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  • Grand Rounds

    A patient with a history of asthma and chronic sinusitis presented with unilateral facial swelling for three years. The facial swelling had gradually worsened over time. On examination, a well demarcated, yellow lesion was present in the right eyelid and cervical lymphadenopathy was present on the right side. An MRI was ordered. On post-contrast T1 fat suppression MRI, a hyperintense lesion was present within the periocular soft tissue on the right involving the lacrimal gland, tracking posteriorly along the lateral orbital wall in the extraconal space. Histopathologic examination of the mass disclosed an inflammatory infiltrate rich in foamy histiocytes with admixed scattered small lymphocytes within skeletal muscle and fibroadipose tissue. A perilesional lymphoid aggregate including follicles was also present. On immunohistochemistry, the histiocytes were positive for CD68 and ALK, and negative for BRAF. The lymphocytic infiltrate was composed of an admixture of CD20 and CD3 positive small lymphocytes; the stains for IgG and IgG4 did not show an increase in IgG4 plasma cells. In situ hybridization for kappa and lambda showed mild kappa predominance but overall polytypic expression of light chains among infrequent plasma cells. These histopathological findings were consistent with a xanthogranuloma. A xanthogranuloma in the context of an adult patient with asthma, chronic sinusitis and lymphadenopathy is consistent with a diagnosis of adult-onset asthma with periocular xanthogranuloma (AAPOX). Adult-onset xanthogranulomas are associated with a number of systemic conditions including IgG4 disease, ALK-positive histiocytosis, and Erdheim Chester disease. Systemic workup including serum IgG4 testing, and molecular genetic testing is indicated to assess for these systemic diseases.

    Presentation Date: 05/23/2024
    Issue Date: 06/07/2024