Institute of Neurosurgery, Catholic University School of Medicine, Rome, Italy.
BMC Cancer. 2012 Jun 6;12:220. doi: 10.1186/1471-2407-12-220.
Melanocytic meningeal tumours are rare extra-axial neoplasms of the nervous system, with only three reported cases in the cavernous sinus. Herein we describe for the first time the association of ocular melanosis and multiple intracranial melanocytic meningeal tumours, with the presenting lesion being in the cavernous sinus. The importance of this association is discussed together with the diagnostic and therapeutic challenges of the case.
A 20-year-old man presented with a left sixth cranial nerve deficit; general examination documented only congenital melanosis of the homolateral eye. MRI examination showed a space occupying lesion in the left cavernous sinus, which was followed conservatively for 2 years, until a new space occupying lesion was evident at the level of the right frontal convexity: both lesions presented with neuroradiological characteristics suggestive of melanin content.The frontal convexity lesion was removed: intraoperatively the dura was markedly and diffusely melanotic. Histological examination documented a melanocytic meningeal tumour, with a proliferative index of 3 %. The patient underwent 3D-Conformal Radiation Therapy on the lesion of the cavernous sinus (total dose 5040 cGy), with initial tumour reduction. Three years later, due to a symptomatic growth, he underwent partial removal of the lesion in the cavernous sinus. Histological examination was unchanged. He then received adjuvant Temozolomide with Low Dose Fractionated Radiation Therapy (LD-FRT). Due to further disease progression cisplatin plus fotemustine were administered, concomitant with LD-FRT: after two cycles MRI documented significant disease regression. After a period of apparent disease control, the patient presented with persistent cough and evidence of multiple thoracic metastases, which lead to his death, seven years after presentation.
Intracranial melanocytic meningeal tumours are challenging lesions, both from a diagnostic and therapeutic point of view; though rare, the possible association with ocular melanosis should be recognized and might facilitate an early diagnosis. Surgery remains the best possible option when feasible. In the event of partial resection, this "benign" disease might be clinically aggressive.
黑色素细胞脑膜瘤是一种罕见的神经系统外轴肿瘤，仅在海绵窦中有 3 例报道。在此，我们首次描述了眼部黑色素沉着与多个颅内黑色素细胞脑膜瘤的相关性，其首发病变位于海绵窦。讨论了这种相关性的重要性，并讨论了该病例的诊断和治疗挑战。
一名 20 岁男性出现左侧第 6 颅神经功能障碍；全身检查仅发现同侧眼睛的先天性黑色素沉着。MRI 检查显示左侧海绵窦占位性病变，该病变保守治疗 2 年，直到右侧额凸出现新的占位性病变：两个病变均具有提示黑色素含量的神经影像学特征。额凸病变被切除：术中硬脑膜明显弥漫性黑色素沉着。组织学检查证实为黑色素细胞瘤，增殖指数为 3%。患者接受了 3D 适形放射治疗（总剂量 5040 cGy），病变体积有所缩小。3 年后，由于症状性生长，他接受了海绵窦病变的部分切除。组织学检查无变化。随后，他接受了替莫唑胺联合低剂量分割放疗（LD-FRT）辅助治疗。由于进一步疾病进展，给予顺铂联合福莫司汀联合 LD-FRT：两个周期后 MRI 显示肿瘤明显缩小。在一段时间的疾病控制后，患者出现持续性咳嗽和多个胸部转移的证据，导致他在发病后 7 年死亡。
颅内黑色素细胞脑膜瘤无论是从诊断还是治疗的角度来看都是具有挑战性的病变；尽管罕见，但应认识到与眼部黑色素沉着的可能相关性，并可能有助于早期诊断。只要可行，手术仍是最佳选择。在部分切除的情况下，这种“良性”疾病可能具有临床侵袭性。
