LSUHSC, Pathology, New Orleans, Louisiana, USA.
Rutgers Robert Wood Johnson Medical School, Pathology, New Brunswick, New Jersey, USA.
Fetal Pediatr Pathol. 2020 Jun;39(3):194-245. doi: 10.1080/15513815.2019.1641859. Epub 2019 Jul 25.
Lumbosacral spinal lipomas and lipomyeloceles are usually identified in early childhood. Terminology, histopathology, and diagnosis for these malformations can be confusing. This is a PubMed review with comparison of embryology, gross, and histopathology, and reporting requisites for these and related closed spinal malformations. The spinal lipoma group (congenital spinal lipomatous malformations) includes subcutaneous, transdural, intradural, and noncontiguous malformations stretching through the entire lower spinal region. This lipomyelocele trajectory overlaps the embryonic tail's caudal eminence. Histopathologically, the lipomyelocele spectrum is a heterogeneous, stereotypical set of findings encountered from dermis to spinal cord. Diagnosis requires detailed correlation of images, intraoperative inspection, and histopathology. Appropriate terminology and clinicopathologic correlation to arrive at a diagnosis is a critical activity shared by pathologist and clinician. Prognostic and management differences depend on specific diagnoses. Familial and genetic influences play little if any role in patient management in closed spinal malformations.
腰骶部脊髓脂肪瘤和脊髓脊膜膨出通常在儿童早期被发现。这些畸形的术语、组织病理学和诊断可能会令人困惑。这是一篇基于 PubMed 的综述，比较了胚胎学、大体和组织病理学，并对这些和相关的闭合性脊髓畸形的报告要求进行了比较。脊髓脂肪瘤组（先天性脊髓脂肪瘤畸形）包括皮下、硬脊膜外、硬脊膜内和非连续畸形，延伸穿过整个下脊柱区域。这种脊髓脊膜膨出的轨迹与胚胎尾部的尾骨隆起重叠。组织病理学上，脊髓脊膜膨出的范围是从真皮到脊髓的一组异质的、典型的发现。诊断需要详细的图像、术中检查和组织病理学的相关性。通过适当的术语和临床病理相关性来做出诊断，是病理学家和临床医生共同的关键活动。预后和治疗管理的差异取决于具体的诊断。在闭合性脊髓畸形中，家族性和遗传性影响对患者的管理几乎没有影响。
