Mammary gland Rosai Dorfman disease. A case report and literature review

Rosai-Dorfman disease (RDD) is an uncommon idiopathic, benign histiocytic lesion. It generally involves the cervical lymph nodes and, less often the extranodal sites. Involvement of the breast is rare, with about 18 cases reported in the English literature to date. We describe a case of breast involvement by extranodal RDD. The patient was a 67 year old woman with a solid breast lesion that was detected during mammography screening. Microscopically, the lesion was well-circumscribed, and made of sheets of S-100 protein-positive large histiocytes displaying lymphocytophagocytosis. Because the clinical presentation and imaging characteristics of RDD frequently mimics invasive breast carcinoma, awareness and appropriate diagnosis of this entity is essential for proper treatment.     

Case report of Rosai–Dorfman disease

This case report discusses the presentation of Rosai–Dorfman disease in a 4 year old female patient, who presented with bilateral level V lymphadenopathy of the cervical neck. The histopathology demonstrated numerous histiocytes, with characteristic emperipolesis. No granulomas or necrosis were identified. Micro-organisms were not identified on special stains, nor on culture. The patient was under observation and spontaneous remission occurred after six months.     

Isolated intracranial Rosai Dorfman disease masquerading as meningioma: a case report

Isolated intracranial Rosai Dorfman disease (sinus histiocytosis with massive lymphadenopathy) is rare. We present a 26-year-old male who presented with left focal motor seizures becoming secondarily generalized of one-year duration. Clinically and radiologically patient was diagnosed to have a right parietal convexity meningioma. However on histopathological examination a final diagnosis of intracranial Rosai Dorfman disease was rendered.     

Extranodal Rosai–Dorfman disease involving the heart: report of two cases

Sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease) is a rare disease that typically occurs in lymph nodes. While many body sites have been reported to be involved in extranodal manifestations of the disease, the cardiovascular system has been largely absent in this literature. We report two cases of Rosai–Dorfman disease that involves the heart. Case 1 was a 40-year-old man with chronic myelomonocytic leukemia who was incidentally noted to have Rosai–Dorfman disease on autopsy after succumbing to respiratory failure in the setting of adult respiratory distress syndrome. Case 2 was a 57-year-old woman known to have Rosai–Dorfman disease involving mediastinal lymph nodes and found to have a right atrial mass on workup for atypical chest pain. Both cases showed a similar histologic picture of large, multinucleated histiocytes with immunoreactivity to S100, emperipolesis, and marked plamacytosis. This study expands our knowledge of organs involved in extranodal Rosai–Dorfman disease.     

Sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman disease) in an HIV-positive patient

Summary We report a case of a 31-year-old HIV infected black female, who presented with asymptomatic generalized lymphadenopathy. Three particularly enlarged lymph nodes were biopsied (2 cervical and 1 axillary). The histological picture was consistent with a diagnosis of sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease. Large histiocytes, positive for a variety of macrophage markers and for the S-100 protein, were observed in the distended sinuses. A few hyperplastic follicles, such as usually seen in HIV-infection-associated lymphadenopathy, were present at the periphery of one lymph node. No infections agent besides HIV could be detected by histological or microbiological analysis or by in situ hybridization. This is the first reported case of SHML associated with HIV infection. The possible relationship between the two diseases is discussed.