Abstract

Chaimaa Aboumedian*, Siham Lkhoyaali, Youssef Mahdi, Amine Benslimane,
Basma El Khannoussi, Hassan Errihani

ABSTRACT

Background: Chordoma is a rare malignant bone tumor arising from embryonic remnants of the notochord. It most commonly affects the sacrum and skull base. It is characterized by a high risk of local recurrence and, less frequently, distant metastasis. Complete surgical resection, when feasible, combined with high-dose radiotherapy, are the cornerstone of treatment. Systemic treatment options remain limited in locally advanced or recurrent disease that is not amenable to local therapy.[1,11] Case Report: We report the case of a 63-year-old hypertensive man followed since 2020 for sacral chordoma. Surgical resection was performed on March 19, 2020, followed by adjuvant radiotherapy to a total dose of 60 Gy, completed on September 25, 2020. Histopathological examination was consistent with conventional chordoma, with clear bone margins but lateral margins that were difficult to assess because of capsular rupture. In 2023, the disease recurred and neither surgery nor re-irradiation was considered feasible. Systemic treatment with imatinib 400 mg/day was initiated and subsequently increased to 800 mg/day because of further progression. Despite this strategy, radiological progression occurred in 2025. Imatinib was temporarily discontinued because of intolerance. Doxorubicin chemotherapy was subsequently administered, but the patient was lost to follow-up. In January 2026, further local progression led to the initiation of pazopanib at 800 mg/day. At the time of writing, the patient remains on pazopanib with an ECOG/WHO performance status of 1–2, and clinical and radiological reassessment is ongoing. Discussion: Management of unresectable recurrent chordoma requires a multidisciplinary approach. Imatinib is among the best-studied targeted therapies because of the frequent expression of PDGFR in chordoma.[2,5,6] This case illustrates the therapeutic challenges of recurrent chordoma and the importance of management in an expert center, with discussion of targeted options, clinical trials, and molecular characterization whenever available.[2,10,11] In a phase II study of 56 patients with advanced PDGFB/PDGFRB-positive chordoma, imatinib 800 mg/day produced an objective RECIST response in only 2% of patients, whereas prolonged disease stabilization was observed in a substantial proportion. Other tyrosine kinase inhibitors, including antiangiogenic agents targeting VEGFR such as pazopanib, may be considered after imatinib failure, although the level of evidence remains low.[5,8,9,10] Conclusion: Unresectable, non-re-irradiable recurrent sacral chordoma represents a complex therapeutic situation. The absence of systemic treatments validated by randomized trials requires individualized treatment strategies. Imatinib may provide disease control in selected patients, whereas pazopanib may be considered as a salvage option after progression, although supporting evidence remains limited. Improved molecular characterization and enrollment in clinical trials remain important.[2,10,11,12]