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Case Reports
. 2024 Jul 11:14:1416241.
doi: 10.3389/fonc.2024.1416241. eCollection 2024.

Case report: Metastatic refractory undifferentiated small round-cell sarcoma successfully treated with surufatinib and camrelizumab

Yong Li  1   2   3 , Jinpeng Huang  1   2   3 , Xian Chen  1   2 , Yongsong Ye  4 , Xiaohua Du  5 , Ioannis A Voutsadakis  6   7 , Mahesh Seetharam  8 , Haibo Zhang  1   2 , Min Lu  1   2   3
Affiliations
Case Reports

Case report: Metastatic refractory undifferentiated small round-cell sarcoma successfully treated with surufatinib and camrelizumab

Yong Li et al. Front Oncol. .

Abstract

Background: Undifferentiated small round-cell sarcomas (uSRCSs) are a subgroup of sarcomas that are difficult to diagnose. Some uSRCSs have specific gene re-arrangements, but others do not. Currently, there is no specific treatments for advanced uSRCSs, and its treatment is largely based on general experience with sarcomas, which includes chemotherapy, targeted therapy, and immunotherapy. In this article, we report a patient with uSRCS who responded to treatment with anti-VEGF inhibitor surufatinib and anti-PD-1 inhibitor camrelizumab after progression on first-line chemotherapy, second-line anlotinib combined with immunotherapy, and third-line chemotherapy.

Case description: In July 2020, a 37-year-old female patient was diagnosed with advanced uSRCS. Results for the Ewing sarcoma RNA binding protein 1 and Wilms tumor suppressor (EWSR1/WT1) fusion gene were negative. The patient was also negative with BCOR (BCL6 co-repressor) and CIC (capicua transcriptional repressor) fusion gene. The next-generation sequencing results revealed point mutations on Phosphatidylinositol-4,5-Bisphosphate 3-Kinase Catalytic Subunit Beta (PIK3CB), Transcription Factor Binding To IGHM Enhancer 3 (TFE3), Mucin 16 (MUC16), and AXL (Axl, also called UFO, ARK, and Tyro7, is part of a family of receptor tyrosine kinases). The patient received 4 cycles of the Ifosfamide and epirubicin hydrochloride regimen, and her best objective response was stable disease. On November 3, 2020, a computed tomography (CT) scan revealed progressive disease (PD). Two cycles of camrelizumab (a programmed death-1 inhibitor) plus anlotinib (an anti- vascular endothelial growth factor drug) were administered, but PD was again observed. Thus, a regimen of gemcitabine plus docetaxel was adopted. Unfortunately, the disease progressed once again after two cycles of the treatment. On February 4, 2021, the patient began to receive targeted therapy with surufatinib combined with camrelizumab. A CT scan showed that the tumor achieved a partial response. As of April 2023, the patient had a progression-free survival time of 26 months.

Conclusions: Surufatinib in combination with camrelizumab could be effective in the treatment of advanced uSRCSs.

Keywords: case report; programmed death-1 inhibitor; surufatinib; target therapy; undifferentiated small round-cell sarcoma.

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Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

Figures

Figure 1
Figure 1
Pathology and immunohistochemistry of a left inguinal lymph node biopsy (A) HE 40x:the tumors appear to be arranged in sheets or nests; (B) HE 400x:The cytoplasm of tumor cells is sparse, the cell boundary is unclear. The round or oval nuclei and nuclear hyperchromatism and fission can be seen. immunohistochemical staining: (C) Vimentin: Diffuse cytoplasmic positive of tumor cells (HE 400x.); (D) INI-1: Nucleus positive (HE 400x.); (E) Ki67: It shows high proliferation of the tumor (400x.). HE, Hematoxylin-eosin stain.
Figure 2
Figure 2
2020–7-10 CT scan: the metastatic tumor in the right lower lobe of the lung and the long diameter was 0.69 cm [red arrow, (A)]. Mediastinal lymph nodes were not significantly enlarged (B). Left acetabular metastases [red arrow, (C)]. Abdominal and abdominal lymph nodes were not significantly enlarged (D) No enlarged lymph nodes were found in the right hilum and the retroperitoneal area. The left pelvic lymph nodes were found and the short diameter was 4.05cm [red arrow, (E)].
Figure 3
Figure 3
2020–9-16 CT scan: The original metastatic tumor in the right lower lobe of the lung disappeared (A); no enlarged lymph nodes were found in the right hilum (B). Left acetabular metastases [red arrow, (C)]. The short diameter of retroperitoneal lymph nodes was 0.91 cm which was a non-confirmed metastases [red arrow, (D)], and the short diameter of left pelvic lymph nodes was 2.98 cm [red arrow, (E)]. The efficacy was SD. SD, stable disease.
Figure 4
Figure 4
2020–11-3 CT scan: The original right lower lobe of the lung metastatic tumor disappeared (A); short diameter of new right hilar lymph node was 0.83 cm [red arrow, (B)]; Left acetabular metastases (red arrow, (C)). The short diameter of retroperitoneal lymph nodes was 1.01 cm which was bigger than before [red arrow, (D)], and the short diameter of left pelvic lymph nodes was 2.76 cm [red arrow, (E)]. The efficacy was PD. PD, progressive disease.
Figure 5
Figure 5
2020–12-22 CT scan: The right lower lobe of the lung metastatic tumor reappeared [red arrow, (A)] and the long diameter was 0.91 cm short diameter of right hilar lymph node was 1.66 cm [red arrow, (B)]; Left acetabular metastases [red arrow, (C)]. the short diameter of retroperitoneal lymph nodes was 1.66 cm, and the short diameter of left pelvic lymph nodes was 3.45 cm [red arrow, (D, E)]. The efficacy was PD. PD, progressive disease.
Figure 6
Figure 6
2021–2-3 CT scan: The right lower lung metastatic tumor was 1.28 cm [red arrow, (A)]; short diameter of right hilar lymph node 2.15 cm [red arrow, (B)]. Left acetabular metastases [red arrow, (C)]. The number of retroperitoneal lymph nodes increased and the short diameter was 1.61 cm [red arrow, (D)], and the left pelvic lymph nodes were 4.02 cm [red arrow, (E)]. The efficacy was PD. PD, progressive disease.
Figure 7
Figure 7
2021–3-9 CT scan: The number of lung metastases increased, and the length of the metastatic tumor in the right lower lobe of lung was 1.62 cm [red arrow, (A)], which was enlarged; short diameter of right hilar lymph node was 2.10 cm [red arrow, (B)]; Left acetabular metastases (C). the short diameter of retroperitoneal lymph nodes was 2.00 cm, and the short diameter of left pelvic lymph nodes was 4.14 cm [red arrow, (D, E)]. The efficacy was unconfirmed progressive disease (iUPD) according irRECIST. irRECIST, immune-related response evaluation criteria in solid tumors.
Figure 8
Figure 8
2021–8-24: Length of metastatic tumor in lower right lung 1.59 cm [red arrow, (A)]; short diameter of right hilar lymph node 1.53 cm [red arrow, (B)]; the short diameter of retroperitoneal lymph nodes was 1.61 cm, and the short diameter of left pelvic lymph nodes was 3.11 cm [red arrow, (C, D)]. The acetabulum was not scanned. The efficacy was SD. SD, stable disease.
Figure 9
Figure 9
2021–12-15: Length of metastatic tumor in lower lobe of lung was 1.13 cm [red arrow, (A)]; short diameter of right hilar lymph node 1.26 cm [red arrow, (B)]; the short diameter of retroperitoneal lymph nodes was 1.56 cm [red arrow, (C)], and the pelvic cavity was not scanned. The efficacy was PR. PR, partial response.
Figure 10
Figure 10
2023–4-13: The original right lower lobe of lung metastatic tumor disappeared (A); The original right hilar enlargement lymph node disappeared (B); Left acetabular metastases [red arrow, (C)]. The short diameter of retroperitoneal lymph nodes was 0.89cm [red arrow, (D)], and the short diameter of left pelvic lymph nodes was 2.13cm [red arrow, (E)].The efficacy was PR. PR, Partial response; SD, Stable disease; PD, Progressive disease; irRECIST, immune-related response evaluation criteria in solid tumors.
Figure 11
Figure 11
A timeline with relevant data from the episode of care.
See this image and copyright information in PMC

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