El-Khashab Mostafa, Koral Korgun, Bowers Daniel C, Johnson-Welch Sarah, Swift Dale, Nejat Farideh
Advanced Neurosurgical Associates, Hackensack, NJ, USA.
Childs Nerv Syst. 2009 Apr;25(4):407-10. doi: 10.1007/s00381-008-0782-6. Epub 2009 Jan 13.
Meningeal melanocytoma is a rare, benign melanotic tumor of the leptomeninges, which occurs anywhere in the cranial or spinal regions but most commonly in supratentorial and thoracic spine regions. The literature on this entity consists of case reports; therefore, there is no agreement on the most effective therapy of this tumor, although total excision seems to be the best therapeutic option.
We report a 17-year-old girl with intermediate grade meningeal melanocytoma involving the C6 nerve root with spinal cord compression resulted in progressive tetraparesis. Clinical and radiological examinations suggested the possibility of an intradural extramedullary solid mass. The tumor was removed subtotally through cervical laminotomy followed by rapid improvement of most neurological deficits. This tumor was unusual because of its very hyperintense homogenous signal on T1-weighted images, invasion of the arachnoid membrane, and extension into the neural foramina. Black dots on the surface of the cord were thought to represent an organized blood clot until the frozen section suggested a melanocytic tumor.
We discuss the distinction of meningeal melanocytoma from other melanocytic tumors of the leptomeninges.
Melanocytic tumors should be considered in the differential diagnosis when a hyperintense lesion of the leptomeninges is identified on T1-weighted images or a very dark mass similar to charcoal or organized hematoma is found in the surgical field. The best management is complete tumor resection, but radiotherapy is reserved in cases of subtotal resection and multiple lesions. Locally aggressive nature of tumor and possibility of recurrence warrant regular follow-up.
脑膜黑素细胞瘤是一种罕见的软脑膜良性黑素性肿瘤,可发生于颅部或脊柱的任何部位,但最常见于幕上和胸椎区域。关于该疾病的文献多为病例报告;因此,尽管全切似乎是最佳治疗选择,但对于该肿瘤的最有效治疗方法尚无共识。
我们报告一名17岁女孩,患有中级脑膜黑素细胞瘤,累及C6神经根并压迫脊髓,导致进行性四肢轻瘫。临床和影像学检查提示硬膜内髓外实性肿块的可能性。通过颈椎板切开术部分切除肿瘤,随后大多数神经功能缺损迅速改善。该肿瘤不同寻常,因其在T1加权图像上具有非常高信号的均匀信号,侵犯蛛网膜并延伸至神经孔。脊髓表面的黑点在冰冻切片提示黑素细胞瘤之前被认为代表有组织的血凝块。
我们讨论了脑膜黑素细胞瘤与其他软脑膜黑素细胞肿瘤的鉴别。
当在T1加权图像上发现软脑膜高强度病变或在手术视野中发现类似木炭或有组织血肿的非常暗的肿块时,鉴别诊断应考虑黑素细胞肿瘤。最佳治疗方法是完整切除肿瘤,但对于次全切除和多发病变的病例可保留放疗。肿瘤的局部侵袭性和复发可能性需要定期随访。