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.

Keywords: Fine needle aspiration; Rosai-Dorfman disease; Sinus histiocytosis with massive lymphadenopathy.

Figure 1

Emperipolesis, RDD. FNA cervical lymph node. Emperipolesis in multinucleated giant cells (arrows) LP (a). Lymphocytes (arrow A) and plasma cells (arrow B) HP (b). Neutrophils and a few plasma cells (c) HP. Lymphocytes, neutophils, plasma cells and associated macrophages (d), HP. Lymphocytes and nuclear debris (e) high power. Nasal biopsy with S-100 positive cells, insert cytospin specimen with S-100 positive multinucleated giant cells. a–c) Diff Quik®; d-e) Papanicolaou stain; f) immunoperoxidase stain with DAB

Figure 2

Emperipolesis in other disorders. Acute and chronic cervicitis. Endocervical cells with indigested neutrophils, cervical smear (a). Endometrial adenocarcinoma tissue fragment with intracytoplasmic lymphocytes and neutophils, cervicovaginal smear (b). Intracytoplasmic lymphocytes, cytomegalovirus (CMV) infection, cervical biopsy (c). Ingested erythrocytes metaplastic endocervical cells, HSV, cervical smear (d). Ingested lymphocytes and debris, lymphoma cerebrospinal fluid (e). Ingested lymphocytes, B cell lymphoma, cervical lymph node FNA (f). (a, b, d, e, f) Papanicolaou; (c) H/E stain