Rosai - Dorfman Disease Masquerading As Sarcoma : A Case Report

  • Sudha P Meena Department of Pathology, Government Medical College, Kota(India)
  • Priyanka Patangia Department of Pathology, AIIMS, Bhopal(India)
  • Naresh N. Rai Department of Pathology, Government Medical College, Kota(India)
Keywords: Rosai-Dorfman disease, Sinus histiocytosis with massive lymphadenopathy, Sarcoma, Immunohistochemistry


Rosai Dorfman disease known as sinus histiocytosis with massive lymphadenopathy is an uncommon benign systemic histo-proliferative disease which affects the cervical lymph nodes commonly. The characteristic histological feature is lymphophagocytosis (emperipolesis) which is the hallmark feature. Here, we report a case of 35‑year‑old Indian female  presenting as a mass on medial aspect of thigh appearing as soft tissue mass, which on cytological examination was misdiagnosed as malignant mesenchymal neoplasm but histopathological examination and immunohistochemistry confirmed the diagnosis. According to the literature reviewed  no case of Rosai Dorfman disease with only ingunal lymphadenopathy  was reported till date which was seen in present case. RDD is a rare inflammatory non neoplastic process that should be considered in the differential diagnosis of  lympadenopathy and soft tissue tumor such as soft tissue sarcoma. The diagnosis  of RDD is made on the basis of clinical suspicion and is confirmed by histopathology and immunohistochemistry.DOI:10.21276/APALM.1464


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Case Report