Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman Disease): A case report and review of 49 cases with fine needle aspiration cytology

Abstract

Rosai–Dorfman disease (RDD), a rare, benign, self-limiting histiocytic proliferative disorder, can be encountered in both nodal and extranodal locations, and fine needle aspiration (FNA), a simple, accurate and economic tool, has been widely used for the diagnosis of superficial and deep-seated lesions. Familiarity with the cytomorphologic features of RDD is important as prognosis and treatment are quite different from other benign or malignant diseases for which it may clinically masquerade. Although large numbers of RDD cases have been reported, review of the literature has revealed 49 reported cases of RDD diagnosed by FNA. Here, we report a case of RDD with nasal and sinus involvement. The patient was seen at our institution, carrying a diagnosis of inflammatory pseudotumor rendered by an outside institution, based on material obtained by nasal and sinus surgical biopsies. Cervical lymph node FNA performed at our institution revealed typical features of RDD. The case, as well as a brief review of the literature and 49 RDD cases with FNA cytology, will be discussed.

Cytological diagnosis of Rosai-Dorfman disease: A case report and revision of the literature

“…As in histology, cytology from FNA biopsy is usually highly cellular, with numerous histiocytes with vesicular nucleus and abundant clear cytoplasm with fine vacuoles and lymphophagocytosis in a reactive background of lymphocytes, plasma cells and occasionally neutrophils (5,10,23). Lymphatic sinuses are occupied by numerous lymphocytes and histiocytes with vesicular nucleus and abundant clear cytoplasm with phagocytized lymphocytes, neutrophils or plasma cells, also known as ‘emperopolesis’ (5,6).…”

“…Extranodal disease has a particular predilection for the head and neck region (75% of cases) (4). Involvement of ≥1 extranodal site has been identified in 43% of cases and only 23% have extranodal disease exclusively (1,5). Documented sites of extranodal involvement include skin, respiratory tract, bone, genitourinary system, oral cavity, central nervous system, eyes and ocular adnexa, salivary gland, tonsil, breast, soft tissue and heart (6–9).…”

“…Lymphatic sinuses are occupied by numerous lymphocytes and histiocytes with vesicular nucleus and abundant clear cytoplasm with phagocytized lymphocytes or plasma cells, also known as ‘emperipolesis’ (5,6,21). …”

“…The cytological characteristics of SHML are highly distinctive. Consequently, fine needle aspiration (FNA) biopsy may be sufficient to make the diagnosis in most cases thus preventing unnecessary invasive procedures (5,6,21,22). …”

Extranodal manifestation of Rosai-Dorfman disease in the breast tissue

“…Pathologically, these histiocytes harbor what are presumably engulfed, intact lymphocytes lending the appearance of “cell within a cell” or emperipolesis [2]. Identification of cell surface S-100 and CD68 receptors and the lack of CD1a expression are the cardinal histologic characteristics of this cell type [1].…”