Rosai-Dorfman Disease (RDD), a rare histiocytic disorder typically characterized by painless cervical lymphadenopathy, can occasionally present extranodal presentations. Here, we report two distinct RDD cases involving the respiratory system, both treated with interventional bronchoscopy resection. The first case involved a solitary tracheal neoplasm with wild-type (Kirsten rat sarcoma viral oncogene homolog). This patient showed no recurrence or metastasis over a 16-month follow-up period. In contrast, the second case displayed multiple tracheal neoplasms with mutation. While there was no recurrence at the resected site, the untreated tracheal lesions initially progressed at the first four months of follow-up but remained stable at the ten-month follow-up. These cases illustrate the variability and potential progression of RDD, and highlight the promising application of bronchoscopic treatment for managing respiratory involvement in RDD.
Download full-text PDF |
Source |
---|---|
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11872440 | PMC |
http://dx.doi.org/10.1016/j.heliyon.2025.e42643 | DOI Listing |
Front Vet Sci
February 2025
Department of Clinical Sciences, College of Veterinary Medicine and Biomedical Sciences, Colorado State University, Fort Collins, CO, United States.
Background: Pulmonary involvement of Non-Langerhans Cell Histiocytosis (PNLCH) is a rare cause of interstitial pulmonary disease in people and are classified as either Erdheim-Chester disease (ECD) or Rosai-Dorfman disease (RDD). In veterinary medicine, feline pulmonary Langerhans cell histiocytosis (PLCH) has been identified as an infiltrative histiocytic disorder with an insidious onset of progressive respiratory distress and is non-responsiveness to empiric therapies. Unfortunately, subsequent death either from respiratory failure or humane euthanasia are the reported outcomes in all reported cases.
View Article and Find Full Text PDFInt J Hematol
March 2025
Department of Pediatrics, National Hospital Organization Kyushu Cancer Center, Fukuoka, Japan.
Background: Rosai-Dorfman-Destombes disease (RDD) is a rare form of histiocytosis, characterized by the accumulation of S100 protein-positive and CD1a-negative histiocytes with emperipolesis. Recently, oncogenic mutations in mitogen-activated protein kinase pathway genes were reported in nearly half of RDD patients.
Methods: We conducted a nationwide retrospective survey of childhood RDD in Japan.
Indian J Nucl Med
January 2025
Spect Lab Nuclear Medicine Services, Pune, Maharashtra, India.
Rosai-Dorfman-Destombes (RDD) disease is also called as sinus histiocytosis and is characterized by enlarged lymph nodes and previously called as non-Langerhans cell histiocytosis. Based on pathologic, molecular, and genetic features, RDD disease has been classified into sporadic noncutaneous (classical nodal, extranodal, neoplasia associated, and autoimmune associated), familial (H syndrome, autoimmune lymphoproliferative syndrome related, and familial NOS), and cutaneous subtypes. Cutaneous RDD disease is not associated with lymphadenopathy or visceral organ involvement.
View Article and Find Full Text PDFFront Neurol
February 2025
Department of Neurosurgery, The Second Hospital & Clinical Medical School, Lanzhou University, Lanzhou, China.
Rosai-Dorfman-Destombes disease (RDD) is a rare histiocytic proliferative disorder, with primary scalp and intracranial involvement being particularly uncommon. The imaging features and clinical manifestations of RDD often overlap with other intracranial lesions, such as meningiomas, leading to misdiagnosis. This study presents a case of primary scalp and intracranial RDD, with a comprehensive analysis of its imaging, pathological, and intraoperative findings, alongside a review of the literature on central nervous system (CNS) RDD and its diagnostic and therapeutic advancements.
View Article and Find Full Text PDFHeliyon
February 2025
Department of Respiratory and Critical Care Medicine, The First Affiliated Hospital of Nanjing Medical University, Nanjing, Jiangsu 210029, PR China.
Rosai-Dorfman Disease (RDD), a rare histiocytic disorder typically characterized by painless cervical lymphadenopathy, can occasionally present extranodal presentations. Here, we report two distinct RDD cases involving the respiratory system, both treated with interventional bronchoscopy resection. The first case involved a solitary tracheal neoplasm with wild-type (Kirsten rat sarcoma viral oncogene homolog).
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!