A Case of Orbital Rosai-Dorfman Disease Responding to Radiotherapy

Background:Rosai-Dorfman disease (RDD) is typically characterized by painless bilateral and symmetrical cervical lymphadenopathy, with associated fever and leukocytosis. The aim of the current study was to summarize the clinical features and imaging characteristics of RDD, in an effort to improve its diagnostic accuracy.Methods:The study was analyzed from 32 patients between January 2011 and December 2017; of these, 16 patients had pathologically diagnosed RDD, eight had pathologically diagnosed meningioma, and eight pathologically diagnosed lymphoma. All patients underwent computed tomography and magnetic resonance imaging (MRI). Clinical features and imaging characteristics of RDD were analyzed retrospectively. The mean apparent diffusion coefficient (ADC) values of lesions at different sites were measured, and one-way analysis of variance and the least significant difference t-test were used to compare the differences between groups and draw receiver operating characteristic curves. The tumors were excised for biopsy and analyzed using immunohistochemistry.Results:The mean ADCs were (0.81 ± 0.10) × 10−3 mm2/s for intercranial RDD, (0.73 ± 0.05) × 10−3 mm2/s for nasopharyngeal RDD, (0.74 ± 0.11) × 10−3 mm2/s for bone RDD, and (0.71 ± 0.04) × 10−3 mm2/s for soft-tissue RDD. The optimum ADC to distinguish intracranial RDD from lymphoma was 0.79 × 10−3 mm2/s (62.5% sensitivity and 100% specificity) and to distinguish meningioma from intracranial RDD was 0.92 × 10−3 mm2/s (62.5% sensitivity and 100% specificity). Levels of C-reactive protein, erythrocyte sediment rate and D-dimer were significantly elevated (81%, 87%, and 75%, respectively). On immunohistochemistry, RDD was positive for both S-100 and CD68 proteins but negative for CD1a.Conclusions:Conventional MRI, combined with diffusion-weighted imaging and ADC mapping, is an important diagnostic tool in evaluating RDD patients. An accurate diagnosis of RDD should consider the clinical features, imaging characteristics, and the pathological findings.

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“…Some reports have described a full resolution of RDD with treatment by radiotherapy, whereas others have shown no response. [ 23 ]…”

Section: Discussionmentioning

confidence: 99%

A Study on Clinical Characteristics and Magnetic Resonance Imaging Manifestations on Systemic Rosai-Dorfman Disease

Cheng

Gao

2018

Chinese Medical Journal

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“…Radiation therapy was known to have limited efficacy in most cases of RDD, although recent reports have shown benefit in a refractory extra-nodal RDD with higher radiation doses [12, 13]. However, radiation dose—response relationship and subsequent response for RDD—is not clear because of lack of reporting cases treated with radiotherapy, infrequent use of high radiation doses, and the rarity of RDD itself.…”

Section: Discussionmentioning

confidence: 99%

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

Maklad

Bayoumi

Tunio

et al. 2013

Case Reports in Hematology

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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.

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“…The role of radiotherapy is not well understood; some reports have described full resolution with this treatment, whereas others have shown no response. [ 4 5 ] Systemic corticosteroids are usually helpful in decreasing nodal size and symptoms, however, they can be immunosuppressive and recurrence of RDD lesions can occur after a short period of interruption. [ 21 ] Chemotherapy has resulted in controversial results.…”

Section: Discussionmentioning

confidence: 99%

Extranodal right-optic nerve Rosai-Dorfman disease: A rare localization case report

et al. 2016

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Background:Rosai–Dorfman is a rare disease that usually occurs in young adults. It is characterized with massive painless cervical lymphadenopathy and histiocyte proliferation. Isolated intracranial involvement is extremely rare. Our aim is to present a new rare case of extranodal Rosai–Dorfman disease that involved the right optic nerve in a 4-year-old boy.Case Description:A 4-year-old boy with right-sided convergent strabismus and amblyopia lasting for 1 year was treated at the Department of pediatric ophthalmology. Initial optical fundus examination was normal. Examination repeated after 1 year noted the atrophy of the optic nerve papilla. Visual evoked potentials of the right eye showed normal findings of prechiasmatic visual pathway with severe dysfunction of the right optic nerve. Magnetic resonance imaging (MRI) of the brain and orbits showed expansive changed and elongated right optic nerve with contrast enhancement, and smaller lesion in the right temporal operculum region visible in T2 and fluid-attenuated inversion recovery sequence. Through small eyebrow “keyhole” osteoplastic frontoorbital craniotomy the fusiform enlarged (to 2 cm) right optic nerve was identified, resected between the eyeball and optic chiasm, and transferred for pathohistological analysis. Early postoperative course had no complications. Histological, immunohistochemical, and ultrastructural analyses revealed extranodal Rosai–Dorfman disease. Right periorbital edema was verified on the 7th postoperative day and regressed to supportive therapy. Control multi slice computed tomography (MSCT) and MRI of endocranium and orbits showed total tumor removal with no signs of complications.Conclusion:Although rare, extranodular intracranial Rosai–Dorfman disease should be taken into account in the differential diagnosis of intracranial and intraorbital lesions, especially in the pediatric age group.

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A Case of Orbital Rosai-Dorfman Disease Responding to Radiotherapy

Cited by 26 publications

References 19 publications

A Study on Clinical Characteristics and Magnetic Resonance Imaging Manifestations on Systemic Rosai-Dorfman Disease

A Study on Clinical Characteristics and Magnetic Resonance Imaging Manifestations on Systemic Rosai-Dorfman Disease

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

Extranodal right-optic nerve Rosai-Dorfman disease: A rare localization case report

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