PDF(Pubmed)
Abstract：
BACKGROUND: Primary meningeal melanocytic neoplasms are exceedingly rare tumors, representing only 0.06% to 0.1% of all primary brain tumors and ranging in spectrum from benign localized tumors to highly aggressive malignant lesions. The diagnosis of these tumors is often challenging from clinical, radiological, and pathologic standpoints. Equally challenging is the distinction between primary meningeal melanocytic neoplasm and metastatic melanoma.
METHODS: The authors reported the case of a 41-year-old man with imaging findings diagnostic of neurofibromatosis type 2: bilateral internal auditory canal lesions (most consistent with bilateral vestibular schwannomas), two dura-based lesions presumed to be meningiomas, multiple spinal lesions consistent with peripheral nerve sheath tumors, and one intramedullary spinal lesion consistent with an ependymoma. Biopsy of these lesions revealed melanocytic neoplasms with mild to moderate atypia and a mildly elevated proliferation index, which made the distinction between benign and malignant challenging. In addition, the disseminated nature of these tumors made it difficult to determinate whether they arose from the meninges or represented metastases from an occult primary melanoma.
CONCLUSIONS: This case illustrated the challenges presented by the diagnosis of meningeal melanocytic neoplasms and highlighted the importance of integrating the clinical and radiographic findings with histologic appearance and molecular studies.
METHODS: The authors reported the case of a 41-year-old man with imaging findings diagnostic of neurofibromatosis type 2: bilateral internal auditory canal lesions (most consistent with bilateral vestibular schwannomas), two dura-based lesions presumed to be meningiomas, multiple spinal lesions consistent with peripheral nerve sheath tumors, and one intramedullary spinal lesion consistent with an ependymoma. Biopsy of these lesions revealed melanocytic neoplasms with mild to moderate atypia and a mildly elevated proliferation index, which made the distinction between benign and malignant challenging. In addition, the disseminated nature of these tumors made it difficult to determinate whether they arose from the meninges or represented metastases from an occult primary melanoma.
CONCLUSIONS: This case illustrated the challenges presented by the diagnosis of meningeal melanocytic neoplasms and highlighted the importance of integrating the clinical and radiographic findings with histologic appearance and molecular studies.
摘要：
背景：原发性脑膜黑素细胞肿瘤是非常罕见的肿瘤，仅占所有原发性脑肿瘤的0.06％至0.1％，范围从良性局部肿瘤到高度侵袭性恶性病变。这些肿瘤的诊断往往是具有挑战性的从临床，放射学,和病理观点。同样具有挑战性的是原发性脑膜黑素细胞肿瘤和转移性黑色素瘤之间的区别。
方法：作者报道了一例41岁男性，其影像学表现诊断为2型神经纤维瘤病：双侧内耳道病变（最符合双侧前庭神经鞘瘤），两个基于硬脑膜的病变推测为脑膜瘤，与周围神经鞘瘤一致的多发性脊柱病变，和一个与室管膜瘤一致的脊髓髓内病变。这些病变的活检显示黑素细胞肿瘤具有轻度至中度的异型性和轻度升高的增殖指数。这使得区分良性和恶性具有挑战性。此外,这些肿瘤的播散性使得很难确定它们是由脑膜引起还是由隐匿性原发性黑色素瘤引起的转移。
结论：该病例说明了脑膜黑素细胞肿瘤的诊断所面临的挑战，并强调了将临床和影像学发现与组织学外观和分子研究相结合的重要性。
方法：作者报道了一例41岁男性，其影像学表现诊断为2型神经纤维瘤病：双侧内耳道病变（最符合双侧前庭神经鞘瘤），两个基于硬脑膜的病变推测为脑膜瘤，与周围神经鞘瘤一致的多发性脊柱病变，和一个与室管膜瘤一致的脊髓髓内病变。这些病变的活检显示黑素细胞肿瘤具有轻度至中度的异型性和轻度升高的增殖指数。这使得区分良性和恶性具有挑战性。此外,这些肿瘤的播散性使得很难确定它们是由脑膜引起还是由隐匿性原发性黑色素瘤引起的转移。
结论：该病例说明了脑膜黑素细胞肿瘤的诊断所面临的挑战，并强调了将临床和影像学发现与组织学外观和分子研究相结合的重要性。
