Comparison of Features and Outcomes of Undifferentiated Pleomorphic Sarcoma of Bone and Soft Tissue

Gusho, Charles A; Lee, Linus; Guntin, Jonathan; Blank, Alan T.

doi:10.1016/j.jss.2021.09.032

Cited by 5 publications

(3 citation statements)

References 24 publications

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“…Soft tissue sarcomas (STSs) are rare malignant tumors of mesenchymal origin that account for approximately 1% of adult malignancies ( 12 ), with a large number of approximately 50 subtypes, of which the less differentiated ones that can exhibit multiple cellular forms are called undifferentiated pleomorphic sarcomas. Undifferentiated pleomorphic sarcomas can occur in any part of the body, with the extremities being the most common site (50% in the lower extremities and 20% in the upper extremities) ( 13 ), with only a few occurring in the retroperitoneum.…”

Section: Discussionmentioning

confidence: 99%

Retroperitoneal undifferentiated pleomorphic sarcoma with total nephrectomy: a case report and literature review

Wang

Fan

et al. 2023

Front. Surg.

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BackgroundUndifferentiated pleomorphic sarcoma (UPS) is a highly malignant soft tissue sarcoma with a poor prognosis and no clear effective clinical means for treatment, and there has been no significant progress in research within this field in recent years. This study aimed to investigate the epidemiology, etiology, clinical features, diagnostic modalities, various treatment modalities, and prognosis of retroperitoneal undifferentiated pleomorphic sarcoma and to contribute to the clinical management of this type of disease. In this study, we report a case of undifferentiated pleomorphic sarcoma with a primary origin in the retroperitoneum. Undifferentiated pleomorphic sarcoma occurring in the retroperitoneum is rarely reported.Case descriptionA 59-year-old man with abdominal distension and pain for 4 months presented to our hospital after the failure of conservative treatment. A 9.6 cm by 7.4 cm mass in the left retroperitoneum was found on a CT scan of the whole abdomen with three degrees of enhancement. After surgical treatment, the tumor and the left kidney were completely removed, and pathological examination and genetic sequencing showed an apparent undifferentiated pleomorphic sarcoma. The patient subsequently declined follow-up treatment and is currently alive and well.ConclusionsAt the current level of clinical technology, the treatment of undifferentiated pleomorphic sarcoma is still in the exploratory stage, and the scarcity of clinical cases of this disease may have hindered the acquisition of clinical trials and research data for this disease. At present, the first choice of treatment for undifferentiated pleomorphic sarcoma is still radical resection. In the existing clinical studies, there are no strong data to support the effect of preoperative neoadjuvant chemoradiotherapy and adjuvant chemoradiotherapy in clinical practice. Similar to other diseases, the use of radiotherapy and chemotherapy before and after surgery may be a potential treatment for this disease in the future. Targeted therapy for this disease still needs further exploration, and we need more reports on related diseases to promote future treatment and research on this disease.

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Section: Discussionmentioning

confidence: 99%

Retroperitoneal undifferentiated pleomorphic sarcoma with total nephrectomy: a case report and literature review

Wang

Fan

et al. 2023

Front. Surg.

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“…With age, cancer diagnoses become more prevalent. Sarcoma, a less common cancer type, constitutes roughly 1% of adult malignancies [ 1 ]. Among the various subtypes, undifferentiated pleomorphic sarcoma (UPS) stands as a prominent diagnosis, accounting for 17.1% of cases.…”

Section: Introductionmentioning

confidence: 99%

A Rare Presentation of Undifferentiated Pleomorphic Sarcoma in the Subpectoral Space

Arthurs,

Suening,

Barrar

et al. 2023

Cureus

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Soft tissue sarcomas (STS) are often described as asymptomatic, rapidly expanding masses, particularly in the extremities or trunk. Undifferentiated pleomorphic sarcoma (UPS), a high-grade variant of STS, ranks as the second most prevalent subtype in the United States. It predominantly affects males between their fifth and seventh decades. Its often benign symptomatology, however, can lead to initial misdiagnosis and subsequent mismanagement. We present the case of a 57-year-old Caucasian male, previously in good health, who experienced a recurring subpectoral lesion causing discomfort and mass-related effects. Initial management included incision and drainage, which provided temporary relief. The biopsy revealed a diagnosis of grade 3 UPS. The lesion's recurrence two months later was accompanied by local invasion into adjacent skin and musculature as well as metastasis to the right hemiliver. A comprehensive understanding of UPS among medical professionals is vital for accurate diagnosis and facilitating prompt intervention to prevent avoidable complications and optimize patient outcomes.

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“…Bone neoplasms are rare solid tumors, accounting for less than 2% of all primary malignancies. SUPS is an extremely rare and aggressive malignancy representing <1% of all primary malignant bone tumors ( 6 ). SUPS often occurs in the bone diaphysis or metaphysis and results in invasive bone damage and a soft tissue mass.…”

Section: Introductionmentioning

confidence: 99%

Single-cell sequencing reveals the landscape of the tumor microenvironment in a skeletal undifferentiated pleomorphic sarcoma patient

et al. 2022

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Skeletal undifferentiated pleomorphic sarcoma (SUPS) is an invasive pleomorphic soft tissue sarcoma with a high degree of malignancy and poor prognosis. It is prone to recur and metastasize. The tumor microenvironment (TME) and the pathophysiology of SUPS are barely described. Single-cell RNA sequencing (scRNA-seq) provides an opportunity to dissect the landscape of human diseases at an unprecedented resolution, particularly in diseases lacking animal models, such as SUPS. We performed scRNA-seq to analyze tumor tissues and paracancer tissues from a SUPS patient. We identified the cell types and the corresponding marker genes in this SUPS case. We further showed that CD8+ exhausted T cells and Tregs highly expressed PDCD1, CTLA4 and TIGIT. Thus, PDCD1, CTLA4 and TIGIT were identified as potential targets in this case. We applied copy number karyotyping of aneuploid tumors (CopyKAT) to distinguish malignant cells from normal cells in fibroblasts. Our study identified eight malignant fibroblast subsets in SUPS with distinct gene expression profiles. C1-malignant Fibroblast and C6-malignant Fibroblast in the TME play crucial roles in tumor growth, angiogenesis, metastasis and immune response. Hence, targeting malignant fibroblasts could represent a potential strategy for this SUPS therapy. Intervention via tirelizumab enabled disease control, and immune checkpoint inhibitors (ICIs) of PD-1 may be considered as the first-line option in patients with SUPS. Taken together, scRNA-seq analyses provided a powerful basis for this SUPS treatment, improved our understanding of complex human diseases, and may afforded an alternative approach for personalized medicine in the future.

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Comparison of Features and Outcomes of Undifferentiated Pleomorphic Sarcoma of Bone and Soft Tissue

Cited by 5 publications

References 24 publications

Retroperitoneal undifferentiated pleomorphic sarcoma with total nephrectomy: a case report and literature review

Retroperitoneal undifferentiated pleomorphic sarcoma with total nephrectomy: a case report and literature review

A Rare Presentation of Undifferentiated Pleomorphic Sarcoma in the Subpectoral Space

Single-cell sequencing reveals the landscape of the tumor microenvironment in a skeletal undifferentiated pleomorphic sarcoma patient

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