Departments of Nuclear Medicine and Radiology, Maidstone and Tunbridge Wells NHS Trust, Maidstone, UK.
Eur J Nucl Med Mol Imaging. 2012 Feb;39 Suppl 1:S61-7. doi: 10.1007/s00259-011-1990-y.
Neuroectodermal tumours arise from chromaffin cells and possess the ability to secrete catecholamines. They are generally rare and may occur in association with a variety of hereditary syndromes such as MEN-2A and 2B, neurofibromatosis type 1 and von Hippel-Lindau disease. The most common types are phaeochromocytoma arising from the adrenal medulla and paraganglioma of extra-adrenal origin. Phaeochromocytomas tend to be benign and are often associated with a gene mutation if the disease is bilateral, while paragangliomas are often malignant, have a more aggressive nature and tend to metastasize. There are no specific histological or immunohistochemical features that indicate the malignant potential and the diagnosis of malignancy can only be established by the presence of distant metastases. Therefore, imaging can play a vital role in the diagnosis, localization, staging and assessment of spread. Traditionally, this is achieved with a combination of cross-sectional (CT and MRI) and functional ((123)I-MIBG or (111)In-octreotide) imaging. However, these modalities are not adequate and achieve moderate sensitivity. The introduction of (68)Ga-DOTA peptide in PET/CT imaging has led to improved receptor targeting and superb PET resolution, as well as accurate localization of lesions. The use of this technique in neuroectodermal tumours has been shown to be superior to all available modalities, but the available data are limited and larger studies are awaited to establish its role in the management of these tumours.
神经外胚层肿瘤起源于嗜铬细胞,具有分泌儿茶酚胺的能力。它们通常很少见,可能与多种遗传性综合征有关,如 MEN-2A 和 2B、神经纤维瘤病 1 型和 von Hippel-Lindau 病。最常见的类型是来自肾上腺髓质的嗜铬细胞瘤和肾上腺外来源的副神经节瘤。嗜铬细胞瘤往往是良性的,如果疾病是双侧的,通常与基因突变有关,而副神经节瘤往往是恶性的,具有更具侵袭性的性质,并倾向于转移。没有特定的组织学或免疫组织化学特征可以表明恶性潜能,恶性的诊断只能通过远处转移的存在来确定。因此,影像学可以在诊断、定位、分期和扩散评估中发挥重要作用。传统上,这是通过结合横断面(CT 和 MRI)和功能((123)I-MIBG 或 (111)In-octreotide)成像来实现的。然而,这些方法并不充分,只能达到中等的敏感性。(68)Ga-DOTA 肽在 PET/CT 成像中的引入导致了受体靶向和极好的 PET 分辨率的提高,以及病变的准确定位。该技术在神经外胚层肿瘤中的应用已被证明优于所有现有方法,但现有数据有限,需要更大的研究来确定其在这些肿瘤管理中的作用。