Intraosseous Benign Notochord Cell Tumors (BNCT): Further evidence supporting a relationship to chordoma
Intraosseous Benign Notochord Cell Tumors (BNCT): Further evidence supporting a relationship to chordoma
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DOI：
10.1097/pas.0b013e31805c9967
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发表时间：
2007-10-01
影响因子：
5.6
通讯作者：
Rosenberg, Andrew E.
中科院分区：
文献类型：
--
作者：
Deshpande, Vikram;Nielsen, GunnIaligur Petur;Rosenberg, Andrew E.
背景：先前的研究已经证实中轴骨内存在良性脊索细胞瘤。有证据表明，它们可能与脊索瘤的发展有关。为了进一步研究BNCT和经典的脉络膜之间的关系，我们回顾了一个大的系列切除骶骨/尾骨脊索瘤，试图确定共存的BNCTs.Design的存在：82骶骨切除/尾骨切除标本进行脉络膜进行了鉴定。对可用的苏木精和伊红切片进行了审查，以确定BNCT并评估其与共存脉络膜的关系。根据先前的描述，BNCT被定义为大细胞的粘性聚集体，由于其空泡化的细胞质而呈现脂肪细胞样。结果：我们发现6个BNCTs，每一个都与骶骨脉络膜相邻但分开。其中女性5例，男性1例，平均年龄58岁。骶骨出现5处病变。1例病变发生在尾骨，累及2个相邻椎体节段。BNCTs的大小从1到20 mm不等，平均大小为6.1 mm。病变完全由脂肪细胞样细胞核组成，没有明显的核内分泌物或粘液样基质。3个病灶内有骨质小梁，1例病灶内有造血成分。在所有的情况下，脉络膜是传统的类型和形态学不同的BNCT.Conclusions：BNCTs被确定在7.3%的骶骨/尾骨切除原发性脉络膜。我们推测，这一发现提供了进一步的证据，BNCT是脉络膜炎的前驱病变。需要进行更多的调查，以进一步了解这种关系。
Background: Previous studies have documented the existence of intraosseous benign notochordal cell tumors (BNCTs) within the axial skeleton. Evidence suggests that they may be associated with the development of chordomas. To further investigate the relationship between BNCT and classic chordoma, we reviewed a large series of resected sacral/coccygeal chordomas in an attempt to identify the presence of coexisting BNCTs.Design: Eighty-two sacrectomy/coccygectomy specimens performed for chordoma were identified. Available hematoxylin and eosin slides were reviewed to identify BNCTs and assess their relationship with the coexisting chordoma. BNCTs were defined, in accordance with prior descriptions, as cohesive aggregates of large cells that appeared adipocytelike because of their vacuolated cytoplasm. The cells exhibited only minimal nuclear atypia and lacked lobulation and myxoid stroma.Results: We identified 6 BNCTs, each was adjacent to but separate from the sacral chordoma. There were 5 females and 1 male, and the mean age was 58 years. Five lesions arose in the sacrum. One lesion arose in the coccyx, and involved 2 contiguous vertebral levels. The BNCTs ranged in size from I to 20 mm with a mean size of 6.1 mm. The lesions were exclusively composed of adipocytelike nuclei without significant nuclear atypia or myxoid stroma. Three lesions contained sclerotic bony trabeculae and intralesional hernatopoietic elements were identified in 1 case. In all cases the chordoma was of the conventional type and were morphologically different from the BNCT.Conclusions: BNCTs were identified in 7.3% of sacral/coccygeal resections performed for primary chordoma. We speculate that this finding provides further evidence that BNCT is the precursor lesion for chordoma. Additional investigations are needed to further understand this relationship.