Abstract

Langerhans cell histiocytosis (LCH) and adult-onset xanthogranuloma (AXG) are rare disorders characterized by the accumulation of macrophage, dendritic cells, or monocyte-derived cells in various tissues of the body. Many researchers now consider LCH a form of malignancy, but this classification remains controversial. As per our knowledge, there are only 36 cases of AXG reported so far in the English literature. Here, we report a case of AXG and single-system LCH found in the oral cavity and cervical lymph nodes, respectively. In this article, we intend to define a clear understanding of some classic clinical, radiological, and histopathological findings of LCH and AXG, to differentiate them from oral malignancies. The primary goal of this article is to increase awareness regarding conditions that closely resemble malignancies and to save patients from the burden of extensive treatment under the presumption of malignant disorders. In the medical field, reporting of rare cases is highly encouraged; however, proper treatment for the patient depends on the accurate diagnosis that, in this case, was made postoperatively, which only added more physical and mental distress for the patient and their family.

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