Refractory Extra nodal Rosai-Dorfman disease of cheek and upper eyelid: A rare case
Keywords:
Rosai Dorfman Disease, Extra nodal, Refractory, Cheek mass, S-100, CD1a
Abstract
Rosai-dorfman disease (RDD) is a rare benign disorder of histiocytes characterized histologically by intracellular engulfment of lymphocytes. Extra-nodal RDD has been reported in 43% of cases. However, soft tissue cheek mass is rare presentation of extra-nodal RDD. A 31-year-old female presented with a history of right cheek mass and right upper eyelid mass gradually increasing for 8 months, with no palpable cervical lymphadenopathy. The masses were excised and sent for histopathological examination. The histiocytes showed positive immunostaining for S100 and negativity for CD1a, consistent with extra-nodal RDD. Patient was on systemic steroids and was doing well. After 12 months of follow-up, patient had recurrence in right cheek, which was again excised. Again the mass recurred after 10 months, was excised and patient was put on radiotherapy to the cheek mass. Extra-nodal RDD with involvement of cheek is a rare presentation. Incorporation of S100 and CD1a is helpful in diagnoses of RDD and differentiating it from other benign histiocytosis. The causes of RDD are not fully understood, and treatment strategies can be different according to severity or vital organ involvement.References
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9. Lima FB, Barcelos PS, Constancio AP, No-gueira CD, Melo-Filho AA. Rosai-Dorfman disease with spontaneous resolution: case re-port of a child. Revista Brasileira de Hematologiae Hemoterapia. 2011;33:312–4.
10. D. Komp. The treatment of sinus histiocytosis with massivelymphadenopathy (Rosai- Dorfman disease). Seminars in Diagnostic Pathology. 1990;7:83–6.
11. Cooper SL, Chavis PS, Fortney JA, Watkins JM, Caplan MJ, Jenrette JM. A case of orbital rosai-dorfman disease responding to radiotherapy. Journal of Pediatric Hematology/ Oncology. 2008;30:744–8.
2. Wenig BM, Abbondanzo SL, Childers EL, Kapadia SB, Heffner DR. Extra nodal sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) of the head and neck. Hum Pathol 1993;24:483–92.
3. Destombes P: Adenitis with lipid excess, in children or young adults, seen in the Antilles and in Mali (4 cases) [in French]. Bull Soc Pathol Exot Filiales. 1965;58:1169-75.
4. Rosai J, Dorfman RF: Sinus histiocytosis with massive lymphadenopathy: A newly recog-nized benign clinicopathological entity. Arch Pathol 1969;87:63-70.
5. Udono HFK, Okamoto H, Tabuchi K. Ro-sai-Dorfman disease presenting multiple intracranial lesions with unique findings on magnetic resonance imaging: Case report. J Neurosurg 1999;91:335-9.
6. Kattner KA, Stroink AR, Roth TC, Lee JM. Rosai-Dorfman disease mimicking parasagittal meningioma: Case presentation and review of literature. Surg Neurol 2000;53:452-7.
7. Kong YY, Kong JC, Shi DR, Lu HF, Zhu XZ, Wang J, et al. Cutaneous Rosai- Dorfman disease: a clinical and histopathologic study of 25 cases in China. Am J Surg Pathol. 2007;31: 341-50.
8. Khoo JJ, Rahmat BO. Cutaneous Rosai- Dorfman disease. The Malaysian Journal of Pathology 2007;29:49–52.
9. Lima FB, Barcelos PS, Constancio AP, No-gueira CD, Melo-Filho AA. Rosai-Dorfman disease with spontaneous resolution: case re-port of a child. Revista Brasileira de Hematologiae Hemoterapia. 2011;33:312–4.
10. D. Komp. The treatment of sinus histiocytosis with massivelymphadenopathy (Rosai- Dorfman disease). Seminars in Diagnostic Pathology. 1990;7:83–6.
11. Cooper SL, Chavis PS, Fortney JA, Watkins JM, Caplan MJ, Jenrette JM. A case of orbital rosai-dorfman disease responding to radiotherapy. Journal of Pediatric Hematology/ Oncology. 2008;30:744–8.
Published
2014-07-20
Issue
Section
Case Report
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