دورية أكاديمية

Clinical outcomes of patients with CIC-rearranged sarcoma: a single institution retrospective analysis.

التفاصيل البيبلوغرافية
العنوان: Clinical outcomes of patients with CIC-rearranged sarcoma: a single institution retrospective analysis.
المؤلفون: Murphy, Jacob, Resch, Erin E., Leland, Christopher, Meyer, Christian F., Llosa, Nicolas J., Gross, John M., Pratilas, Christine A.
المصدر: Journal of Cancer Research & Clinical Oncology; Mar2024, Vol. 150 Issue 3, p1-12, 12p
مستخلص: Purpose: CIC-rearranged sarcomas represent a type of undifferentiated small round cell sarcoma (USRCS) characterized by poor survival, rapid development of chemotherapy resistance, and high rates of metastasis. We aim to contribute to the growing body of knowledge regarding diagnosis, treatment, clinical course, and outcomes for these patients. Methods: This case series investigates the clinical courses of ten patients with CIC-rearranged sarcoma treated at the Johns Hopkins Hospital from July 2014 through January 2024. Clinical data were retrospectively extracted from electronic medical records. Results: Patients ranged from 10 to 67 years of age at diagnosis, with seven patients presenting with localized disease and three with metastatic disease. Tumors originated from soft tissues of various anatomic locations. Mean overall survival (OS) was 22.1 months (10.6–52.2), and mean progression-free survival (PFS) was 16.7 months (5.3–52.2). Seven patients received intensive systemic therapy with an Ewing sarcoma-directed regimen or a soft tissue sarcoma-directed regimen. Three patients experienced prolonged disease-free survival without systemic treatment. Conclusion: Most patients in this case series demonstrated aggressive clinical courses consistent with those previously described in the literature, although we note a spectrum of clinical outcomes not previously reported. The diversity of clinical courses underscores the need for an improved understanding of individual tumor biology to enhance clinical decision-making and patient prognosis. Despite its limitations, this article broadens the spectrum of reported clinical outcomes, providing a valuable addition to the published literature on this rare cancer. [ABSTRACT FROM AUTHOR]
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قاعدة البيانات: Complementary Index
الوصف
تدمد:01715216
DOI:10.1007/s00432-024-05631-7