J Korean Med Sci.  2009 Aug;24(4):760-762. 10.3346/jkms.2009.24.4.760.

Sinus Histiocytosis with Massive Lymphadenopathy: A Case Report with Pleural Effusion and Cervical Lymphadenopathy

• Ju J
• Kwon YS
• Jo KJ
• Chae DR
• Lim JH
• Ban HJ
• Chi SY
• Oh IJ
• Kim KS
• Kim YI
• Kim YC
• Lim SC

• KMID: 1779218
• DOI: http://doi.org/10.3346/jkms.2009.24.4.760

Abstract

Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare disorder characterized by a nonneoplastic proliferation of distinctive histiocyte cells within lymph node sinuses and lymphatics in extranodal sites. SHML occurs worldwide and is primarily a disease of childhood and early adulthood. A 26-yr-old man presented with painless palpable lymph node in cervical area. Radiographic studies revealed pleural effusion with lymphadenopathy and calcification in mediastinum. The cervical lymph node biopsy showed dilated sinuses filled with histiocytes with clear cytoplasm. The cells stained positive with CD68 and S-100. These cytologic and immunohistochemical findings were considered consistent with the diagnosis of SHML.

Keyword

Histiocytosis, Sinus; Massive Lymphadenopathy; Rosai-Dorfman Disease

MeSH Terms

Adult
Antigens, CD/metabolism
Antigens, Differentiation, Myelomonocytic/metabolism
Histiocytes/pathology
Histiocytosis, Sinus/*diagnosis/metabolism/pathology
Humans
Lymph Nodes/pathology
Male
Neck
Pleural Effusion/*radiography
S100 Proteins/metabolism
Tomography, X-Ray Computed

Figure

• 

  Fig. 1 Chest CT revealed pleural effusion and mediastinal lymphadenopathies.

• 

  Fig. 2 Excisional biopsy of the cervical lymph node revealed dilated sinuses filled with histiocytes with abundant pale eosinophilic cytoplasm (arrows) (H&E, ×400).

• 

  Fig. 3 Immunohistochemical staining for CD68 and S-100 protein. Histiocytes show positive immunoreactivity with brown color for CD 68 (A, ×400) and S-100 (B, ×400).

Reference

1. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy. A newly recognized benign clinicopathological entity. Arch Pathol. 1969. 87:63–70.

2. Tiju JW, Hsiao CH, Tsai TF. Cutaneous Rosai-Dorfman disease: remission with thalidomide treatment. Br J Dermatol. 2003. 148:1060–1061.

3. Pettinato G, Manivei JC, d'Amore ES, Petrella G. Fine needle aspiration cytology and immunocytochemical characterization of the histiocytes in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman syndrome). Acta Cytol. 1990. 34:771–777.

4. Chan JK, Tsang WY. Uncommon syndromes of reactive lymphadenopathy. Semin Oncol. 1993. 20:648–657.

5. Bonetti F, Chilosi M, Menestrina F, Scarpa A, Pelicci PG, Amorosi E, Fiore-Donati L, Knowles DM 2nd. Immunohistological analysis of Rosai-Dorfman histiocytosis. A disease of S-100+CD1-histiocytes. Virchows Arch A Pathol Anat Histopathol. 1987. 411:129–135.

6. Ahsan SF, Madgy DN, Poulik J. Otolaryngologic manifestations of Rosai-Dorfman disease. Int J Pediatr Otorhinolaryngol. 2001. 59:221–227.

7. Paulli M, Bergamaschi G, Tonon L, Viglio A, Rosso R, Facchetti F, Geerts ML, Magrini U, Cazzola M. Evidence for a polyclonal nature of the cell infiltrate in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Br J Haematol. 1995. 91:415–418.

8. El Kohen A, Planquart X, Al Hamany Z, Bienvenu L, Kzadri M, Herman D. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): two case reports. Int J Pediatr Otorhinolaryngol. 2001. 61:243–247.

9. Karpas A, Worman C, Arno J, Nagington J. Sinus histiocytosis with massive lymphadenopathy: virological, immunological, and morphological studies. Br J Haematol. 1980. 45:195–200.

10. 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.

11. Eisen RN, Buckley PJ, Rosai J. Immunophenotypic characterization of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Semin Diagn Pathol. 1990. 7:74–82.

12. Paulli M, Rosso R, Kindl S, Boveri E, Marocolo D, Chioda C, Agostini C, Magrini U, Facchetti F. Immunophenotypic characterization of the cell infiltrate in five cases of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Hum Pathol. 1992. 23:647–654.

13. Deshpande V, Verma K. Fine needle aspiration (FNA) cytology of Rosai Dorfman disease. Cytopathology. 1998. 9:329–335.

14. Ruggiero A, Attina G, Maurizi P, Mule A, Tarquini E, Barone G, Lazzareschi I, Riccardi R. Rosai-Dorfman disease: two case reports and diagnostic role of fine-needle aspiration cytology. J Pediatr Hematol Oncol. 2006. 28:103–106.