Department of Neurosurgery, University of Oklahoma, Oklahoma City, Oklahoma.
Department of Pathology, University of Oklahoma, Oklahoma City, Oklahoma.
World Neurosurg. 2024 May;185:e1093-e1100. doi: 10.1016/j.wneu.2024.03.028. Epub 2024 Mar 13.
Oligosarcoma is a rare central nervous system (CNS) neoplasm that may arise following oligodendroglioma resection, which demonstrates a unique genetic profile and aggressive clinical phenotype. We present a systematic review and illustrative case example emphasizing the clinical and prognostic features of this unusual and unfavorable neuro-oncologic disease.
Systematic literature review and illustrative case report.
A 41-year-old man who had undergone 2 neurosurgical resections for a World Health Organization grade II oligodendroglioma (Ki-67 = 5-10%, 1p/19q codeleted, IDH2 mutated), without adjuvant chemoradiation, presented with seizures seven years after resection. An extra-axial mass was identified adjacent to the resection cavity, in which gross total resection was achieved. Pathology confirmed World Health Organization grade IV oligosarcoma (Ki-67 = 20%). Adjuvant chemoradiation was initiated, with disease control observed over 6 months of follow-up. Seven publications met inclusion criteria. Oligosarcoma has been confirmed in 36 lesions, arising in 35 patients; 5 were primary oligosarcoma, while 31 occurred in the setting of prior resected oligodendroglioma or oligoastrocytoma. Features shared by these lesions include regain of H3K27me3 expression, 1p/19q codeletion, homozygous deletion of CDKN2A/B, loss of 6q, loss of NF1 and YAP1, and attenuation of CpG island methylator. Median survival after oligosarcoma diagnosis was 1.3 years (range, 0-5.2; n = 35).
Oligosarcoma is a prognostically unfavorable CNS neoplasm with characteristic imaging and pathologic features, and a strong association with previously resected oligodendroglioma. Aggressive treatment is recommended, including gross total resection and adjuvant chemoradiation. Further study is required to define optimal treatment protocol for this CNS malignancy.
少突肉瘤是一种罕见的中枢神经系统（CNS）肿瘤，可能在少突胶质细胞瘤切除后发生，其具有独特的遗传特征和侵袭性临床表型。我们进行了系统的文献回顾和病例举例，强调了这种罕见且不良的神经肿瘤疾病的临床和预后特征。
系统的文献回顾和病例举例报告。
一名 41 岁男性，曾因世界卫生组织（WHO）二级少突胶质细胞瘤（Ki-67=5-10%，1p/19q 共缺失，IDH2 突变）接受了 2 次神经外科切除术，未接受辅助放化疗，在切除后 7 年出现癫痫发作。在切除腔附近发现一个轴外肿块，行大体全切除。病理证实为世界卫生组织（WHO）四级少突肉瘤（Ki-67=20%）。患者开始接受辅助放化疗，在 6 个月的随访中观察到疾病得到控制。有 7 篇文献符合纳入标准。少突肉瘤已在 35 例患者的 36 个病变中得到证实；其中 5 例为原发性少突肉瘤，而 31 例发生在先前切除的少突胶质细胞瘤或少突星形细胞瘤的背景下。这些病变的共同特征包括 H3K27me3 表达恢复、1p/19q 共缺失、CDKN2A/B 纯合缺失、6q 缺失、NF1 和 YAP1 缺失以及 CpG 岛甲基化减弱。在诊断为少突肉瘤后，中位生存时间为 1.3 年（范围为 0-5.2 年；n=35）。
少突肉瘤是一种预后不良的中枢神经系统肿瘤，具有特征性的影像学和病理学特征，与先前切除的少突胶质细胞瘤密切相关。建议采用积极的治疗方法，包括大体全切除和辅助放化疗。需要进一步研究来确定这种中枢神经系统恶性肿瘤的最佳治疗方案。
