inclusion criteria. Median age at baseline MRI was 9.6 years (range 0.9-17.5) with median follow-up of 5.2 years (range 0.3-18.6). Twenty-five (63%) were female. Thirty-four (85%) had pituitary dysfunction, including 31 with central diabetes insipidus (cDI). cDI was not predictive of proliferative disease (PfD): 69% of those with presumed primary hypophysitis (PPH) versus 93% with PfD ( = .1). Fourteen (35%) patients were diagnosed with PfD (germinoma = 8, Langerhans cell histiocytosis = 5, lymphoma = 1) at median of 1.3 years (range 0.3-4.0) after initial MRI. Progressive thickening of the stalk over time was associated with PfD (86% vs 4% in PPH, < .0001), as was thickening of the entire stalk (56% in PfD vs 27% in PPH, < .0001) with different imaging trends over time observed in PfD versus PPH. A "sack of marbles" appearance with heterogeneous enhancement on post-contrast imaging was associated with germinoma.
In this cohort, 35% of children with iPST were diagnosed with PfD. The association of cDI and PfD was not statistically significant. Progressive thickening of the entire stalk was predictive of PfD and a "sack of marbles" pattern was found to be highly suggestive of germinoma.
很少有研究评估儿童孤立性垂体柄增粗(iPST)的预测因素。
在这项回顾性研究中,对放射学、内分泌学和神经肿瘤学数据库进行查询,以确定2000年1月至2019年6月期间患有iPST的患者。使用定量、半定量和定性指标对MRI进行盲法纵向评估。将神经影像学参数与临床参数进行关联。
共识别出47例患者,其中40例符合纳入标准。基线MRI时的中位年龄为9.6岁(范围0.9 - 17.5岁),中位随访时间为5.2年(范围0.3 - 18.6年)。25例(63%)为女性。34例(85%)存在垂体功能障碍,其中包括3例中枢性尿崩症(cDI)。cDI并非增殖性疾病(PfD)的预测因素:推测为原发性垂体炎(PPH)的患者中69%出现cDI,而PfD患者中这一比例为93%(P = 0.1)。14例(35%)患者在初次MRI检查后的中位1.3年(范围0.3 - 4.0年)被诊断为PfD(生殖细胞瘤8例,朗格汉斯细胞组织细胞增多症5例,淋巴瘤1例)。随着时间推移,垂体柄逐渐增粗与PfD相关(PPH中为86%,PfD中为4%,P < 0.0001),整个垂体柄增粗情况也是如此(PfD中为56%,PPH中为27%,P < 0.0001),PfD和PPH随时间呈现不同的影像学变化趋势。增强后成像上出现不均匀强化的“弹珠袋”样表现与生殖细胞瘤相关。
在该队列中,35%的iPST儿童被诊断为PfD。cDI与PfD之间的关联无统计学意义。整个垂体柄的逐渐增粗是PfD的预测因素,且发现“弹珠袋”样表现高度提示生殖细胞瘤。