Exploratory Study on Toripalimab and Anlotinib Combined With Standard Chemotherapy for Refractory Dermatofibrosarcoma Protuberans

NCT07646080 | Not Yet Recruiting Phase 2

• 1 other identifier: JY2026-036
• Study Type: interventional
• Target: 20
• Locations: 0 countries
• Sites: N/A

Brief Summary

This study aims to evaluate the efficacy and safety of toripalimab and anlotinib hydrochloride combined with standard chemotherapy in patients with refractory dermatofibrosarcoma protuberans (DFSP) resistant to imatinib therapy, and to provide evidence for the exploration of DFSP treatment.

Conditions

Dermatofibrosarcoma Protuberans (DFSP)

Outcome Measures

Primary Outcomes (1)

• Objective Response Rate (ORR)

  The Objective Response Rate (ORR) is defined as the percentage of patients whose best response on or before the first occurrence of disease progression is a complete response (CR) or partial response (PR). Tumor responses were assessed by investigators using Response Evaluation Criteria in Solid Tumors (RECIST) version 1.1.

  From the date of first study treatment until disease progression or death from any cause, whichever occurs first, assessed up to 24months.

Secondary Outcomes (7)

• Duration of Response (DoR)

  From the date of first documented response (complete response [CR] or partial response [PR]) to the time of disease progression or death from any cause, whichever occurs first, assessed up to 24months.

• Progression-Free Survival (PFS)

  Through study completion, an average of 2 years.

• Overall Survival (OS)

  Through study completion, an average of 2 years.

• Disease Control Rate (DCR)

  From the date of first study treatment until disease progression or death from any cause, whichever occurs first, assessed up to 24 months.

• Best Overall Response (BOR)

  Through study completion, average follow-up of 2 years.

Study Arms (1)

Toripalimab, Anlotinib Hydrochloride Combined with Standard Chemotherapy

EXPERIMENTAL

All enrolled patients will receive study intervention starting on Day 1 of each 3-week cycle until disease progression, intolerable toxicity, or study withdrawal: Toripalimab 240 mg (fixed dose) administered intravenously once every 3 weeks (Q3W); Anlotinib 10 mg administered orally once daily on Days 1-14 of each 3-week cycle; and standard chemotherapy based on anthracycline or gemcitabine once every 3 weeks (Q3W).

Drug: Toripalimab; Drug: Anlotinib; Drug: Standard Chemotherapy

Interventions

Toripalimab DRUG

All enrolled patients will receive study intervention starting on Day 1 of each 3-week cycle until disease progression, intolerable toxicity, or study withdrawal: Toripalimab 240 mg (fixed dose) administered intravenously once every 3 weeks (Q3W).

Toripalimab, Anlotinib Hydrochloride Combined with Standard Chemotherapy

Anlotinib DRUG

All enrolled patients will receive study intervention starting on Day 1 of each 3-week cycle until disease progression, intolerable toxicity, or study withdrawal: Anlotinib 10 mg administered orally once daily on Days 1-14 of each 3-week cycle.

Toripalimab, Anlotinib Hydrochloride Combined with Standard Chemotherapy

Standard Chemotherapy DRUG

All enrolled patients will receive study intervention starting on Day 1 of each 3-week cycle until disease progression, intolerable toxicity, or study withdrawal: standard chemotherapy based on anthracycline or gemcitabine once every 3 weeks (Q3W).

Toripalimab, Anlotinib Hydrochloride Combined with Standard Chemotherapy

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)

You may qualify if:

• Male or female patients aged ≥18 years.
• Locally advanced, unresectable or metastatic dermatofibrosarcoma protuberans (DFSP) with histologically confirmed specific subtypes; disease progression following standard imatinib therapy, or no satisfactory alternative treatment options. Specific subtypes include: fibrosarcomatous DFSP (FS-DFSP) or DFSP with transformation to high-grade sarcoma, such as undifferentiated pleomorphic sarcoma, leiomyosarcoma, rhabdomyosarcoma, etc.
• Eastern Cooperative Oncology Group (ECOG) performance status of 0 or 1.
• At least one measurable lesion at baseline according to RECIST 1.1 criteria.
• Adequate organ and bone marrow function within 14 days prior to enrollment:
• Hemoglobin ≥9 g/dL
• Platelet count ≥75,000/mm³
• Absolute neutrophil count ≥1500/mm³
• Serum albumin ≥2.5 g/dL
• PT, aPTT, and INR ≤1.5 × ULN
• AST and ALT ≤3 × ULN, or \<5 × ULN in patients with liver metastases
• Total bilirubin ≤1.5 × ULN (without liver metastasis), or \<3 × ULN (with Gilbert syndrome or liver metastasis at baseline)
• Creatinine clearance ≥30 mL/min calculated by the Cockcroft-Gault formula
• Left ventricular ejection fraction (LVEF) ≥50% as assessed by ECHO or MUGA scan within 28 days prior to enrollment.

You may not qualify if:

• Patients with any of the following will be excluded:
• Spinal cord compression, leptomeningeal disease, or clinically active central nervous system (CNS) metastases.
• Active primary immunodeficiency, known HIV infection, active hepatitis B virus (HBV) or hepatitis C virus (HCV) infection.
• History of non-infectious interstitial lung disease (ILD)/non-infectious pneumonitis requiring corticosteroid therapy, current ILD/non-infectious pneumonitis, or suspected ILD/non-infectious pneumonitis that cannot be ruled out by imaging at screening.
• Myocardial infarction within 6 months prior to enrollment, symptomatic congestive heart failure (CHF, NYHA class II-IV), unstable angina, or recent cardiovascular event (including stroke) within \<6 months.
• Pulmonary criteria:
• Clinically significant pulmonary comorbidities including but not limited to underlying pulmonary disease (e.g., pulmonary embolism, severe asthma, severe COPD, restrictive lung disease, pleural effusion within 3 months before enrollment);
• Documented autoimmune, connective tissue, or inflammatory disease (e.g., rheumatoid arthritis, Sjögren's syndrome, sarcoidosis, etc.) or suspected pulmonary involvement at screening; full disease details must be documented in the eCRF;
• Prior pneumonectomy.
• Poor compliance unable to cooperate with study treatment and procedures.

Sponsors & Collaborators

• Yanjie Zhang, MD: lead

MeSH Terms

Conditions

Dermatofibrosarcoma

Interventions

toripalimab; anlotinib

Condition Hierarchy (Ancestors)

Fibrosarcoma; Neoplasms, Fibrous Tissue; Neoplasms, Connective Tissue; Neoplasms, Connective and Soft Tissue; Neoplasms by Histologic Type; Neoplasms; Sarcoma

Central Study Contacts

Haihua Yuan

CONTACT

+86-021-56691101-7261; ayuan790415@shsmu.edu.cn

Study Design

• Study Type: interventional
• Phase: phase 2
• Allocation: NA
• Masking: NONE
• Purpose: TREATMENT
• Intervention Model: SINGLE GROUP
• Sponsor Type: OTHER
• Responsible Party: SPONSOR INVESTIGATOR
• PI Title: Director of Oncology Department , Principal Investigator, Clinical Professor Affiliation: Shanghai Ninth People's Hospital Affiliated to Shanghai Jiao Tong University

Study Record Dates

• First Submitted: April 14, 2026
• First Posted: June 12, 2026
• Study Start: June 15, 2026
• Primary Completion (Estimated): December 31, 2028
• Study Completion (Estimated): December 31, 2029
• Last Updated: June 12, 2026

Record last verified: 2026-06

Data Sharing

• IPD Sharing: Will not share