Yu Run
Department of Medicine, UCLA David Geffen School of Medicine, Los Angeles, CA 90095, United States.
World J Radiol. 2025 May 28;17(5):106975. doi: 10.4329/wjr.v17.i5.106975.
Primary sellar atypical teratoid/rhabdoid tumor (AT/RT) is the most aggressive sellar mass. Although rare, sellar AT/RT exhibits a very relentless clinical course and usually results in death within months to a few years after diagnosis. The best clinical evidence suggests that surgical debulking and timely adjuvant chemoradiation are most effective in prolonging survival. A preoperative radiological diagnosis of sellar AT/RT thus is crucial in informing patients and physicians about this devastating disease. This minireview summaries the imaging features of sellar AT/RT. magnetic resonance imaging features of sellar AT/RT and the much more common sellar mass, pituitary macroadenoma, are similar in most aspects: They are both isointense to brain gray matter on T1 and T2 imaging and enhance upon gadolinium administration. Suprasellar extension and cavernous sinus invasion are present in practically all cases of sellar AT/RT, but are also present in 50%-75% of pituitary macroadenomas, especially in large ones, suggesting that suprasellar extension and cavernous sinus invasion disproportionate to the tumor size may favor sellar AT/RT diagnosis. Since sellar AT/RT grows very rapidly and does not allow significant remodeling of perisellar structures, the imaging features of perisellar structures such as optic chiasm and cavernous sinus may be key for imaging diagnosis of sellar AT/RT although they have not been well described in sellar AT/RT. In limited cases of sellar AT/RT, optic chiasm degeneration and thinning, which are very common in pituitary macroadenoma, are not present, giving hope for using features of perisellar structures to diagnose sellar AT/RT by imaging.
原发性鞍区非典型畸胎样/横纹肌样瘤(AT/RT)是最具侵袭性的鞍区肿物。尽管罕见,但鞍区AT/RT的临床病程进展非常迅速,通常在诊断后数月至数年内导致死亡。最佳临床证据表明,手术减瘤和及时的辅助放化疗在延长生存期方面最为有效。因此,鞍区AT/RT的术前影像学诊断对于让患者和医生了解这种毁灭性疾病至关重要。本综述总结了鞍区AT/RT的影像学特征。鞍区AT/RT与更为常见的鞍区肿物垂体大腺瘤的磁共振成像特征在大多数方面相似:在T1和T2成像上它们均与脑灰质等信号,注射钆剂后强化。几乎所有鞍区AT/RT病例均存在鞍上扩展和海绵窦侵犯,但在50%-75%的垂体大腺瘤中也存在,尤其是大型垂体大腺瘤,这表明与肿瘤大小不相称的鞍上扩展和海绵窦侵犯可能有助于鞍区AT/RT的诊断。由于鞍区AT/RT生长非常迅速,不允许鞍周结构有明显重塑,尽管鞍区AT/RT中对鞍周结构如视交叉和海绵窦的影像学特征描述不多,但这些结构的影像学特征可能是鞍区AT/RT影像学诊断的关键。在有限的鞍区AT/RT病例中,垂体大腺瘤中非常常见的视交叉变性和变薄并不存在,这为利用鞍周结构特征通过影像学诊断鞍区AT/RT带来了希望。