Targeting the Tumor Microenvironment of Ewing Sarcoma

Morales, Erin; Olson, Michael; Iglesias, Fiorella; Luetkens, Tim; Atanackovic, Djordje

doi:10.2217/imt-2020-0341

Cited by 7 publications

(7 citation statements)

References 104 publications

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“…Immunotherapy involving epigenetic and specific pathways to the tumor and its microenvironment, including monoclonal antibodies, checkpoint inhibition, cancer vaccines, and genetically engineered T cells, has been currently studied in the preclinical setting and clinical trials and is a promising new approach. 30 In summary, we present the clinicopathologic and molecular features of 25 cases of ES arising primarily in the GI tract. GI ES has a strong predilection for the small intestine.…”

Section: Discussionmentioning

confidence: 99%

“…The presence of abdominopelvic dissemination and metastatic disease portended a poor prognosis. Immunotherapy involving epigenetic and specific pathways to the tumor and its microenvironment, including monoclonal antibodies, checkpoint inhibition, cancer vaccines, and genetically engineered T cells, has been currently studied in the preclinical setting and clinical trials and is a promising new approach 30 …”

Section: Discussionmentioning

confidence: 99%

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Gastrointestinal Ewing Sarcoma

Yin,

Shao,

Sun

et al. 2023

American Journal of Surgical Pathology

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Occurrence of extraskeletal Ewing sarcoma (ES) in the gastrointestinal (GI) tract is extremely rare. Here, we report 25 cases of ES arising primarily in the GI tract with a focus on the clinicopathological and molecular features, differential diagnosis, and biological behavior. Thirteen patients (52%) were male, and 12 (48%) were female with age ranging from 9 to 59 years (mean: 36.2 years; median: 38 years). Twenty-one tumors (84%) occurred in the small intestine, 3 (12%) in the stomach, and 1 (4%) in the anal canal. At operation, 8/18 (44.4%) patients presented with abdominopelvic disseminated disease. Tumor size measured from 2 to 25 cm (mean: 8.2 cm; median: 6 cm) in maximum size. Microscopically, the tumors were composed of infiltrative small round, ovoid, or short spindle cells arranged mostly in lobular and solid sheet-like patterns with a rich capillary vasculature. Focal formation of Homer Wright–type rosettes and pseudoalveolar architecture was noted each in 2 (8%) cases and 3 (12%) cases. Besides CD99 (25/25; 100%), Fli-1 (15/15, 100%), and NKX2.2 (14/16; 87.5%), the tumor cells also showed variable staining of CD117 (14/17; 82.4%). Of 25 cases, 23 (92%) demonstrated EWSR1 rearrangement by fluorescence in situ hybridization analysis. The 2 cases with negative fluorescence in situ hybridization results were found to harbor EWSR1::ERG and EWSR1::FLI1 fusion by further RNA sequencing, respectively, with a median follow-up of 12 months (range: 1 to 42 months), 5/19 (26.3%) patients developed visceral metastasis and 12/19 (63.2%) patients died of the disease (range:1 to 33 months; median: 9 months). This study showed that GI ES had a predilection for the small intestine, although other sites of the GI tract could also be involved. GI ES had a poor prognosis with a high rate of mortality, particularly in patients with abdominopelvic disseminated disease. In light of appropriate therapeutic strategies and prognostic considerations, it is essential not to misdiagnose GI ES as gastrointestinal stromal tumor owing to the expression of aberrant CD117.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Gastrointestinal Ewing Sarcoma

Yin,

Shao,

Sun

et al. 2023

American Journal of Surgical Pathology

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“… 270 , 271 Therefore, DC-based vaccines are the most common vaccination approach for pediatric sarcomas, and patients who received a DC vaccine had a prolonged survival time compared with control patients. 272 , 273 In some encouraging cases, patients with recurrent or metastatic Ewing’s sarcoma, even in the presence of chemotherapy-induced immune cell loss, have shown significant improvement and extended survival time after receiving immunotherapy such as the DC vaccines, influenza vaccines, and autologous T lymphocytes. 274 Cancer-associated vaccines have effectively enhanced T lymphocyte-mediated immunity even after treatment with chemotherapy or radiotherapy, but they have not yet achieved satisfactory effects in the treatment of most solid tumors, including osteosarcoma.…”

Section: Conventional Immunotherapy For Osteosarcomamentioning

confidence: 99%

Managing the immune microenvironment of osteosarcoma: the outlook for osteosarcoma treatment

Tian

Cao

et al. 2023

Bone Res

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Osteosarcoma, with poor survival after metastasis, is considered the most common primary bone cancer in adolescents. Notwithstanding the efforts of researchers, its five-year survival rate has only shown limited improvement, suggesting that existing therapeutic strategies are insufficient to meet clinical needs. Notably, immunotherapy has shown certain advantages over traditional tumor treatments in inhibiting metastasis. Therefore, managing the immune microenvironment in osteosarcoma can provide novel and valuable insight into the multifaceted mechanisms underlying the heterogeneity and progression of the disease. Additionally, given the advances in nanomedicine, there exist many advanced nanoplatforms for enhanced osteosarcoma immunotherapy with satisfactory physiochemical characteristics. Here, we review the classification, characteristics, and functions of the key components of the immune microenvironment in osteosarcoma. This review also emphasizes the application, progress, and prospects of osteosarcoma immunotherapy and discusses several nanomedicine-based options to enhance the efficiency of osteosarcoma treatment. Furthermore, we examine the disadvantages of standard treatments and present future perspectives for osteosarcoma immunotherapy.

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“…Emerging data suggest an alternative schedule of trabectedin in combination with low‐dose irinotecan may be effective with confirmed partial responses reported 42 . Finally, immunotherapy has not yet demonstrated robust activity in EWS with active ongoing efforts exploring this potential therapeutic approach 43,44 …”

Section: State Of the Disease: Biologicalmentioning

confidence: 99%

Children's Oncology Group's 2023 blueprint for research: Bone tumors

Reed,

Grohar,

Rubin

et al. 2023

Pediatric Blood & Cancer

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The Children's Oncology Group (COG) Bone Tumor Committee is responsible for clinical trials and biological research on localized, metastatic, and recurrent osteosarcoma and Ewing sarcoma (EWS). Results of clinical trials in localized disease completed and published in the past 10 years have led to international standard‐of‐care chemotherapy for osteosarcoma and EWS. A recent focus on identifying disease subgroups has led to the identification of biological features associated with poor outcomes including the presence of circulating tumor DNA (ctDNA) at diagnosis, and specific genomic alterations—MYC amplification for osteosarcoma and STAG2 and TP53 mutation for EWS. Studies validating these potential biomarkers are under way. Clinical trials evaluating the addition of multitargeted kinase inhibitors, which are active in relapsed bone sarcomas, to standard chemotherapy are under way in osteosarcoma and planned in EWS. In addition, the Committee has data analyses and a clinical trial under way to evaluate approaches to local management of the primary tumor and metastatic sites. Given the rarity of bone sarcomas, we have prioritized international interactions and are in the process of forming an international data‐sharing consortium to facilitate refinement of risk stratification and study of rare disease subtypes.

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Targeting the Tumor Microenvironment of Ewing Sarcoma

Cited by 7 publications

References 104 publications

Gastrointestinal Ewing Sarcoma

Gastrointestinal Ewing Sarcoma

Managing the immune microenvironment of osteosarcoma: the outlook for osteosarcoma treatment

Children's Oncology Group's 2023 blueprint for research: Bone tumors

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