Benign but fatal: management of endotracheal Rosai-Dorfman disease with acute onset
Rosai-Dorfman disease (RDD) is a non-malignant condition mainly manifesting as a proliferation of histiocytes in lymph nodes. Endotracheal RDD (ERDD) with an acute onset presentation is extremely rare. There are few case reports of ERDD mainly concerning its pathology, diagnostics and bronchoscopic treatment, without providing sufficient clinical information from a comprehensive perspective. As a novel and challenging technique, tracheal resection and reconstruction (TRR) with spontaneous-ventilation video-assisted thoracoscopic surgery (SV-VATS) has been reported as feasible and safe in highly selected patients, but few c...
Source: Thorax - January 12, 2023 Category: Respiratory Medicine Authors: Wang, B., Song, Z.-m., Li, J.-d., Zhao, Y.-l., Sun, P.-l., Tian, J.-k., Yin, J.-z., Zhang, Y. Tags: Thorax Case based discussion Source Type: research
Kidney involvement in Rosai-Dorfman disease
A 32-year-old woman was referred to us for bilateral hydroureteronephrosis due to suspected idiopathic retroperitoneal fibrosis, for which she had received several immunosuppressive therapies (i.e., glucocorticoids, rituximab, and tocilizumab) without benefit. No previous imaging scans were available for review. An abdominal computed tomography revealed bilateral massive pararenal lesions extending into the renal pelvis (Figure 1). The lesions had high metabolic activity at 18fluorodeoxyglucose positron emission tomography, which also showed signs of diffuse sinusitis with osteosclerosis of facial bones and 18fluorodeoxy...
Source: Kidney International - January 1, 2023 Category: Urology & Nephrology Authors: Martina Mazzariol, Francesco Peyronel, Filippo Fagni, Andrea Minervini, Raffaella Santi, Simone Agostini, Augusto Vaglio Tags: Nephrology Image Source Type: research
Case report: A new pathogenic variant of LRBA deficiency with a complex phenotype and Rosai-Dorfman disease
We reported a new pathogenic variant of LRBA deficiency with a complex phenotype—neonatal diabetes, very early-onset inflammatory bowel disease, and polyarthritis—who presented with lymph node enlargement. A case of Rosai-Dorfman’s disease (RDD) was confirmed. The occurrence of an RDD lesion in LRBA-deficiency has never been reported so far. (Source: Frontiers in Immunology)
Source: Frontiers in Immunology - December 9, 2022 Category: Allergy & Immunology Source Type: research
162. Rosai-Dorfman disease of the mandible
Rosai-Dorfman-Destombes disease (RDD) is a rare, benign, self limiting disease with an excellent prognosis in most cases. It classically affects children and young adults and presents with cervical lymphadenopathy in the majority of cases. Presentation in extra-nodal sites only is less common and within in the mandible is rare with only 6 reported cases in the literature. (Source: The British Journal of Oral and Maxillofacial Surgery)
Source: The British Journal of Oral and Maxillofacial Surgery - December 1, 2022 Category: ENT & OMF Authors: Colin Maciver, Colin Maciver, Hayat Ali, Rawia Mohamed Source Type: research
68Ga-FAPI and 18F-FDG PET/CT Images of a Patient With Rosai-Dorfman Disease With Liver Involvement
We report a case of Rosai-Dorfman disease with liver and bone marrow involvement. In this case, 18F-FDG PET/CT revealed FDG avidity in multiple lymph nodes, liver, and bone marrow. 68Ga-FAPI PET/CT showed higher uptake of 68Ga-FAPI than that of 18F-FDG in the same lesions. The findings of 68Ga-FAPI PET/CT in this patient highlighted the potential value of 68Ga-FAPI for staging in Rosai-Dorfman disease. (Source: Clinical Nuclear Medicine)
Source: Clinical Nuclear Medicine - November 15, 2022 Category: Nuclear Medicine Tags: Interesting Images Source Type: research
Rosai-Dorfman Disease involving the eye and ocular adnexa. Clinicopathologic series of 17 patients
CONCLUSION: RDD of the eye and ocular adnexa in Mexican-mestizo patients is common in middle-aged women frequently as an isolated orbital lesion. Histopathology is necessary for accurate diagnosis. Most patients had a favourable outcome after medical and surgical treatment. To the best of our knowledge this is the largest case series of RDD of the eye and ocular adnexa in Latin-American patients.PMID:36373616 | DOI:10.1177/11206721221138319 (Source: European Journal of Ophthalmology)
Source: European Journal of Ophthalmology - November 14, 2022 Category: Opthalmology Authors: Dolores R íos Y Valles-Valles Sharon L Herrera-Cifuentes Abelardo A Rodr íguez-Reyes H éctor A Rodríguez-Martínez Ivette Hern ández-Ayuso Alejandra Z árate-Osorno Source Type: research
Rosai-Dorfman Disease involving the eye and ocular adnexa. Clinicopathologic series of 17 patients
CONCLUSION: RDD of the eye and ocular adnexa in Mexican-mestizo patients is common in middle-aged women frequently as an isolated orbital lesion. Histopathology is necessary for accurate diagnosis. Most patients had a favourable outcome after medical and surgical treatment. To the best of our knowledge this is the largest case series of RDD of the eye and ocular adnexa in Latin-American patients.PMID:36373616 | DOI:10.1177/11206721221138319 (Source: European Journal of Ophthalmology)
Source: European Journal of Ophthalmology - November 14, 2022 Category: Opthalmology Authors: Dolores R íos Y Valles-Valles Sharon L Herrera-Cifuentes Abelardo A Rodr íguez-Reyes H éctor A Rodríguez-Martínez Ivette Hern ández-Ayuso Alejandra Z árate-Osorno Source Type: research
Rosai-Dorfman-Destombes Disease in the Pediatric Head and Neck
CONCLUSION: Pediatric RDD presents at a young age and most commonly involving cervical lymphadenopathy. Ongoing surveillance in the setting of persistence or recurrence without clearly defined prognostic risk factors is important.PMID:36330598 | DOI:10.1177/00034894221130822 (Source: The Annals of Otology, Rhinology, and Laryngology)
Source: The Annals of Otology, Rhinology, and Laryngology - November 4, 2022 Category: ENT & OMF Authors: Nam K Lee Mark A Lovell Brian W Herrmann Source Type: research
Rosai-Dorfman-Destombes Disease in the Pediatric Head and Neck
CONCLUSION: Pediatric RDD presents at a young age and most commonly involving cervical lymphadenopathy. Ongoing surveillance in the setting of persistence or recurrence without clearly defined prognostic risk factors is important.PMID:36330598 | DOI:10.1177/00034894221130822 (Source: The Annals of Otology, Rhinology, and Laryngology)
Source: The Annals of Otology, Rhinology, and Laryngology - November 4, 2022 Category: ENT & OMF Authors: Nam K Lee Mark A Lovell Brian W Herrmann Source Type: research
Rosai-Dorfman-Destombes Disease in the Pediatric Head and Neck
CONCLUSION: Pediatric RDD presents at a young age and most commonly involving cervical lymphadenopathy. Ongoing surveillance in the setting of persistence or recurrence without clearly defined prognostic risk factors is important.PMID:36330598 | DOI:10.1177/00034894221130822 (Source: The Annals of Otology, Rhinology, and Laryngology)
Source: The Annals of Otology, Rhinology, and Laryngology - November 4, 2022 Category: ENT & OMF Authors: Nam K Lee Mark A Lovell Brian W Herrmann Source Type: research
Treatment-Refractory Nodular Scleritis
A 59-year-old woman with a 5-year history of intermittent bilateral uveitis and scleritis presented with painful left uveitis and an episcleral nodule shown to be a uveal-episcleral lesion on ultrasound biomicroscopy. Biopsy of a nasal sinus mass 3 years earlier showed a histiocytic infiltrate with emperipolesis, consistent with Rosai-Dorfman-Destombes disease. What would you do next? (Source: JAMA Ophthalmology)
Source: JAMA Ophthalmology - November 1, 2022 Category: Opthalmology Source Type: research
Rosai-Dorfman disease in the thymus: A case report
Asian J Surg. 2022 Oct 21:S1015-9584(22)01415-4. doi: 10.1016/j.asjsur.2022.09.136. Online ahead of print.NO ABSTRACTPMID:36280490 | DOI:10.1016/j.asjsur.2022.09.136 (Source: Asian Journal of Surgery)
Source: Asian Journal of Surgery - October 24, 2022 Category: Surgery Authors: Siyang Liu Enyu Tang Guangquan Xu Jiaji Lei Source Type: research
MUM1/IRF4 is Highly Expressed in Dermatopathic Lymphadenopathy: Potential Utility in Diagnosis and Differential Diagnosis
Dermatopathic lymphadenopathy (DL) is a distinctive type of lymph node hyperplasia that typically occurs in the setting of chronic dermatologic diseases. DL generally self-resolves following disappearance of the underlying skin stimulus and does not require any specific therapy. We recently observed multiple myeloma oncogene 1/interferon regulatory factor 4 (MUM1/IRF4) expression in a case of DL using immunohistochemical methods. The goal of this study was to systematically assess DL cases for MUM1/IRF4 expression and to survey other histiocytic and Langerhans cell lesions. We particularly focused on Langerhans cell histio...
Source: The American Journal of Surgical Pathology - October 20, 2022 Category: Pathology Tags: Original Articles Source Type: research
Strong Coexpression of Transcription Factors PU.1 and Oct-2 in Rosai-Dorfman Disease
CONCLUSIONS: PU.1 emerged as a robust marker with crisp nuclear staining in RDD histiocytes as well as in engulfed inflammatory cells. Strong coexpression of PU.1 and Oct-2 is a useful diagnostic marker in differentiating histiocytic/dendritic cell proliferations.PMID:36239684 | DOI:10.1093/ajcp/aqac119 (Source: American Journal of Clinical Pathology)
Source: American Journal of Clinical Pathology - October 14, 2022 Category: Pathology Authors: Kala Gnanasekaran Kiruthiga Sheren Younes Yasodha Natkunam Source Type: research
Strong Coexpression of Transcription Factors PU.1 and Oct-2 in Rosai-Dorfman Disease
CONCLUSIONS: PU.1 emerged as a robust marker with crisp nuclear staining in RDD histiocytes as well as in engulfed inflammatory cells. Strong coexpression of PU.1 and Oct-2 is a useful diagnostic marker in differentiating histiocytic/dendritic cell proliferations.PMID:36239684 | DOI:10.1093/ajcp/aqac119 (Source: American Journal of Clinical Pathology)
Source: American Journal of Clinical Pathology - October 14, 2022 Category: Pathology Authors: Kala Gnanasekaran Kiruthiga Sheren Younes Yasodha Natkunam Source Type: research
