Efficacy of toripalimab in combination with anlotinib in recurrent undifferentiated pleomorphic sarcoma of the sinonasal region: a case report with biomarker analysis

Abstract

Background: Soft tissue sarcoma (STS) typically originates in the muscles and is associated with a poor prognosis. Undifferentiated pleomorphic sarcoma (UPS) is the most commonly diagnosed subtype of STS; however, UPS occurring in the sinonasal region is exceedingly rare and lacks effective treatment options.

Objective: This case report presents a patient with sinonasal UPS who experienced disease progression after surgery and chemotherapy but showed a positive response to combination therapy with toripalimab and anlotinib. Additionally, it explores the underlying biomarkers associated with this case.

Case: A 63-year-old woman with no significant past medical history was diagnosed with sinonasal UPS. The lesions recurred despite seven extensive surgical resections, and standard chemotherapy failed to control the disease, leading to progressive disease (PD).

Results: The patient was treated with a combination of toripalimab and anlotinib, resulting in a significant partial response (PR) after just two cycles. Continued PR was observed after an additional six cycles, indicating the potential for a prolonged response with ongoing therapy. Genotyping and immunohistochemistry revealed that the sarcoma cells were rapidly dividing and enriched in vasculature prior to systemic treatment.

Conclusion: These findings suggest that the combination of toripalimab and anlotinib may be an effective treatment option for advanced cases of UPS in the sinonasal region.

Keywords: anlotinib; case report; recurrence; toripalimab; undifferentiated pleomorphic sarcoma.

Figure 1

Initial clinical presentation of the sarcoma and biopsy histologic images. (A) Horizontal (left), coronal (middle), and sagittal (right) computed tomography (CT) scan of sinus before surgery resection in the local hospital. The tumor is indicated by red arrows. (B) A panel of immunohistochemical and in situ hybridization markers was performed to confirm the soft tissue sarcoma (STS) subtype diagnosis. Samples were collected on 19 July 2022 during the first surgery. The markers that showed positive staining are displayed in the bottom panel. Scale bar, 50 μm.

Figure 2

The treatment and follow-up timeline for the patient.

Figure 3

MRI findings of the case. (A–C) Horizontal (A), coronal (B), and sagittal (C) MRI of sinus before the sixth surgery on 28 May 2024. The tumor is indicated by red arrows. (D–F) Horizontal (D), coronal (E), and sagittal (F) MRI of sinus 1 month after the sixth surgery on 7 July 2024. (G–I) Horizontal (G), coronal (H), and sagittal (I) MRI of sinus before toripalimab + anlotinib treatment showed that the right nasal cavity, sinus, right maxillary sinus, turbinate, and palate displayed postoperative changes. The maxillofacial area was sunken, and the adjacent soft tissues were significantly enhanced. The right temporalis muscle and internal and external pterygoid muscles were thickening, and the signal was increased. There were strips of significantly enhanced shadows in the intermuscular space, which were dumbbell-shaped from the lateral wall of the upper maxillary sinus to the sinus cavity. The size is approximately 50 × 25 × 27 mm. The tumor is indicated by red arrows. (J–L) Horizontal (J), coronal (K), and sagittal (L) MRI of sinus after two cycles of toripalimab + anlotinib treatment showed reduced tumor lesions. (M–O) Horizontal (M), coronal (N), and sagittal (O) MRI of sinus after four cycles of toripalimab + anlotinib treatment showed continuous partial response. (P–R) Horizontal (P), coronal (Q), and sagittal (R) of sinus after eight cycles of toripalimab + anlotinib treatment showed continuous partial response.

Figure 4

Immunohistochemical staining of CD31, Ki67, and PD-L1. (A) Immunohistochemical DAB staining showed negative expression of PD-L1(22C3) on sarcoma tissues before the initiation of systemic treatment. Scale bar, 50 μm. (B) Immunohistochemical DAB staining showed the expression of Ki67 on the sarcoma tissues before the initiation of systemic treatment. Scale bar, 50 μm. (C) Immunohistochemical DAB staining showed high expression of CD31 on the sarcoma tissues before the initiation of systemic treatment. Scale bar, 50 μm. (D) Routine hematoxylin and eosin (H&E) staining of the sarcoma tissue before the initiation of systemic treatment. Scale bar, 50 μm.