Background. Rosai-Dorfman Disease (RDD) is rare benign hematologic disorder of histiocytes, which usually manifests as painless lymphadenopathy, fever, leukocytosis and hypergammaglobulinemia. Extranodal RDD has been reported in 43% of cases, with skin as commonly involved site followed by head and neck region. However, soft tissue cheek mass is rare presentation of extra-nodal RDD. Case Presentation. A 26-year-old Saudi man presented with a six-month history of right cheek swelling and left upper eyelid swelling. Physical examination revealed right cheek mass of size ?cm and left upper eyelid mass of size ?cm and no palpable cervical lymphadenopathy. Incisional biopsy of cheek mass showed positivity for S100 and negativity for CD1a, consistent with extra-nodal RDD. Patient did not respond to systemic steroids and was given radiation therapy to left orbit with minimal response. Then, he was started on chemotherapy Rituximab, cyclophosphamide, vincristine, and prednisolone (RCVP) 8 cycles followed by reirradiation 10?Gy in 10 fractions with 6?MeV electron with complete response. After 12 months of followup, patient had recurrence in right cheek and was started on radiotherapy to the cheek mass. Conclusion. 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. Treatment consists of surgery, steroids, chemotherapy, and radiation therapy. 1. Introduction Rosai-Dorfman Disease (RDD) is also referred to as sinus histiocytosis with massive lymphadenopathy (SHML), which is rare, benign, and self-limited and is a non-Langerhans proliferative disorder of histiocytes [1]. It is clinically characterized by painless cervical lymphadenopathy, fever, anemia, leukocytosis, elevated erythrocyte sedimentation rate (ESR), and a polyclonal hypergammaglobulinemia [2]. It may affect any age group or any gender and without racial predilection; however, 80% of the patients are aged 20 years or younger at onset [3]. Extranodal RDD has been reported in 43% of cases, and the skin is the most common extranodal site [4]. However, when the disease is confined to the skin, it is classified as cutaneous Rosai-Dorfman disease (CRDD). The skin lesions have variable forms, ranging from single papules to psoriasiform, xanthomatous, acneiform, vasculitis-like, and pseudotumor-like multiple nodules and plaques; any part of the body may be affected, but more common in head and neck region [5]. Herein we presented 26-year-old Saudi man, who present with RDD of
References
[1]
T. Brenn, E. Calonje, S. R. Granter et al., “Cutaneous Rosai-Dorfman disease is a distinct clinical entity,” American Journal of Dermatopathology, vol. 24, no. 5, pp. 385–391, 2002.
[2]
C. M. Stefanato, P. S. Ellerin, and J. Bhawan, “Cutaneous sinus histiocytosis (Rosai-Dorfman disease) presenting clinically as vasculitis,” Journal of the American Academy of Dermatology, vol. 46, no. 5, pp. 775–778, 2002.
[3]
A. Abdollahi, F. A. Ardalan, and M. Ayati, “Extranodal Rosai-Dorfman disease of the kidney,” Annals of Saudi Medicine, vol. 29, no. 1, pp. 55–57, 2009.
[4]
J. L. Frater, J. S. Maddox, J. M. Obadiah, and M. Y. Hurley, “Cutaneous Rosai-Dorfman disease: comprehensive review of cases reported in the medical literature since 1990 and presentation of an illustrative case,” Journal of Cutaneous Medicine and Surgery, vol. 10, no. 6, pp. 281–290, 2006.
[5]
C. I. Lu, T. T. Kuo, W. R. Wong, and H. S. Hong, “Clinical and histopathologic spectrum of cutaneous Rosai-Dorfman disease in Taiwan,” Journal of the American Academy of Dermatology, vol. 51, no. 6, pp. 931–939, 2004.
[6]
Y. Y. Kong, J. C. Kong, D. R. Shi et al., “Cutaneous Rosai-Dorfman disease: a clinical and histopathologic study of 25 cases in China,” American Journal of Surgical Pathology, vol. 31, no. 3, pp. 341–350, 2007.
[7]
J. J. Khoo and B. O. Rahmat, “Cutaneous Rosai-Dorfman disease,” The Malaysian Journal of Pathology, vol. 29, no. 1, pp. 49–52, 2007.
[8]
N. Arakaki, G. Gallo, R. Majluf et al., “Extranodal rosai-dorfman disease presenting as a solitary mass with human herpesvirus 6 detection in a pediatric patient,” Pediatric and Developmental Pathology, vol. 15, pp. 324–328, 2012.
[9]
M. Paulli, G. Bergamaschi, L. Tonon et al., “Evidence for a polyclonal nature of the cell infiltrate in sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease),” British Journal of Haematology, vol. 91, no. 2, pp. 415–418, 1995.
[10]
F. B. Lima, P. S. Barcelos, A. P. Constancio, C. D. Nogueira, and A. A. Melo-Filho, “Rosai-Dorfman disease with spontaneous resolution: case report of a child,” Revista Brasileira de Hematologia e Hemoterapia, vol. 33, pp. 312–314, 2011.
[11]
D. Komp, “The treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease),” Seminars in Diagnostic Pathology, vol. 7, pp. 83–86, 1990.
[12]
S. L. Cooper, P. S. Chavis, J. A. Fortney, J. M. Watkins, M. J. Caplan, and J. M. Jenrette, “A case of orbital rosai-dorfman disease responding to radiotherapy,” Journal of Pediatric Hematology/Oncology, vol. 30, no. 10, pp. 744–748, 2008.
[13]
C. G. Hadjipanayis, G. Bejjani, C. Wiley, T. Hasegawa, M. Maddock, and D. Kondziolka, “Intracranial Rosai-Dorfman disease treated with microsurgical resection and stereotactic radiosurgery: case report,” Journal of Neurosurgery, vol. 98, no. 1, pp. 165–168, 2003.