Head and Neck Sinus Histiocytosis with Massive Lymphadenopathy Radiology–Pathology Correlation

Abstract

Sinus histiocytosis with massive lymphadenopathy, or Rosai-Dorfman disease, is a rare, benign type of non-Langerhans cell histiocytosis. The radiological findings are often nonspecific, potentially mimicking malignancies. The diagnosis is ultimately made based on pathology, in which the lymph nodes are characterized by a dilated subcapsular sinus filled with histiocytes that can exhibit emperipolesis. Immunohistochemically, the histiocytes are variably CD68 positive and reliably negative for CD1a. The features of head and neck sinus histiocytosis with massive lymphadenopathy are exemplified in this radiology-pathology correlation sine qua non article.

A Report of Rosai–Dorfman Disease in an Adolescent

“…Perhaps the most important differential diagnosis of RDD is that of Langerhans cell histiocytosis (LCH) as both are S100 positive. In RDD, the CD1a stain is negative [ 10 ]. Also, the abundant eosinophils in LCH make it morphologically different from RDD [ 19 ].…”

“…The aetiology of RDD is enigmatic; however, RAF/MEK/ERK pathway mutations have been implicated, suggesting that the disease may be clonal in some cases [ 10 ]. Previous studies have suggested that viruses such as HHV, HIV, EBV, and CMV and bacteria including Brucella and Klebsiella spp.…”

“…Radiological findings of RDD are non-specific, resembling lymphoma. Diagnosis is based on clinical manifestation and ultimately on histopathological examination [ 10 ]. Classically, histiocytes in intranodal Rosai–Dorfman disease engulf small lymphocytes, plasma cells, and erythrocytes without phagocytosis.…”