Background: Rosai-Dorfman disease (RDD) is a rare benign idiopathic histiocytic proliferation. Most commonly reported cases are lymph nodal. Only 30-40% of cases occur in extranodal sites. Although the morphological features of RDD are well documented, there is limited information about the morphometric variations in the histiocytes of this entity. Method: Twenty-two cases of RDD diagnosed on fine-needle aspiration cytology (FNAC) were retrieved from the archives. Both Papanicolaou- and May-Grünwald-Giemsa-stained slides were available for evaluation in all cases. Nuclear area, diameter and histiocyte size were measured taking reactive histiocytes as controls. Results: Among the 22 patients (male/female ratio 3:2; age range 5-55 years, mean 26 years), 3 cases were extranodal and 19 cases were nodal. The nodal sites included cervical, axillary, inguinal and submandibular lymph nodes. The extranodal sites were the retroperitoneum, mediastinum and skin. The most common clinical presentation was enlarged lymph nodes. Cytological features included numerous large benign histiocytes with emperipolesis. All the morphometric parameters were significantly (p < 0.01) higher in RDD histiocytes than in histiocytes in the reactive lymph node. Conclusions: In view of the rarity of the disease and the variable clinical presentation in RDD, accurate diagnosis is necessary. This is the first study to document the morphometric parameters of RDD histiocytes and their comparison with histiocytes in the reactive lymph node.

1.
Rosai J, Dorfman RF: Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol 1969;87:63-70.
2.
Sanchez R, Rosai J, Dorfman R: SHML: an analysis of 113 cases with special emphasis on its extranodal manifestations. Lab Invest 1977;36:349-350.
3.
Foucar E, Rosai J, Dorfman R: Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol 1990;7:19-73.
4.
Goel MM, Agarwal PK, Agarwal S: Primary Rosai-Dorfman disease of bone without lymphadenopathy diagnosed by fine needle aspiration cytology. A case report. Acta Cytol 2003;47:1119-1122.
5.
Lauwers GY, Perez-Atayde A, Dorfman RF, Rosai J: The digestive system manifestations of Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy): review of 11 cases. Hum Pathol 2000;31:380-385.
6.
Ji H, Zhang B, Tian D, et al: Rosai-Dorfman disease of the lung. Respir Care 2012;57:1679-1681.
7.
Sundaram C: Rosai-Dorfman disease and neurological manifestations. Neurol India 2011;59:329-330.
8.
Nayler SJ, Cooper K: Inflammatory pseudotumour and Rosai-Dorfman disease of soft tissue. J Clin Pathol 1997;50:620-621.
9.
Naidu RK, Urken ML, Som PM, et al: Extranodal head and neck sinus histiocytosis with massive lymphadenopathy. Otolaryngol Head Neck Surg 1990;102:764-767.
10.
Pulsoni A, Anghel G, Falcucci P, et al: Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): report of a case and literature review. Am J Hematol 2002;69:67-71.
11.
Payne JF, Srivastava SK, Wells JR, Grossniklaus HE: Rosai-Dorfman disease simulating nodular scleritis and panuveitis. Arch Ophthalmol 2011;129:518-520.
12.
Malur PR, Bannur HB, Kodkany SB: Orbital Rosai-Dorfman disease: report of a case with fine needle aspiration cytology and histopathology. Acta Cytol 2007;51:581-582.
13.
Avelino MA, Elias TG, Rezende RM, et al: Rosai-Dorfman disease as a differential diagnosis of nasosinusal polyposis in children. Braz J Otorhinolaryngol 2012;78:136.
14.
Güven G, Ilgan S, Altun C, Gerek M, Gunhan O: Rosai Dorfman disease of the parotid and submandibular glands: salivary gland scintigraphy and oral findings in two siblings. Dentomaxillofac Radiol 2007;36 :428-433.
15.
Illing EA, Halum SL: Rosai-Dorfman disease with isolated laryngeal involvement. Ear Nose Throat J 2012;91:439-440.
16.
Chhabra S, Agarwal R, Garg S, Singh H, Singh S: Rosai-Dorfman disease: A case report with extranodal thyroid involvement. Diagn Cytopathol 2012;40:447-449.
17.
Ji H, Zhang B, Tian D, et al: Rosai-dorfman disease of the lung. Respir Care 2012;57:1679-1681.
18.
Yontz L, Franco A, Sharma S, Lewis K, McDonough C: A case of Rosai-Dorfman disease in a pediatric patient with cardiac involvement. J Radiol Case Rep 2012;6:1-8.
19.
Gwin K, Cipriani N, Zhang X, Schmidt R, Hyjek E: Bilateral breast involvement by disseminated extranodal Rosai-Dorfman disease. Breast J 2011;17:309-311.
20.
Long E, Lassalle S, Cheikh-Rouhou R, et al: Intestinal occlusion caused by Rosai-Dorfman disease mimicking colonic diverticulitis. Pathol Res Pract 2007;203:233-237.
21.
Romero Arenas MA, Singhi AD, Hruban RH, Cameron AM: Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) of the pancreas: third reported occurrence. J Gastrointest Cancer 2012;43:626-629.
22.
Tabata H, Hisasue S, Tsukamoto T: Extranodal Rosai-Dorfman disease of the kidney and bone. Int J Urol 2012;19:486-487.
23.
Fernandopulle SM, Hwang JS, Kuick CH, et al: Rosai-Dorfman disease of the testis: an unusual entity that mimics testicular malignancy. J Clin Pathol 2006;59:325-327.
24.
Castioni J, Mihaescu A, So AK: Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and oligoarthritis. Joint Bone Spine 2011;78:641-643.
25.
Singh NG, Kapila K, Mathur S, Ray R, Verma K: Rosai-Dorfman disease manifesting as multiple subcutaneous nodules. Report of a case with diagnosis on a fine needle aspirate. Acta Cytol 2004;48:215-218.
26.
Chow CP, Ho HK, Chan GC, Cheung AN, Ha SY: Congenital Rosai-Dorfman disease presenting with anemia, thrombocytopenia, and hepatomegaly. Pediatr Blood Cancer 2009;52:415-417.
27.
Kaur PP, Birbe RC, DeHoratius RJ: Rosai-Dorfman disease in a patient with systemic lupus erythematosus. J Rheumatol 2005;32:951-953.
28.
Agarwal A, Pathak S, Gujral S: Sinus histiocytosis with massive lymphadenopathy - a review of seven cases. Indian J Pathol Microbiol 2006;49:509-515.
29.
Levine PH, Jahan N, Murari P, Manak M, Jaffe ES: Detection of human herpes virus 6 in tissues involved by sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). J Infect Dis 1992;16:291-295.
30.
Mehraein Y, Wagner M, Remberger K, et al: Parvovirus B19 detected in Rosai-Dorfman disease in nodal and extranodal manifestations. J Clin Pathol 2006;59:1320-1326.
31.
Al-Daraji W, Anandan A, Klassen-Fischer M, et al: Soft tissue Rosai-Dorfman disease: 29 new lesions in 18 patients, with detection of polyomavirus antigen in 3 abdominal cases. Ann Diagn Pathol 2010;14:309-316.
32.
Lampert F, Lennert K: Sinus histiocytosis with massive lymphadenopathy: fifteen new cases. Cancer 1976;37:783-789.
33.
Wen CH, Huang MS, Wang SL, Su YC, Yang SF, Chai CY: Diagnostic value of computerized nuclear morphometry for the prediction of malignancy in liver fine needle aspiration cytology. Acta Cytol 2009;53:77-82.
34.
Pambuccian SE, Becker RL Jr, Ali SZ, Savik K, Rosenthal DL: Differential diagnosis of Hürthle cell neoplasms on fine needle aspirates. Can we do any better with morphometry? Acta Cytol 1997;41:197-208.
35.
Marchevsky AM, Hauptman E, Gil J, Watson C: Computerized interactive morphometry as an aid in the diagnosis of pleural effusions. Acta Cytol 1987;31:131-136.
36.
Hughes-Jones NC, Wickramasinghe SN, Hatton SR: Normal haemoglobin, blood cells and haemopoiesis; in: Lecture Notes: Haematology, ed 8. Oxford, Wiley- Blackwell, 2009, pp 1-15.
37.
Krombach F, Münzing S, Allmeling AM, Gerlach JT, Behr J, Dörger M: Cell size of alveolar macrophages: an interspecies comparison. Environ Health Perspect 1997:105(suppl 5): 1261-1263.
38.
Kram A, Peychewa M, Bachurska S, Domagala W: Morphometric distinction of signet-ring cell adenocarcinoma cells from foamy macrophages in gastric endoscopic biopsies. Pol J Pathol 2011;62:145-147.
39.
Warnke RA, Weiss LM, Chan JK, Cleary M, Dorfman RM: Chronic histiocytic and dendritic cell proliferation; in: Tumors of Lymph Nodes and Spleen. Washington, Armed Forces Institute of Pathology, 1994, pp 358-360.
40.
Iyer VK, Handa KK, Sharma MC: Variable extent of emperipolesis in the evolution of Rosai Dorfman disease: diagnostic and pathogenetic implications. J Cytol 2009;26:111-116.
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