An uncommon benign histiocytic illness, Rosai-Dorfman disease (RDD) mostly affects lymph nodes but can also manifest as extranodal involvement. We describe a case of a female patient, sixty years of age, who had joint discomfort, sleeplessness, weight loss, and headache and eye problems. A heterogeneously hypodense perirectal mass was seen on imaging. Other histiocytic diseases, metastatic cancer, and lymphoma were among the differential diagnosis. Through biopsy and immunohistochemistry staining, which revealed S-100 and CD68 positivity with CD1a negative, a definitive diagnosis of RDD was made. Over a 2-year period following surgical excision, the patient's symptoms significantly improved and there was no sign of recurrence. This example emphasizes the significance of taking RDD into account when making a differential diagnosis for perirectal tumors and the function of imaging in conjunction with histological testing in guiding management.
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Luther B. Adair (2024) studied this question.
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