Rosai-Dorfman disease with nodal and extranodal involvement: Case report
DOI:
https://doi.org/10.62059/4r6wrk04Keywords:
emperipolesis, histiocytosis, lymphadenopathy, extranodalAbstract
Rosai-Dorfman disease, or sinusoidal histiocytosis with massive lymphadenopathy, is a rare disorder (1/200,000 cases) of unknown etiology, associated with autoimmune, infectious, or neoplastic processes. Its clinical course is variable, ranging from self-limited forms to disseminated disease with higher mortality. Extranodal involvement occurs in approximately 43% of cases, with multifocal visceral and salivary gland involvement being infrequent and considered to have a worse prognosis. Diagnosis is established through histopathological and immunohistochemical studies. We present the case of a male patient with lymphadenopathy and extranodal involvement of salivary glands, initially diagnosed with myoepithelial carcinoma and unresponsive to chemotherapy. Histopathological review revealed histiocyte proliferation with emperipolesis and an immunophenotype compatible with Rosai-Dorfman disease. Treatment with the CHOP regimen was initiated, with a partial response and subsequent tomographic stability under maintenance therapy, highlighting the importance of diagnostic re-evaluation in atypical presentations that mimic malignant neoplasms.References
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Copyright (c) 2026 Dra. Karla Viera Vargas, Dr. Freddy Nicolalde Pozo, Dr. Luis Ayala Robles (Autor/a)
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