Julie Bennett, Liana Nobre, Eric Bouffet, Oussama Abla, Bryan Maguire, Afsaneh Amirabadi, Cynthia Hawkins, Jonathan D Wasserman, Birgit Ertl-Wagner, Ute Bartels
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引用次数: 0
Abstract
Background: Few studies have evaluated predictive factors of isolated pituitary stalk thickening (iPST) in children.
Methods: 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.
Results: 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 (P = .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, P < .0001), as was thickening of the entire stalk (56% in PfD vs 27% in PPH, P < .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.
Conclusions: 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.