Shooshani Tara, Han Michael, Tao Jeremiah P, Spiegel Samuel J, Estopinal Maria Del Valle
School of Medicine, University of California, Irvine, CA 92697, USA.
Department of Ophthalmology, University of California, Irvine, CA 92697, USA.
Diagnostics (Basel). 2025 Mar 20;15(6):780. doi: 10.3390/diagnostics15060780.
Neurolymphomatosis (NL) is a rare manifestation of hematologic malignancies, characterized by a neoplastic infiltration of the peripheral nervous system and cranial nerves (CNs). Non-Hodgkin lymphomas (NHLs) account for 90% of NL cases, while acute leukemia represents 10% of the cases. NL can occur as the first manifestation of a malignancy (primary), or as a relapse or progression of a previously treated disease (secondary). Herein, we report a unique case of NL involving the left orbit and CNs in a 74-year-old female with primary central nervous system (CNS) diffuse large B-cell lymphoma (DLBCL). Our patient developed secondary neurolymphomatosis involving the left orbit and CNs II, III, V, and VI, supported by clinical, radiologic, and histologic findings. The lacrimal gland enhancement was histopathologically proven to be caused by the direct spread of CNS DLBCL to the lacrimal nerve, a branch of CN V, identifying NL as one of the conditions that can affect this organ. The lacrimal gland could be considered as a more accessible biopsy site when the involvement of CN V is suspected.
神经淋巴瘤（NL）是血液系统恶性肿瘤的一种罕见表现形式，其特征为外周神经系统和颅神经（CNs）的肿瘤浸润。非霍奇金淋巴瘤（NHLs）占NL病例的90%，而急性白血病占病例的10%。NL可作为恶性肿瘤的首发表现（原发性），或作为先前治疗疾病的复发或进展（继发性）出现。在此，我们报告一例独特的NL病例，该病例发生于一名74岁患有原发性中枢神经系统（CNS）弥漫性大B细胞淋巴瘤（DLBCL）的女性患者的左侧眼眶和颅神经。我们的患者出现了累及左侧眼眶及CNs II、III、V和VI的继发性神经淋巴瘤，临床、影像学和组织学检查结果均支持这一诊断。组织病理学证实泪腺强化是由CNS DLBCL直接蔓延至泪腺神经（CN V的一个分支）所致，这表明NL是可影响该器官的疾病之一。当怀疑有CN V受累时，泪腺可被视为一个更容易进行活检的部位。
