Department of Pathology, All India Institute of Medical Sciences Jodhpur, Rajasthan 342005, India.
Curr Med Imaging. 2023;19(7):780-783. doi: 10.2174/1573405618666220901123032.
Chordomas are midline tumors that arise from remnants of the primitive notochord, while the heterotopic rests are usually situated extradural within the bones of the axial skeleton. The tumor is locally aggressive with a high recurrence rate but rarely gives rise to metastasis.
A 55-year-old female presented with complaints of headache and intermittent vomiting for six months and diminution of vision for the last four months. MRI, T1 weighted without contrast of the paranasal sinus, suggested sphenoid sinus malignancy or metastasis. The mass was excised and sent for histopathological examination. Based on the radiological findings, light microscopy, special stains, and immunohistochemistry, a final diagnosis of chordoma was rendered.
The chordoma is a well-known entity at the skull base. Skull-based chordomas account for only 0.1-0.2% of all chordomas. The incidence rate of chordomas is 0.08 per 100,000, with a higher incidence rate of 0.1 in males than in females. However, chordoma presenting as a sphenoidal mass will create a diagnostic challenge for all otorhinolaryngologists and head and neck surgeons.
This case report aims to add this rare tumor to the differential diagnosis of neoplasms of the sphenoid sinus.
脊索瘤是一种起源于原始脊索遗迹的中线肿瘤，而异位残余通常位于轴性骨骼的硬膜外骨骼内。该肿瘤具有局部侵袭性和高复发率，但很少发生转移。
一名 55 岁女性因头痛和间歇性呕吐六个月，视力下降四个月来就诊。鼻窦的 MRI（T1 加权无对比）提示蝶窦恶性肿瘤或转移。切除肿块并送检组织病理学检查。根据影像学发现、光镜检查、特殊染色和免疫组织化学检查，最终诊断为脊索瘤。
脊索瘤是颅底的一种常见实体瘤。颅底脊索瘤仅占所有脊索瘤的 0.1-0.2%。脊索瘤的发病率为 0.08/10 万，男性发病率略高于女性，为 0.1。然而，蝶窦肿块的脊索瘤将给所有耳鼻喉科医生和头颈外科医生的诊断带来挑战。
本病例报告旨在将这种罕见肿瘤纳入蝶窦肿瘤的鉴别诊断。
