Hirosawa Renata M, Santos Antonio B A, França Mariana M, Fabris Viciany Erique, Castro Ana Valéria B, Zanini Marco A, Nunes Vania S
Division of Endocrinology and Metabolism, Department of Internal Medicine, Faculty of Medicine of Botucatu, UNESP, 18618-970 Botucatu, SP, Brazil.
ISRN Endocrinol. 2011;2011:259392. doi: 10.5402/2011/259392. Epub 2011 Feb 16.
Chordomas are tumors derived from cells that are remnants of the notochord, particularly from its proximal and distal extremes, they are mainly midline and represent approximately 1% of all malignant bone tumors and 0.1 to 0.2% of intracranial neoplasms. Chordomas involving the sellar region are rare. Herein, we describe a 57-year-old male patient presenting with a history of retro-orbital headache, progressive loss of vision, and clinical features of hypopituitarism, for over 2 months. During evaluation, the CT scan revealed a large contrast-enhancing intrasellar tumor with a 3.6-cm largest diameter. The patient underwent transsphenoidal partial resection of the tumor, and histological examination was consistent with the diagnosis of chondroid chordoma. Although chordomas are rare, they may be considered to constitute a differential diagnostic of pituitary adenomas, especially if a calcified intrasellar tumor with bone erosion is diagnosed.
脊索瘤是起源于脊索残余细胞的肿瘤,特别是来自其近端和远端,它们主要位于中线,约占所有恶性骨肿瘤的1%,颅内肿瘤的0.1%至0.2%。累及鞍区的脊索瘤很罕见。在此,我们描述一名57岁男性患者,有眶后头痛、进行性视力丧失和垂体功能减退的临床特征,持续2个多月。评估期间,CT扫描显示鞍内有一个大的强化肿瘤,最大直径为3.6厘米。患者接受了经蝶骨肿瘤部分切除术,组织学检查与软骨样脊索瘤的诊断一致。尽管脊索瘤罕见,但它们可能被视为垂体腺瘤的鉴别诊断,特别是如果诊断出鞍内有钙化且伴有骨质侵蚀的肿瘤。