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Background: Rosai-Dorfman disease (RDD) is a rare benign proliferative disease whose etiology is not clear and may be related to infection or unexplained immune dysfunction. The authors present a case of RDD with lung involvement in a 10-year-old patient.
Case Summary: A 10-year-old girl found that her left cervical lymph nodes were enlarged for more than 7 mo, and the largest range was about 6.5 cm × 5.9 cm × 8.1 cm. Cervical magnetic resonance imaging showed multiple masses in the left neck, with low signal intensity on T1-weighted images and high signal intensity on T2-weighted images. A malignant tumor, with a high possibility of lymph node metastasis, was initially considered. At the same time, lung computed tomography showed multiple nodules of different sizes scattered on both sides of the lung, with uniform internal density. Thus, a possible metastatic tumor was considered. Finally, RDD was diagnosed by pathology and immunohistochemistry. According to the antibiogram, clindamycin was administered for 2 wk, and prednisone acetate was administered for 7 wk. Nine months later, the ulcer in the left neck was better than before, but the imaging showed that the lesion was not controlled.
Conclusion: The diagnosis of RDD cannot be made by a single tool and its treatment is a long-term exploratory process. Follow-up is necessary.
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http://dx.doi.org/10.12998/wjcc.v9.i17.4285 | DOI Listing |
Cureus
July 2025
Radiation Oncology, American British Cowdray Medical Center, Mexico City, MEX.
Rosai-Dorfman disease (RDD) is a rare subtype of sinus histiocytosis, with primary cutaneous RDD (PCRDD) being an even rarer form, characterized by cutaneous lesions without systemic involvement. PCRDD presents a significant diagnostic challenge and is often resistant to conventional therapies, including surgical resection, steroids, and chemotherapy. Currently, no established management guidelines exist for this condition.
View Article and Find Full Text PDFPediatr Dermatol
September 2025
Department of Dermatology, Venereology and Leprosy, Father Muller Medical College, Mangalore, Karnataka, India.
Cutaneous Rosai-Dorfman disease (CRDD) is a rare non-Langerhans cell histiocytosis that mimics various granulomatous and neoplastic dermatoses, often leading to misdiagnosis. We report an 18-year-old male with a 13-year history of progressively enlarging erythematous plaques over the nasal bridge and right forearm, initially diagnosed as granulomatous inflammation. Histopathological examination revealed emperipolesis only after meticulous serial sectioning, and immunohistochemistry confirmed the diagnosis of CRDD.
View Article and Find Full Text PDFAm J Case Rep
August 2025
Department of Pathology and Laboratory Medicine, Nemours Children's Health, Orlando, FL, USA.
BACKGROUND Sinus histiocytosis with massive lymphadenopathy (also known as Rosai-Dorfman disease [RDD]), was originally recognized in 1969 by Rosai and Dorfman. RDD is characterized by the accumulation of activated histiocytes in various tissues and organs, but most commonly in lymph nodes. RDD is subclassified into 2 forms.
View Article and Find Full Text PDFPediatr Radiol
August 2025
Children's Healthcare of Atlanta, 3901 Beaubien Blvd, Atlanta, Detroit, MI, 48201, USA.
Rosai-Dorfman disease is an infiltrative neoplastic process that can manifest with a variety of symptoms based on its location. Rarely, Rosai-Dorfman disease can present as an intracardiac mass or with compression of extracardiac vasculature. When cardiovascular involvement is considered, cardiac MRI can aid in tissue characterization, rule out alternative differential diagnoses, and assess physiological consequences of the mass.
View Article and Find Full Text PDFBalkan Med J
August 2025
Department of Pathology, Salah Azaiez Institute, University Tunis Elmanar Faculty of Medicine, Tunis, Tunisia.