Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, Minnesota, USA.
Department of Neurosurgery, University of Minnesota, Minneapolis, Minnesota, USA.
World Neurosurg. 2019 Nov;131:90-94. doi: 10.1016/j.wneu.2019.07.142. Epub 2019 Jul 26.
Glioblastoma multiforme (GBM) is a rapid-growing central nervous system neoplasm. We report a case of GBM with extensive intramedullary lumbar drop metastasis and highly unusual osseous spine metastasis from a primary infratentorial GBM occurring 10 years after the initial diagnosis, which to our knowledge has not been described previously.
This 37-year-old man presented with new-onset headaches of increasing severity. Brain magnetic resonance imaging (MRI) demonstrated a heterogeneously enhancing mass in the left superior temporal lobe with adjacent edema. The lesion was initially biopsied in December 2006 and diagnosed as GBM (World Health Organization grade IV) with characteristic features of a highly cellular infiltrating glial neoplasm with nuclear pleomorphism, abundant microvascular proliferation, and abundant necrosis with pseudopalisading nuclei. Ki-67 immunostaining revealed that 15%-20% tumor cell nuclei were positive, indicating a high proliferative index. Histologically, this neoplasm demonstrated characteristic "cell wrapping." Immunoreactivity was variably but strongly positive for glial fibrillary acidic protein in neoplastic cells. In 2018, additional MRI revealed disease throughout the spine and bone biopsy of the thoracic spine showed the same glial neoplasm with primitive neuroectodermal tumor-like components (GBM-PNET).
This case is meant to highlight that, although rare, infratentorial GBM-PNET has a higher frequency of isocitrate dehydrogenase 1 (IDH1) mutation and may metastasize to the spine years after the initial diagnosis despite the likely better prognosis.
多形性胶质母细胞瘤（GBM）是一种快速生长的中枢神经系统肿瘤。我们报告了一例广泛发生于胸髓内的 GBM 肿瘤伴腰椎转移病例，以及一例非常罕见的原发于幕下的 GBM 肿瘤发生于初次诊断后 10 年出现的脊柱骨转移病例，据我们所知，目前尚未有相关文献报道。
这名 37 岁男性出现进行性加重的新发头痛。脑部磁共振成像（MRI）显示左颞叶上份存在混杂强化肿块，伴有周围水肿。该病灶最初于 2006 年 12 月进行活检，并诊断为 GBM（世界卫生组织分级 IV），具有高度细胞浸润性神经胶质肿瘤的特征，包括核异型性、丰富的微血管增生和大量伴有假栅状核的坏死。Ki-67 免疫组化显示 15%-20%的肿瘤细胞核阳性，表明其具有较高的增殖指数。组织学上，该肿瘤具有特征性的“细胞包裹”。肿瘤细胞的神经胶质纤维酸性蛋白免疫反应呈不同程度但强烈阳性。2018 年，进一步的 MRI 显示脊柱多处受累，胸椎骨活检显示相同的神经胶质肿瘤伴有原始神经外胚层肿瘤样成分（GBM-PNET）。
本病例旨在强调，尽管罕见，但幕下 GBM-PNET 具有更高的异柠檬酸脱氢酶 1（IDH1）突变频率，并且尽管可能具有更好的预后，但其仍可能在初次诊断后数年转移至脊柱。
