Sarkar Nilanjan, Chakravarthy Somen, Chakravarty Rohit, Mukhopadhyay Sandipan
Radiology, Tata Main Hospital, Jamshedpur, IND.
Cureus. 2022 Apr 21;14(4):e24335. doi: 10.7759/cureus.24335. eCollection 2022 Apr.
Ecchordosis physaliphora (EP) is a notochordal remnant tissue rarely encountered during routine clinical practice. These lesions usually do not produce any significant symptoms as they are slow-growing and mostly small in size. Symptoms are due to mass effects on adjacent structures when they are large or extra-tumoral hemorrhage. Because of histological similarity with chordoma, diagnosis is challenging, and this differentiation is essential as the disease course and treatment differ significantly. Imaging plays a crucial role in identifying and distinguishing these lesions. We report the case of a 16-year-old male who presented with intermittent headache and neck pain for six months. His routine clinical examinations were within normal limits. On neurological assessment, there was no focal neurodeficit. Evaluation of cranial nerves did not reveal any evidence of palsy. Routine hematological tests were also normal. A computed tomography (CT) scan of the brain revealed a mass in front of the pons. Magnetic resonance imaging (MRI) for further evaluation revealed a T1 hypointense and T2/fluid-attenuated inversion recovery hyperintense lesion in the pre-pontine cistern. There was no enhancement in the mass either in the post-contrast CT or MRI scans. There was no bony erosion and clivus was normal. Based on the location and characteristic imaging features, a diagnosis of EP was made. There may be several other lesions that may present as a mass in the pre-pontine region. Histopathological tests may find it difficult to distinguish between lesions that originate from notochord remnants. Imaging studies play a vital role in confirming the diagnosis and help in planning treatment and follow-up.
泡状脊索瘤(EP)是一种在常规临床实践中很少遇到的脊索残余组织。这些病变通常不会产生任何明显症状,因为它们生长缓慢且大多体积较小。当病变较大或发生瘤外出血时,症状是由于对相邻结构的占位效应所致。由于与脊索瘤在组织学上相似,诊断具有挑战性,而这种鉴别至关重要,因为疾病进程和治疗方法有显著差异。影像学在识别和区分这些病变方面起着关键作用。我们报告一例16岁男性患者,他出现间歇性头痛和颈部疼痛6个月。他的常规临床检查结果正常。经神经学评估,未发现局灶性神经功能缺损。对颅神经的评估未发现任何麻痹迹象。常规血液学检查也正常。脑部计算机断层扫描(CT)显示脑桥前方有一个肿块。为进一步评估进行的磁共振成像(MRI)显示脑桥前池有一个T1低信号和T2/液体衰减反转恢复序列高信号病变。增强CT或MRI扫描中肿块均无强化。没有骨质侵蚀,斜坡正常。根据病变位置和特征性影像学表现,诊断为EP。在脑桥前区可能还有其他几种病变可表现为肿块。组织病理学检查可能难以区分起源于脊索残余的病变。影像学研究在确诊中起着至关重要的作用,并有助于制定治疗和随访计划。