Clinical outcomes of patients with CIC-rearranged sarcoma: a single institution retrospective analysis. 2024

Jacob Murphy, and Erin E Resch, and Christopher Leland, and Christian F Meyer, and Nicolas J Llosa, and John M Gross, and Christine A Pratilas
Johns Hopkins University School of Medicine, 733 N Broadway, Baltimore, MD, 21205, USA.

OBJECTIVE 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. CONCLUSIONS 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.

UI MeSH Term Description Entries
D006761 Hospitals Institutions with an organized medical staff which provide medical care to patients. Hospital
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000066491 Clinical Decision-Making Process of formulating a diagnosis based on medical history and physical or mental examinations, and/or choosing an appropriate intervention. Medical Decision-Making,Clinical Decision Making,Decision-Making, Clinical,Decision-Making, Medical,Medical Decision Making
D012189 Retrospective Studies Studies used to test etiologic hypotheses in which inferences about an exposure to putative causal factors are derived from data relating to characteristics of persons under study or to events or experiences in their past. The essential feature is that some of the persons under study have the disease or outcome of interest and their characteristics are compared with those of unaffected persons. Retrospective Study,Studies, Retrospective,Study, Retrospective
D012509 Sarcoma A connective tissue neoplasm formed by proliferation of mesodermal cells; it is usually highly malignant. Sarcoma, Epithelioid,Sarcoma, Soft Tissue,Sarcoma, Spindle Cell,Epithelioid Sarcoma,Epithelioid Sarcomas,Sarcomas,Sarcomas, Epithelioid,Sarcomas, Soft Tissue,Sarcomas, Spindle Cell,Soft Tissue Sarcoma,Soft Tissue Sarcomas,Spindle Cell Sarcoma,Spindle Cell Sarcomas
D012512 Sarcoma, Ewing A malignant tumor of the bone which always arises in the medullary tissue, occurring more often in cylindrical bones. The tumor occurs usually before the age of 20, about twice as frequently in males as in females. Ewing's Tumor,Sarcoma, Ewing's,Ewing Sarcoma,Ewing Tumor,Ewing's Sarcoma,Ewings Sarcoma,Ewings Tumor,Sarcoma, Ewings,Tumor, Ewing,Tumor, Ewing's

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