Murphy Jacob, Resch Erin E, Leland Christopher, Meyer Christian F, Llosa Nicolas J, Gross John M, Pratilas Christine A
Johns Hopkins University School of Medicine, 733 N Broadway, Baltimore, MD, 21205, USA.
Department of Oncology, The Sidney Kimmel Comprehensive Cancer Center, Johns Hopkins University School of Medicine, 1650 Orleans St, Baltimore, MD, 21287, USA.
J Cancer Res Clin Oncol. 2024 Mar 4;150(3):112. doi: 10.1007/s00432-024-05631-7.
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.
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.
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.
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.
CIC重排肉瘤是一种未分化的小圆细胞肉瘤（USRCS），其特点是生存率低、化疗耐药性迅速发展且转移率高。我们旨在为关于这些患者的诊断、治疗、临床病程及预后的知识积累做出贡献。
本病例系列研究了2014年7月至2024年1月在约翰霍普金斯医院接受治疗的10例CIC重排肉瘤患者的临床病程。临床数据从电子病历中回顾性提取。
患者诊断时年龄在10至67岁之间，7例为局限性疾病，3例为转移性疾病。肿瘤起源于不同解剖部位的软组织。平均总生存期（OS）为22.1个月（10.6 - 52.2），平均无进展生存期（PFS）为16.7个月（5.3 - 52.2）。7例患者接受了以尤因肉瘤为导向的方案或软组织肉瘤为导向的方案的强化全身治疗。3例患者未经全身治疗却经历了较长的无病生存期。
本病例系列中的大多数患者表现出与先前文献中描述一致的侵袭性临床病程，尽管我们注意到了一些先前未报道的临床结局范围。临床病程的多样性强调了需要更好地了解个体肿瘤生物学，以加强临床决策和改善患者预后。尽管存在局限性，但本文拓宽了报道的临床结局范围，为关于这种罕见癌症的已发表文献增添了有价值的内容。
