Raffa Anas
Radiology, King Abdulaziz University Faculty of Medicine, Jeddah, SAU.
Cureus. 2022 Mar 27;14(3):e23544. doi: 10.7759/cureus.23544. eCollection 2022 Mar.
This study presented the rare case of a seven-year-old patient with atypical presentation and neuroradiological features of giant ecchordosis physaliphora. The patient underwent cross-sectional imaging due to persistent headache without neurological or visual symptoms. CT scan imaging of the head revealed a hypodense tumor in the prepontine cistern. This lesion caused smooth scalloping of the dorsal clivus without aggressive erosion or calcification, and an osseous stalk was also identified between the lesion and the dorsal wall of the clivus. Sagittal T1 weighted image (T1WI) MRI showed a bilobed, solid and cystic, well-defined lesion, measuring 3.5 cm in terms of craniocaudal diameter, found alongside the midline within the prepontine cistern. After the evaluation of radiological images, the patient was then subjected to endoscopic transnasal complete tumor excision. Histological examination revealed sheets and lobules of clear cells with cytoplasmic globules "physaliphorous cells", and myxoid stroma. There was nuclear pleomorphism associated with focal areas of necrosis. After full recovery and discharge, the patient was followed up for the first year with four-month interval brain MRI scans showing no evidence of residual tumors. On the 12 months follow-up scan, the MRI scan revealed a 1.5 x 0.7 cm recurrent mass in the retroclival right paramidline prepontine cistern, which was most notably seen on the diffusion-weighted images. Series of proton beam therapy with annual MRI scans demonstrated regression of the tumor, eventually allowing the patient to live free of neurological symptoms up to this day. Results suggest that the utilization of radiological imaging such as CT and MRI scans was successful in identifying the ecchordosis physaliphora and differentiating it from chordomas. It can also be inferred that atypical radiological and histopathological findings of ecchordosis physaliphora lesions might suggest that they are prone to recurrence, which is an atypical feature for such entities. Further studies are recommended to explore and better understand these uncommon observations in patients with ecchordosis physaliphora.
本研究报告了一例罕见的七岁患者，其表现不典型，具有巨大泡状脊索瘤的神经放射学特征。该患者因持续头痛但无神经或视觉症状而接受了横断面成像检查。头部CT扫描显示脑桥前池有一个低密度肿瘤。该病变导致斜坡背侧出现平滑的扇贝样改变，无侵袭性侵蚀或钙化，并且在病变与斜坡后壁之间还发现了一个骨性蒂。矢状位T1加权像（T1WI）MRI显示一个分叶状、实性和囊性、边界清晰的病变，其颅尾径为3.5厘米，位于脑桥前池中线旁。在对放射影像进行评估后，该患者随后接受了内镜经鼻完全肿瘤切除术。组织学检查显示有透明细胞片和小叶，伴有胞质小球（“泡状细胞”）以及黏液样基质。存在核多形性，并伴有局灶性坏死区域。在完全康复并出院后，对该患者进行了为期一年的随访，每隔四个月进行一次脑部MRI扫描，结果显示没有残留肿瘤的迹象。在12个月的随访扫描中，MRI显示在脑桥前池右旁中线斜坡后有一个1.5×0.7厘米的复发肿块，在扩散加权图像上最为明显。一系列质子束治疗并每年进行MRI扫描显示肿瘤有所消退，最终使患者直至今日都没有神经症状。结果表明，利用CT和MRI扫描等放射影像学检查成功识别了泡状脊索瘤，并将其与脊索瘤区分开来。还可以推断，泡状脊索瘤病变的非典型放射学和组织病理学表现可能表明它们易于复发，这是此类病变的一个非典型特征。建议进一步开展研究，以探索并更好地理解泡状脊索瘤患者的这些不常见表现。
