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J Cytol. 2020 Jan-Mar;37(1):46-52. doi: 10.4103/JOC.JOC_4_19. Epub 2019 Dec 23.

Cytological Diagnosis of Rosai-Dorfman Disease: A Study of Twelve Cases with Emphasis on Diagnostic Challenges.

Author information

1
Department of Pathology, Rangaraya Medical College, Kakinada, Andhra Pradesh, India.
2
Department of Pathology, Crest Labs, Vijayawada, Andhra Pradesh, India.
3
Department of Pathology, Cpath labs, Hyderabad, Telangana, India.
4
Department of Pediatric Surgery, Rangaraya Medical College, Kakinada, Andhra Pradesh, India.

Abstract

Context:

Rosai-Dorfman disease, also known as Sinus histiocytosis with massive lymphadenopathy, is a benign proliferative disorder of histiocytes. It typically affects lymph nodes; however, extranodal disease is being increasingly reported. The latter entity poses exceptional diagnostic challenge clinico-radiologically by forming mass lesions. Fine needle aspiration cytology (FNAC) is the investigation of choice to avoid unnecessary surgery as the majority are self-limiting.

Aims:

The objective is to assess the utility of FNAC in the diagnosis of Rosai-Dorfman disease and to highlight the diagnostic difficulties.

Material and Methods:

The cytology features of 12 cases of Rosai-Dorfman disease were analyzed and correlated with histopathology and immunohistochemistry.

Results:

The present study included six nodal and six extranodal Rosai-Dorfman disease. The cytology smears showed a variable number of large histiocytes exhibiting characteristic emperipolesis. Ten cases diagnosed as Rosai-Dorfman disease on cytology were confirmed on histopathology. The presence of granulomas, atypical histiocytes, insignificant emperipolesis, and eosinophil infiltration were the challenges we faced.

Conclusions:

FNAC, a simple and cost-effective method with its unique cytology features is the first line of investigation in the diagnosis of Rosai-Dorfman disease.

KEYWORDS:

Emperipolesis; Rosai–Dorfman Disease; Sinus Histiocytosis with massive lymphadenopathy; eosinophils; granulomas

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