Rosai - Dorfman Disease Masquerading As Sarcoma : A Case Report

  • Sudha P Meena Department of Pathology, Government Medical College, Kota(India)
  • Priyanka Patangia Department of Pathology, AIIMS, Bhopal(India)
  • Naresh N. Rai Department of Pathology, Government Medical College, Kota(India)
Keywords: Rosai-Dorfman disease, Sinus histiocytosis with massive lymphadenopathy, Sarcoma, Immunohistochemistry

Abstract

Rosai Dorfman disease known as sinus histiocytosis with massive lymphadenopathy is an uncommon benign systemic histo-proliferative disease which affects the cervical lymph nodes commonly. The characteristic histological feature is lymphophagocytosis (emperipolesis) which is the hallmark feature. Here, we report a case of 35‑year‑old Indian female  presenting as a mass on medial aspect of thigh appearing as soft tissue mass, which on cytological examination was misdiagnosed as malignant mesenchymal neoplasm but histopathological examination and immunohistochemistry confirmed the diagnosis. According to the literature reviewed  no case of Rosai Dorfman disease with only ingunal lymphadenopathy  was reported till date which was seen in present case. RDD is a rare inflammatory non neoplastic process that should be considered in the differential diagnosis of  lympadenopathy and soft tissue tumor such as soft tissue sarcoma. The diagnosis  of RDD is made on the basis of clinical suspicion and is confirmed by histopathology and immunohistochemistry.DOI:10.21276/APALM.1464

References

1. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphade¬nopathy. A newly recognized benign clinicopathological entity. Arch Pathol. 1969;87(1):63-70.

2. Kong Y, Kong J, Shi D, Lu H, Zhu X, Wang J, Chen Z: Cutaneous Rosai– Dorfman Disease: a clinical and histopathologic study of 25 cases in China. Am J Surg Pathol 2007, 21:341–350.

3. Pinto DCG, Vidigal TA, Castro B, Santos BH, DeSousa NJA: Rosai–Dorfman disease in the differential diagnosis of cervical lymphadenopathy. Bras J Otorrinolaringol 2008, 74:632–635

4. Komaragiri et al.: Extranodal Rosai–Dorfman disease: a rare soft tissue neoplasm masquerading as a sarcoma. World Journal of Surgical Oncology 2013 11:63.

5. Foucar E, Rosai J, Dorfman RF. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Sem Diagn Pathol 1990;7:19 –73.

6. Ioachim HL, Medeiros J, eds. Ioachim’s Lymph Node Pathology. 4th ed. Philadelphia: Wolters Kluwer/Lippincott Williams & Wilkins; 2009.

7. Chang LY, Kou T, Chan HL. Extranodal Rosai-Dorfman disease with cutaneous,ophthalmic and laryngeal involvement: report of a case treated with isotretinoin. Int J Dermatol. 2002;41:888-91.

8. Pitamber HV, Grayson W. Five case of cutaneous Rosai-Dorfman disease. Clin Exp Dermatol. 2003;28:17-21.

9. Landim FM, Rios Hde O, Costa CO, Feitosa RG, Rocha Filho FD, Costa AA. Cutaneous Rosai-Dorfman disease. An Bras Dermatol. 2009;84:275-8.

10. Becker M, Gaiser T, Middel P, Rompel R. Clinicopathologic challenge. Destombes- Rosai-Dorfman disease (DRDD) (sinus histiocytosis with massive lymphadenopathy). Int J Dermatol. 2008;47:125-7.
Published
2018-03-22
Section
Case Report