Sato Daisuke, Hasegawa Hirotaka, Kimura Soichiro, Sato Junichiro, Shinya Yuki, Umekawa Motoyuki, Yasunaga Yoichi, Makita Noriko, Saito Nobuhito
Department of Neurosurgery, University of Tokyo Hospital, Bunkyo-ku, Tokyo, Japan.
Department of Endocrinology and Nephrology, University of Tokyo Hospital, Bunkyo-ku, Tokyo, Japan.
Surg Neurol Int. 2024 May 10;15:159. doi: 10.25259/SNI_116_2024. eCollection 2024.
Chordomas are rare, locally aggressive neoplasms recognized as derivatives of the notochord vestiges. These tumors typically involve the midline axial skeleton, and intracranial chordomas exhibit proclivity for the spheno-occipital region. However, purely intrasellar occurrences are extremely rare. We report a case of intrasellar chordoma, which masqueraded as a pituitary neuroendocrine tumor.
An 87-year-old female presented with an acutely altered mental state after a few-week course of headaches and decreased left vision. Adrenal insufficiency was evident, and magnetic resonance imaging revealed an intrasellar lesion with heterogeneous contrast enhancement and marked T2 hyperintensity. Central adrenal insufficiency due to an intrasellar lesion was suspected. Cortisol replacement was initiated, and transsphenoidal surgery was performed. Anterosuperior displacement of the normal pituitary gland and the absence of the bony dorsum sellae were notable during the procedure. Histological examination led to a diagnosis of conventional chordoma, and upfront adjuvant stereotactic radiosurgery was executed. She has been free from tumor progression for 12 months.
This case and literature review suggested that the pathognomonic features of intrasellar chordoma were heterogeneous contrast enhancement, marked T2 hyperintensity, osteolytic destruction of the dorsum sellae, and anterosuperior displacement of the pituitary gland. Clinical outcomes seemed slightly worse than those of all skull base chordomas, which were the rationale for upfront radiosurgery in our case. Neurosurgeons should include intrasellar chordomas in the differential diagnosis of intrasellar lesions, carefully dissect them from the adjacent critical anatomical structures, and consider upfront radiosurgery to achieve optimal patient outcomes.
脊索瘤是罕见的、具有局部侵袭性的肿瘤，被认为是脊索遗迹的衍生物。这些肿瘤通常累及中轴线骨骼，颅内脊索瘤好发于蝶枕区域。然而，单纯鞍内发生的情况极为罕见。我们报告一例鞍内脊索瘤病例，该病例曾被误诊为垂体神经内分泌肿瘤。
一名87岁女性，在经历了数周的头痛和左眼视力下降后，出现急性精神状态改变。明显存在肾上腺功能不全，磁共振成像显示鞍内有一病变，呈不均匀强化，T2加权像呈明显高信号。怀疑鞍内病变导致中枢性肾上腺功能不全。开始进行皮质醇替代治疗，并实施了经蝶窦手术。手术过程中发现正常垂体腺向前上方移位，蝶鞍背骨质缺失。组织学检查诊断为传统型脊索瘤，并进行了术前辅助立体定向放射外科治疗。她已无肿瘤进展达12个月。
该病例及文献回顾表明，鞍内脊索瘤的特征性表现为不均匀强化、T2加权像明显高信号、蝶鞍背骨质溶解破坏以及垂体腺向前上方移位。临床结果似乎比所有颅底脊索瘤略差，这也是我们对该病例进行术前放射外科治疗的原因。神经外科医生应将鞍内脊索瘤纳入鞍内病变的鉴别诊断中，小心地将其与相邻的关键解剖结构分离，并考虑术前放射外科治疗以实现最佳的患者预后。
