Primary clear cell sarcoma of soft tissue in the posterior cervical spine invading the medulla oblongata: A case report

BACKGROUND: Clear cell sarcoma (CCS) is a rare and highly malignant soft tissue tumor, usually occurring in the deep soft tissues of the distal tendons and aponeurosis of the extremities, especially the feet and knees. CCS originating in the head and neck is extremely rare. The clinical manifestations of CCS in the head and neck are not typical, and the imaging manifestations have certain characteristics, but the diagnosis still depends on pathological examination and genetic testing. CASE SUMMARY: A 33-year-old male patient had paroxysmal headache for more than 4 years, accompanied by nausea and vomiting, which could be relieved after rest. Computed tomography angiography showed a left paraspinal soft tissue mass. Contrast-enhanced imaging showed obvious uneven enhancement with adjacent bone lytic destruction. Magnetic resonance imaging examination showed isosignal on T(1)-weighted images, slightly high signal on T(2)-weighted images (T(2)WI), high signal on Tirm fat suppression sequence, significantly high signal on diffusion weighted imaging, and obvious and uneven enhancement. The lesion invaded the anterior medulla oblongata through the left atlantoaxial foramen and compressed the cervical spinal cord on T(2)WI. Primary CCS of soft tissue was diagnosed by pathology and genetic examination. CONCLUSION: CCS should be considered in the differential diagnosis of soft tissue tumors of the head and neck, and their diagnosis depends on pathological examination and genetic testing.