A Study of BL-B01D1 in Patients With Locally Advanced or Metastatic Chordoma

Inclusion Criteria:

1. Sign the informed consent form voluntarily and follow the protocol requirements;

2. Gender is not limited;

3. Age: ≥18 years old and ≤75 years old;

4. Locally advanced (unresectable) or metastatic chordoma confirmed by histopathology;

5. ECOG ≤2;

6. The expected survival time as judged by the investigator was ≥3 months;

7. The toxicity of previous antineoplastic therapy has returned to ≤ grade 1 as definedby NCI-CTCAE v5.0;

8. No severe cardiac dysfunction, left ventricular ejection fraction ≥50%;

9. Organ function level must meet the requirements;

10. Coagulation function: international normalized ratio (INR) ≤1.5, and activatedpartial thromboplastin time (APTT) ≤1.5ULN;

11. Urine protein ≤2+ or < 1000mg/24h;

12. For premenopausal women with childbearing potential, a pregnancy test must beperformed within 7 days before starting treatment, a serum or urine pregnancy testmust be negative, and the patient must not be lactating; All enrolled patientsshould take adequate barrier contraception during the entire treatment cycle and for 6 months after the end of treatment.

Exclusion Criteria:

1. Chemotherapy, biological therapy, immunotherapy, etc. within 4 weeks or 5 half-livesbefore the first dose, small molecule targeted therapy within 5 days, and palliativeradiotherapy within 2 weeks;

2. A history of central nervous system hemorrhage/infarction requiring treatment within 6 months before enrollment;

3. History of severe heart disease and cerebrovascular disease;

4. QT prolongation, complete left bundle branch block, III degree atrioventricularblock, severe arrhythmia;

5. Unstable thrombotic events requiring therapeutic intervention within 6 months beforescreening; Infusion-related thrombosis was excluded;

6. Active autoimmune and inflammatory diseases;

7. Other malignant tumors that progressed or required treatment within 5 years beforethe first dose;

8. Poorly controlled hypertension (systolic blood pressure after adequate medicaltherapy &gt; 150 mmHg or diastolic blood pressure &gt; 100 mmHg);

9. Poor glycemic control;

10. Patients with a previous history of ILD requiring hormone therapy, or current ILD or ≥G2 radiation pneumonitis, or suspected to have such a condition during screening;

11. Complicated with pulmonary diseases leading to clinically severe respiratoryfunction impairment;

12. Patients with a history of allergy to recombinant humanized or human-mouse chimericantibodies or to any of the excipients of BL-B01D1;

13. Received previous organ transplantation or allogeneic hematopoietic stem celltransplantation (Allo-HSCT);

14. Human immunodeficiency virus antibody positive, active tuberculosis, activehepatitis B virus infection or active hepatitis C virus infection;

15. Had a serious infection within 4 weeks before the first dose of study drug;Indications of active pulmonary infection within 2 weeks before the first dose ofstudy drug;

16. Imaging examination indicated that the tumor had invaded or enveloped the largeblood vessels of the chest, neck, abdomen, ilium, and pharynx, except that theinvestigator thought that it would not affect the patient's medication;

17. With a history of psychotropic drug abuse and inability to quit or a history ofsevere neurological or psychiatric illness;

18. Serious unhealed wound, ulcer, or fracture within 4 weeks before signing theinformed consent;

19. Clinically significant bleeding or obvious bleeding tendency within 4 weeks beforesigning the informed consent;

20. Patients scheduled for vaccination or receiving live vaccine within 28 days beforethe first dose;

21. Had participated in another clinical trial within 4 weeks before the first dose;

22. Other circumstances that the investigator deemed inappropriate for participation inthe trial.