Bydon Ali, Gutierrez Jorge A, Mahmood Asim
Hermelin Brain Tumor Center, Henry Ford Hospital, Detroit, MI 48202, USA.
J Neurooncol. 2003 Sep;64(3):259-63. doi: 10.1023/a:1025628802228.
A 79-year-old female presented with difficulty ambulating and was found to have weakness and hyperreflexia in the lower extremities. Magnetic resonance imaging (MRI) revealed a large T8-T9 intraspinal tumor. She underwent a thoracic laminectomy, and excision of an intradural extramedullary lesion. The surgical specimen was soft, black tissue that consisted of a moderately cellular, deeply pigmented tumor. The neoplastic cells proved to be melanocytic, and were devoid of overt features of anaplasia, i.e., prominent nuclear pleomorphism, necrosis, significant mitotic activity, and high proliferation indices. Four months postoperatively, MRI demonstrated focal areas of enhancement in the conus medullaris and in the fourth ventricle, indicating leptomeningeal spread. Subsequently, the patient underwent whole brain radiation. On repeat imaging, there was nodular enhancement of the fourth ventricle and throughout the spinal cord. Despite chemotherapy and radiation therapy, the disease advanced and the patient expired. Meningeal melanocytoma is a rare, histologically benign tumor with good prognosis. However, local aggressive behavior has been recorded, especially in cases of subtotal gross resection. On a literature review, there was one case of cranial posterior fossa meningeal melanocytoma with associated lesions in both suprarenal glands and the left kidney, but there were no cases with distant metastasis. In this report, we present an unusual case of spinal meningeal melanocytoma with diffuse spread throughout the craniospinal axis that proved to be fatal.
一名79岁女性因行走困难就诊,检查发现下肢无力及反射亢进。磁共振成像(MRI)显示胸8 - 胸9水平有一巨大椎管内肿瘤。她接受了胸椎椎板切除术及硬脊膜内髓外病变切除术。手术标本为柔软的黑色组织,由中等细胞密度、色素沉着深的肿瘤组成。肿瘤细胞经证实为黑素细胞性,无明显间变特征,即无显著核多形性、坏死、明显的有丝分裂活性及高增殖指数。术后4个月,MRI显示圆锥和第四脑室内有局灶性强化区,提示软脑膜播散。随后,患者接受了全脑放疗。复查影像学检查时,第四脑室及整个脊髓出现结节状强化。尽管进行了化疗和放疗,病情仍进展,患者最终死亡。脑膜黑素细胞瘤是一种罕见的组织学上良性的肿瘤,预后良好。然而,已记录到其具有局部侵袭性行为,尤其是在次全切除的病例中。经文献检索,有1例颅后窝脑膜黑素细胞瘤伴有双侧肾上腺及左肾相关病变,但无远处转移病例。在本报告中,我们呈现了1例不寻常的脊髓脑膜黑素细胞瘤病例,其在整个颅脊髓轴呈弥漫性播散,最终导致患者死亡。