Is it a Metastatic Disease: A Case Report and New Understanding of Rosai–Dorfman Disease?

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Abstract

Rosai–Dorfman disease (RDD) is a rare histiocytic proliferative disorder, whose etiology remains unclear. Most patients experience a limited clinical course followed by recovery; however, a small subset of patients show persistence over years, with death occurring in a few cases because of multiorgan involvement. About 40% of patients have extranodal manifestations, with skin and meninges being the main extranodal sites. Cutaneous involvement occurs in approximately 10% of cases; however, RDD seldom affects the skin alone. In this study, the authors report the case of a 58-year-old woman who first presented with a cutaneous mass on the left inner thigh, then on the right wrist, and on the right chest area for the third time. She was misdiagnosed with nonspecific inflammatory disease at the first and second subcutaneous lesions. After the third excision surgery, the patient was diagnosed with RDD based on the hematoxylin and eosin stain and immunohistochemical staining results. The authors retrospectively reviewed the tissue slides from the previous 2 surgeries; interestingly, all the 3 tissue specimens demonstrated similar morphological features. Some RDD cells were observed in the walls of blood vessels in the tissue, with some invasion in the lumen of the vessels; therefore, the authors inferred that the second and third occurrences of RDD in locations different from the first-time lesion were caused by the vascular invasion of RDD cells from the first-time lesion.

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