Steroid-Resistant Extranodal Rosai-Dorfman Disease of Cheek Mass and Ptosis Treated with Radiation Therapy

Abstract

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 3 × 3 cm and left upper eyelid mass of size 1 × 2 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.

Successful treatment of refractory cutaneous Rosai–Dorfman disease with vincristine

“…1i). This last finding, as already previously reported, 4 may be related to the recruitment of specific CD8 + T lymphocytes that mediate the contact allergy to SADBE, rather than a CD4/CD8 switch correlated with the hair regrowth. The hypothesis of a direct effect of topical immunotherapy on Th17 cells is sustained by the recent observation of a reduction of skin IL-17 upon diphenylcyclopropenone treatment for a scalp metastatic melanoma.…”

“…1i). This last finding, as already previously reported, 4 may be related to the recruitment of specific CD8 + T lymphocytes that mediate the contact allergy to SADBE, rather than a CD4/CD8 switch correlated with the hair regrowth.…”

“…CVP (cyclophosphamide, prednisone and vincristine), and RCVP (rituximab, cyclophosphamide, vincristine and prednisolone) regimens have shown good response in some patients. 3,4 In our case, after failure of various therapies that had been reported effective for RDD, we tried vincristine, to which the patient responded very well in a short time. To our knowledge, this is the first report in which RDD was successfully treated with vincristine alone.…”

The Non-Langerhans Cell Histiocytoses (Rare Histiocytoses) – Clinical Aspects and Therapeutic Approaches

“…Less often, the disease can be found in kidneys, the urogenital tract, and muscles [65,94,112]. Unspecific symptoms such as fever may be seen, and laboratory examination may reveal elevated erythrocyte sedimentation rate, anemia, leukocytosis and a polyclonal hyper-gammaglobinemia, in particuar IgG4 syndrome, which will be described in detail below [128]. In general, the course is self-limited with good prognosis, but relapses and organ involvement resulting in severe complications have been reported.…”

Extranodal intraosseous Rosai–Dorfman disease in the lower jaw: a case report and review of the literature

“…Corticosteroid-based schemes are currently prescribed and are reported to be effective in cases of nodal RDD or extranodal disease without bone involvement [18]. Exceptional observations of steroid-resistant extranodal soft-tissue RDD were shown to benefit from radiotherapy [19]. On the other hand, surgical management either in form of conservative curettage or radical resection is generally chosen for intraosseous RDD.…”