Layfield L J, Liu K, Dodd L G, Olatidoye B A
Department of Pathology, University of Utah Health Sciences Center, Salt Lake City 84132, USA.
Diagn Cytopathol. 1998 Nov;19(5):378-81. doi: 10.1002/(sici)1097-0339(199811)19:5<378::aid-dc14>3.0.co;2-c.
Chordomas are relatively rare neoplasms occurring at both ends of a neuro axis. The majority follow an indolent course of multiple local recurrences, ultimately leading to the patient's death. Rare examples have been associated with sarcomatous components, usually resembling malignant fibrous histiocytoma, fibrosarcoma, or osteosarcoma. These tumors have followed a more aggressive course, with poor response to treatment and death following a relatively short time course. Cytologic material from a single case obtained by fine-needle aspiration revealed a high-grade malignancy composed of short atypical spindle cells containing modest amounts of granular cytoplasm. Physaliphorous cells were absent, and myxoid material was not a significant component of the smears. Rare polygonal cells with foamy cytoplasm were identified. Histologic study revealed a high-grade spindle-cell sarcoma in which were scattered small aggregates of vacuolated cells resembling physaliphorous cells. Anaplastic giant cells were present, and the overall appearance of the neoplasm resembled a malignant fibrous histiocytoma. Despite the radiographic appearance of a conventional chordoma in this case, the cytologic findings were indicative of a high-grade sarcoma consistent with a dedifferentiated chordoma.
脊索瘤是发生于神经轴两端的相对罕见的肿瘤。大多数肿瘤呈惰性病程，多次局部复发，最终导致患者死亡。罕见病例与肉瘤成分相关，通常类似恶性纤维组织细胞瘤、纤维肉瘤或骨肉瘤。这些肿瘤病程更具侵袭性，对治疗反应不佳，在相对较短的病程后死亡。通过细针穿刺获取的单个病例的细胞学材料显示为高级别恶性肿瘤，由含有适量颗粒状胞质的短非典型梭形细胞组成。未见泡状核细胞，黏液样物质不是涂片的主要成分。发现罕见的具有泡沫状胞质的多边形细胞。组织学研究显示为高级别梭形细胞肉瘤，其中散在分布着类似泡状核细胞的小空泡状细胞聚集。存在间变性巨细胞，肿瘤的整体外观类似恶性纤维组织细胞瘤。尽管该病例的影像学表现为典型的脊索瘤，但细胞学结果提示为与去分化脊索瘤一致的高级别肉瘤。
