An unusual case of necrobiotic xanthogranuloma with IgG-λ monoclonal gammopathy presenting as cerebral manifestations

[摘要] Necrobiotic xanthogranuloma (NXG) disease is a rare nonLangerhans cell tissue proliferative disease that was firstreported in 1980 (1). It is often associated with the abnormalproliferation of monoclonal gammopathy (M) protein.Among all cases with NXG, 50% of are immunoglobulinG (IgG)-κ type, the rarer IgG-λ type accounts for about21%, and the remaining 12% consists of IgA, IgG,polyclonal, and unspecified types, with 17% being withoutparaproteinemia (2). Studies have shown that paraproteinsplay an essential role in granulation tissue formation (3).The disease often occurs in women over 60 years old, andthe female-to-male ratio is about 1.7:2.7 (2,4). Nelsonet al. (2) proposed the diagnostic criteria of NXG basedon primary and secondary clinical and histopathologicalfindings. The primary conditions include (I) yellow ororange papulonodular plaques on the skin that are (II)consistent with histopathological features demonstratingpalisading granulomas with lymphoplasmacytic infiltrate andzones of necrobiosis. The secondary conditions include (I)paraproteinemia, plasmacytoid disease, and/or other relatedlymphoproliferative disorders; and (II) distribution in theperiorbital skin. After exclusion of a foreign body, infection,or other determinable diseases, the diagnosis is establishedby meeting the primary conditions and at least 1 secondarycondition. NXG most commonly involves the skin (5),NXG involvement of the nervous system is especially rare.Herein, we report a case of NXG of the meninges, brainparenchyma, and spleen. We also summarize its clinicalcharacteristics, imaging manifestations, histopathology, andtherapeutic methods.

[学科分类] 外科医学

[关键词] Necrobiotic xanthogranuloma (NXG);immunoglobulin G-λ (IgG-λ);cerebral;meninges;magnetic resonance imaging (MRI)