Sacral chordoma with pulmonary metastasis: Case report and a brief review of the literature

Chordoma is an uncommon, locally invasive malignancy that originates from residual notochords, and the sacral location constitutes half of the cases. Despite the multimodal treatment, the treatment becomes convoluted due to anatomical limitations and recurrence rates. We report a 48-year-old man with an 8-month history of progressive sacrococcygeal pain and neurological manifestations—initial presentation involved nocturnal exacerbation of pain, bowel/bladder dysfunction, and weakness of lower extremities. On physical examination, decreased sacral reflexes and palpation of a presacral mass on digital rectal examination were noted. MRI revealed a lobulated, T2-hyperintense mass in the sacrum, with extension into the surrounding soft tissues. Chordoma was confirmed by CT-guided core biopsy, which revealed typical physaliferous cells, positive immunostaining for brachyury, and expression of PDGFR.

After multidisciplinary tumor board discussion, the patient was treated with surgical resection of a 6 cm tumor with focally positive margins, followed by concurrent high-dose radiotherapy (76 Gy) and imatinib mesylate (400 mg/d). Even though the patient responded initially, he developed pulmonary metastasis 19 months later.  He refused surgical treatment or radiation therapy and accepted only medical treatment, to which he responded with serious side effects, and expired four months later due to widespread metastasis. The case illustrates the multifaceted approach to managing sacral chordoma, highlighting the importance of early diagnosis, multidisciplinary care coordination, and aggressive multimodal treatment. Molecular profiling and targeted therapy integration are treatment modalities that are still in the process of development. Although management is at its best, the emergence of treatment resistance underscores the need for innovative treatment approaches and the vital role of long-term surveillance in chordoma patients.