Treatment Of Rosai-Dorfman Disease Research Articles

Intralesional Kenalog Injections for Recurrent Cutaneous Rosai-Dorfman Disease (RDD) in the Lower Extremity

Rosai-Dorfman disease (RDD) is a rare disorder of histiocytic origin with nodal, extranodal, and cutaneous classifications often presenting as eruptive rashes, most commonly affecting the face, trunk, and thigh. Due to its rarity, no consensus exists regarding standard of care for these lesions, as most knowledge regarding treatment is provided by case reports. To our knowledge, we report the first case of a patient with a recurrent lower extremity mass consistent with cutaneous RDD, despite surgical excision and topical steroids treatment. A 51-year-old female presented with recurrent RDD after failed treatment with clobetasol ointment and surgical excision at an outside practice. Examination revealed violaceous plaques with overlying scale, without lymphadenopathy. With the failure of the previous excision, the patient was prescribed 5 mg/ml intralesional Kenalog (ILK) injections to suppress inflammation of the lesion. She experienced improved clinical response to treatment and remission of active disease with residual post-inflammatory hyperpigmentation at 10 months after two rounds of injections, although the patient reported vast improvement at 5 months as well. Treatment of RDD is difficult due to its rarity, hindering clinical trial development. Therapeutics reported to be efficacious include topical, intralesional, and systemic steroids, retinoids, methotrexate, chemotherapy, cryotherapy, phototherapy, and radiation. Clinical management is variable and often requires multiple forms of treatment on a case-to-case basis, highlighting the need for larger clinical studies to establish a standard of care. Our case poses interest in the use of ILK for RDD and should be considered in recurrent cases after prior surgical excision.

Painless cervical lymphadenopathy in an elderly patient – a rare case of Rosai-Dorfman disease and Hodgkin's lymphoma

Rosai-Dorfman disease (RDD) is a rare, benign disease with extensive lymphadenopathy. In this case, an 81-year-old gentleman with previous pulmonary tuberculosis presented painless cervical lymphadenopathy associated with generalized weakness, loss of appetite, and cough. The causes of cervical lymphadenopathy in the elderly are comprehensive; thorough history, examination, and appropriate investigations are vital in diagnosing diseases. A lymph node biopsy is recommended if blood investigations and imaging are inconclusive. He was later diagnosed with RDD associated with Hodgkin’s lymphoma, and dexamethasone treatment was initiated. Concomitant diagnoses are rare but should not be disregarded. This case report is a reminder that RDD remains a differential diagnosis of cervical lymphadenopathy. Regular monitoring is required as immune dysregulation from the treatment of RDD may exacerbate quiescently treated pulmonary tuberculosis.

EXTRANODAL ROSAI-DORFMAN DISEASE AFFECTING THE MAXILLA AND MAXILLARY SINUS: A CASE REPORT AND LITERATURE REVIEW

<h3>Introduction</h3> Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a benign, self-limiting non-Langerhans cell histiocytosis of unknown etiology. It commonly affects younger individuals in their first 2 decades of life, showing no significant gender or racial predilection. RDD traditionally presents with bilateral, painless cervical lymphadenopathy with constitutional symptoms. Examples of extranodal RDD have been described in literature, whereby the nasal cavity and paranasal sinuses are common locations, being the site of involvement in 11% of cases. <h3>Case Report</h3> This report highlights a case of extranodal RDD presenting as a progressively enlarging left maxillary intrabony mass in a previously healthy 10-year-old female patient. Radiographic examination revealed a mixed radiolucent-radiopaque lesion with poorly defined margins affecting the left maxilla, extending superiorly to involve the ipsilateral maxillary sinus. Histologic evaluation showed a diffuse infiltrate consisting predominantly of plasma cells with interspersed large cells showing a clear cytoplasm and a vesicular nuclear chromatin pattern. No active emperipolesis was seen within these large cell clear cells. CD3 and CD20 immunohistochemical stains highlighted the presence of scattered reactive T- and B-cells respectively. CD138 confirmed the presence of numerous background plasma cells, which ultimately stained positive for IgG₄. The large clear cells stained positive for S100 and CD68 but were negative for CD1a. <h3>Conclusions</h3> Because of its rarity, wide phenotypic expression, and uncertain etiology, the treatment of RDD is not standardized. Extranodal RDD affecting the craniofacial complex tends to have an indolent behavior and is often self-limiting.

Rosai-Dorfman病的治疗新进展

Rosai-Dorfman disease (RDD) , also known as sinus histiocytosis with massive lymphadenopathy, is a rare benign self-limited histiocytic proliferative disorder with undetermined etiology. There is still no specific treatment for RDD. Surgical excision is the first-line treatment, while topical and systemic therapy can be chosen for the patients who are not unfit for surgery. Classic topical therapies include topical treatment or local injection with glucocorticoids. Photodynamic therapy and laser immunotherapy are considered as promising topical treatments. Traditional systemic treatments include oral glucocorticoids, immunomodulators, acitretin, and so on. Rituximab is a new treatment option for patients with CD20-positive RDD. This review summarizes new progress in the treatment of RDD in recent years. Key words: Histiocytosis, sinus; Therapy; Surgical procedures, operative; Drug therapy; Sinus histiocytosis with massive lymphadenopathy

Author response: Lymphoplasmacyte-rich meningioma involving the whole intracranial dura mater

After reading Drs. Fukuhara and Makifuchi's comment on our NeuroImage,1 and more related literature,2–4 we agree that lymphoplasmacyte-rich meningioma mimics the whole intracranial pachymeningitis. However, we disagree with the viewpoint that lymphoplasmacyte-rich meningioma mimicking pachymeningitis is due to immunoglobulin G4 (IgG4)–related disease. The patient died during our last follow-up. The total survival time of our patient after the biopsy was about 11 months. In fact, we gave the patient steroid therapy when repeated MRI showed no progression 3 months after biopsy, which was inspired by our previous experience with the treatment of Rosai-Dorfman disease,5 but it was ineffective. Although no detailed serologic evaluations of immunoglobulin levels were performed, immunostainings for IgG4 for plasma cell counts demonstrated an IgG4 of 15 per high-powered field, which fell short of the diagnosis of IgG4-related disease.3 In addition, diffuse membranous epithelial membrane antigen expression highlighted the diagnosis of lymphoplasmacyte-rich meningioma.

Rosai-Dorfman disease: tumor biology, clinical features, pathology, and treatment.

Rosai-Dorfman disease (RDD) is a rare, nonmalignant clinical entity characterized by a group of clinical symptoms and characteristic pathological features. Articles that reviewed tumor biology, clinical features, pathology, and treatment for RDD were identified in a search of the literature for the years 1990 to 2014. The results from this body of literature were reviewed and summarized. Patients with RDD generally present with massive, painless cervical lymphadenopathy, fevers, and elevated inflammatory markers. Extranodal disease is typical, with the most common sites being the skin and the central nervous system. Rarely, the gastrointestinal tract is involved. Immunohistochemistry remains the mainstay of diagnosis with S100 and CD68 positive cells while CD1a will be negative of involved histiocytes. Histologically, the disease shows the classical characteristic finding of emperipolesis. Many patients do not require treatment; however, surgical resection remains the mainstay of treatment for symptomatic disease. The role of steroids, chemotherapy, and radiation therapy continue to be based on small case series and case reports. RDD has a variable clinical presentation; therefore, a high degree of suspicion and a thorough pathological review are necessary to diagnose this rare clinical entity. Although some patients will experience spontaneous resolution, others may require surgical resection or steroid therapy and radiation or chemotherapy. Given the rarity of the disease and the lack of a clear therapeutic pathway, referring patients to a tertiary center is recommended for confirming the diagnosis and treatment considerations.

Retrospective analysis of treatment strategies for nasal Rosai-Dorfman disease: Physiological perspective

Background and Aim: Rosai-Dorfman disease (RDD) is a rare form of lymphadenopathy with sinus histiocytosis, whose pathogenesis is undefined. Therefore, the purpose of this study was to explore the best treatment of RDD in the nasal cavity (NC), paranasal sinus (PS), and nasopharynx. Methods: We conducted a review on the treatment stratigies published between 1969 and 2014 with duration of follow-up for nasal RDD along with 5 patients who received treatment in our hospital. Results: The clinical data from literature review and combining with our 5 patients, we have 87 patients in total. There is a slight male to female predominance (male:Female = 1.81:1), average age was 37.36-year, the average duration of follow-up was 23.6-month. Lesions on 32 cases (36.8%) limited to the NC or PSs and 37 cases (42.5%) with involvement of other extranodal sites and 34 cases (40.2%) with lymphadenopathy (one unknown). 62 patients received operations for their initial treatment, 30 (48.39%) patients with recurrence, 21 of the patients received enlarged resection, and 5 (23.81%) patients with recurrence; 41 patients received cytoreductive surgery, and 25 (60.98%) patients recurrence; 20 cases received simple medical treatment, 16 (80.00%) patients with no treatment effect and 1 patients died. 5 patients recieved no therapy, survived with the disease with no progress. Conclusion: Therapeutic effect of medical treatment was was not reported adequately in literature. Enlarged resection was the best way to treat nasal RDD. Enlarged resection combining with medical treatment could reduce the rate of recurrence, but this theory is just from few case reports and literature review. Finally, we found there was no relation between recurrence rate with age, sex, lymphadenopathy and other extranodal sites.

Extranodal Rosai-Dorfman disease with bilateral orbital involvement: Report of a case treated with systemic steroid alone

We report the case of a 41-year-old female with bilateral orbital Rosai-Dorfman disease (RDD) and the result of corticosteroid therapy. The patient developed a swollen mass of bilateral upper and lower eye-lid over a six-month period. Diagnosis was based on the characteristic histopathologic features. She was treated with oral corticosteroids 60 mg/per day for ten days and the dose of corticostreoid was tapered every week. She is currently being treated with 10 mg/per day for 4 months. In general, the prognosis is good, with experiencing remission following a course of oral corticosteroids. There is no evidence that corticosteroids are beneficial in the treatment of RDD in the literature, but data regarding such treatment are sparse. A more formalized surveillance of these cases would help to define more clearly the natural history of the disease and the effects of various treatment modalities.

Venorelbine and methotrexate for the treatment of Rosai‐Dorfman disease: Response

Venorelbine and methotrexate for the treatment of Rosai‐Dorfman disease: Response

Rosai-Dorfman Disease (Extranodal Sinus Histiocytosis) in a Patient with HIV

Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy (SHML), is a rare histiocytosis of unknown etiology that most commonly involves the cervical lymph nodes. Extranodal involvement occurs in 30-40% of cases, most often in the head and neck. Characteristic histologic findings include emperiopolesis (engulfment) of lymphocytes and S-100 protein positivity. Treatment of Rosai-Dorfman disease is unnecessary unless the disorder becomes life- or organ-threatening, since the disease will resolve spontaneously in most patients. We present what, to the best of our knowledge, is the first reported case of Rosai-Dorfman disease limited to the skin in a patient infected with human immunodeficiency virus. SHML is described and diagnostic and therapeutic measures are reviewed.