School of Nursing and Health Studies, Georgetown University, Washington, DC, USA.
Orbital Oncology & Ophthalmic Plastic Surgery, Department of Plastic Surgery, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.
Cancer Imaging. 2021 May 26;21(1):39. doi: 10.1186/s40644-021-00409-3.
Neurolymphomatosis involving the cranial nerves (CNs) is rare. We sought a better understanding of the primary disease sites and patterns of spread in neurolymphomatosis of the orbit and retro-orbital cranial nerves.
Patients with lymphoma and MRI evidence of neurolymphomatosis of CN II, III, IV, V, or V were retrospectively reviewed. Demographics and primary disease site and sites of neurolymphomatosis on MRI were recorded. Wilcoxon rank sum test was used to compare number of sites of neurolymphomatosis with lymphoma type and survival.
The study included 18 patients. The most frequent types of lymphoma were diffuse large B-cell (DLBCL) (n = 9) and marginal zone (n = 3). In 9 patients, lymphoma presented as a mass (n = 7) or infiltrative disease (n = 2) directly involving the orbit; in 6, a maxillofacial mass spread directly to CNs; and in 3, lymphoma at remote sites spread to orbital CNs. Overall, 81 sites of neurolymphomatosis were noted. The most common sites were the maxillary nerve (V) including at the infraorbital fissure or foramen rotundum (17 patients; 19 nerves), pterygopalatine fossa (16 patients; 19 nerves), and cavernous sinus (9 patients; 12 nerves). Number of sites of neurolymphomatosis was significantly lower for DLBCL than for other lymphoma types (p = 0.007). Number of sites of neurolymphomatosis did not affect survival (p = 0.26). The mean interval between the pathologic diagnosis and MRI documentation of the full extent of neurolymphomatosis was 39 days after pathologic diagnosis.
Based on our study results, neurolymphomatosis in the orbit appears to be frequently associated with an orbital and/or maxillofacial mass and commonly involves CN V, the pterygopalatine fossa, and the cavernous sinus. DLBCL may be associated with fewer sites of neurolymphomatosis than other lymphomas. In patients with lymphoma, a systematic search for neurolymphomatosis is imperative for early detection.
颅神经（CN）受累的神经淋巴瘤病罕见。我们旨在更好地了解眼眶和眶颅神经神经淋巴瘤病的原发疾病部位和播散模式。
回顾性分析了患有淋巴瘤且 MRI 显示 II、III、IV、V 或 V 颅神经神经淋巴瘤病的患者。记录患者的人口统计学资料、原发疾病部位以及 MRI 上的神经淋巴瘤病部位。采用 Wilcoxon 秩和检验比较神经淋巴瘤病的部位与淋巴瘤类型和生存之间的关系。
本研究共纳入 18 例患者。最常见的淋巴瘤类型为弥漫性大 B 细胞淋巴瘤（DLBCL）（n=9）和边缘区淋巴瘤（n=3）。9 例患者的淋巴瘤表现为肿块（n=7）或直接累及眼眶的浸润性疾病（n=2）；6 例患者的上颌骨肿块直接向 CN 扩散；3 例患者的远处部位的淋巴瘤向眶颅神经扩散。共发现 81 个神经淋巴瘤病部位。最常见的部位是上颌神经（V），包括眶下裂或圆孔（17 例患者；19 根神经）、翼腭窝（16 例患者；19 根神经）和海绵窦（9 例患者；12 根神经）。DLBCL 的神经淋巴瘤病部位明显少于其他淋巴瘤类型（p=0.007）。神经淋巴瘤病的部位数量不影响生存（p=0.26）。病理诊断后至 MRI 完全记录神经淋巴瘤病的平均时间间隔为 39 天。
根据我们的研究结果，眼眶神经淋巴瘤病似乎常与眼眶和/或上颌骨肿块相关，常累及 V 颅神经、翼腭窝和海绵窦。DLBCL 可能比其他淋巴瘤的神经淋巴瘤病部位更少。在患有淋巴瘤的患者中，必须进行系统性搜索以早期发现神经淋巴瘤病。
