Intriguing challenging encounter of Clivus Chordoid Chordoma in a young girl: a case report

Background: Clival chordomas (CC) are rare, slow-growing tumors arising from primitive embryonic notochordal remnants, typically in the spheno-occiput, sacrococcygeal region, and clivus. They are slow-growing, locally aggressive tumors with limited metastatic potential and usually present with symptoms like headache, diplopia, and cranial nerve palsies. Diagnosis relies on MRI and histopathological examination with immunohistochemistry to distinguish them from chondrosarcoma. Case presentation: A five-year-old girl presented with a four-month history of diplopia and right-sided hemiparesis. MRI revealed a hyperintense lobulated mass at the skull base, compressing the spinal cord without cervical vertebral erosion. Post-contrast imaging demonstrated heterogeneous enhancement. A comprehensive baseline evaluation, including a metastatic assessment to exclude distant spread, was conducted. Preoperative assessment was subsequently performed during a tumor board meeting involving oncologists, pathologists, anesthesiologists, and radiologists. Based on these evaluations, a gross total resection of the tumor was planned. Differential diagnoses included chordoma and chondrosarcoma, with ecchordosis physaliphora excluded radiologically. Under general anesthesia, the tumor was successfully resected via a transoronasal approach. Histopathological examination revealed lobules of large cohesive cells within abundant myxoid stroma, consistent with a diagnosis of chondroid chordoma. Immunohistochemical staining demonstrated diffuse positivity for S100 and CK, effectively ruling out chondrosarcoma.Conclusion: This case highlights the clinical, radiological, and histopathological features of CC and emphasizes the importance of immunohistochemical markers for accurate diagnosis. Complete surgical resection remains the mainstay of treatment, complemented by adjunctive radiotherapy for optimal management of this rare tumor.