Treatment of Langerhans Cell Histiocytosis and Histiocytic Disorders: A Focus on MAPK Pathway Inhibitors
Paediatr Drugs. 2023 May 19. doi: 10.1007/s40272-023-00569-8. Online ahead of print.ABSTRACTHistiocytic disorders are rare diseases defined by the clonal accumulation of a macrophage or dendritic cell origin. These disorders include Langerhans cell histiocytosis, Erdheim-Chester disease, juvenile xanthogranuloma, malignant histiocytoses, and Rosai-Dorfman-Destombes disease. These histiocytic disorders are a diverse group of disorders with different presentations, management, and prognosis. This review focuses on these histiocytic disorders and the role of pathological ERK signaling due to somatic mutations in the mitoge...
Source: Paediatric Drugs - May 19, 2023 Category: Pediatrics Authors: Ashley V Geerlinks Oussama Abla Source Type: research
Treatment of Langerhans Cell Histiocytosis and Histiocytic Disorders: A Focus on MAPK Pathway Inhibitors
Paediatr Drugs. 2023 May 19. doi: 10.1007/s40272-023-00569-8. Online ahead of print.ABSTRACTHistiocytic disorders are rare diseases defined by the clonal accumulation of a macrophage or dendritic cell origin. These disorders include Langerhans cell histiocytosis, Erdheim-Chester disease, juvenile xanthogranuloma, malignant histiocytoses, and Rosai-Dorfman-Destombes disease. These histiocytic disorders are a diverse group of disorders with different presentations, management, and prognosis. This review focuses on these histiocytic disorders and the role of pathological ERK signaling due to somatic mutations in the mitoge...
Source: Paediatric Drugs - May 19, 2023 Category: Pediatrics Authors: Ashley V Geerlinks Oussama Abla Source Type: research
How I Diagnose Rosai-Dorfman Disease
CONCLUSIONS: Management of RDD is dependent on the extent of organ involvement and clinical symptoms. In patients who require therapy, next-generation sequencing is recommended to identify MAPK/ERK pathway mutations for targeted therapy.PMID:37167084 | DOI:10.1093/ajcp/aqad047 (Source: American Journal of Clinical Pathology)
Source: American Journal of Clinical Pathology - May 11, 2023 Category: Pathology Authors: Aishwarya Ravindran Karen L Rech Source Type: research
Intracranial Rosai Dorfman disease - A rare differential diagnosis of multiple meningiomas: a case report
We report the case of a 59-year-old man, who began 8 months before admission with headache, diminished visual acuity in the temporal hemifields, hyposmia, and seizures. Magnetic resonance imaging showed three midline skull-base lesions in anterior, media, and posterior fossae. We performed a complete resection of symptomatic lesions using a bifrontal craniotomy. The histopathological analysis determined RDD, therefore, we started steroid treatment. Our case description is due to the diagnosis and location, one of the rarest reported to date in the literature.PMID:37156245 | DOI:10.24875/CIRU.21000784 (Source: Cirugia y Cirujanos)
Source: Cirugia y Cirujanos - May 8, 2023 Category: Surgery Authors: Jos é L Navarro-Olvera Gustavo Parra-Romero Antonio Cruz-Cruz Erick G ómez-Apo Laura Ch ávez-Macias Jos é D Carrillo-Ruiz Source Type: research
Intracranial Rosai Dorfman disease - A rare differential diagnosis of multiple meningiomas: a case report
We report the case of a 59-year-old man, who began 8 months before admission with headache, diminished visual acuity in the temporal hemifields, hyposmia, and seizures. Magnetic resonance imaging showed three midline skull-base lesions in anterior, media, and posterior fossae. We performed a complete resection of symptomatic lesions using a bifrontal craniotomy. The histopathological analysis determined RDD, therefore, we started steroid treatment. Our case description is due to the diagnosis and location, one of the rarest reported to date in the literature.PMID:37156245 | DOI:10.24875/CIRU.21000784 (Source: Cirugia y Cirujanos)
Source: Cirugia y Cirujanos - May 8, 2023 Category: Surgery Authors: Jos é L Navarro-Olvera Gustavo Parra-Romero Antonio Cruz-Cruz Erick G ómez-Apo Laura Ch ávez-Macias Jos é D Carrillo-Ruiz Source Type: research
Intracranial Rosai Dorfman disease - A rare differential diagnosis of multiple meningiomas: a case report
We report the case of a 59-year-old man, who began 8 months before admission with headache, diminished visual acuity in the temporal hemifields, hyposmia, and seizures. Magnetic resonance imaging showed three midline skull-base lesions in anterior, media, and posterior fossae. We performed a complete resection of symptomatic lesions using a bifrontal craniotomy. The histopathological analysis determined RDD, therefore, we started steroid treatment. Our case description is due to the diagnosis and location, one of the rarest reported to date in the literature.PMID:37156245 | DOI:10.24875/CIRU.21000784 (Source: Cirugia y Cirujanos)
Source: Cirugia y Cirujanos - May 8, 2023 Category: Surgery Authors: Jos é L Navarro-Olvera Gustavo Parra-Romero Antonio Cruz-Cruz Erick G ómez-Apo Laura Ch ávez-Macias Jos é D Carrillo-Ruiz Source Type: research
Surgical management and outcome of primary intracranial Rosai-Dorfman disease: a single-institute experience and pooled analysis of individual patient data
AbstractPrimary intracranial Rosai-Dorfman disease (PIRDD) is considered a nonmalignant nonneoplastic entity, and the outcome is unclear due to its rarity. The study aimed to elaborate the clinic-radiological features, treatment strategies, and progression-free survival (PFS) in patients with PIRDD. Patients with pathologically confirmed PIRDD in our institute were reviewed. Literature of PIRDD, updated until December 2019, was systematically searched in 7 databases (Embase, PubMed, Cochrane database, Web of Science, Wanfang Data Knowledge Service Platform, the VIP Chinese Science and Technology Periodical Database (VIP), ...
Source: Neurosurgical Review - March 27, 2023 Category: Neurosurgery Source Type: research
Bilateral breast Rosai ‐Dorfman disease screen detected by mammography
We present a case of a 59-year-old female presenting with abnormal bilateral findings on screening mammography, who was found to have a rare presentation of Rosai-Dorfman disease. (Source: Clinical Case Reports)
Source: Clinical Case Reports - March 20, 2023 Category: General Medicine Authors: Christina Sumner,
Karma Salem,
Luma Abunimer,
Abdulwahab Ewaz,
Linsheng Zhang,
Ashley Monsrud,
Nabil Calisi Tags: CASE REPORT Source Type: research
Primary diffuse Rosai-Dorfman disease in central airway: a case report and literature review
Rosai-Dorfman disease (RDD) is a rare benign non-langerhans cell histiocytosis, mainly involving lymph nodes and skin. It is even rarer occurring only in central airway of lung and in diffuse form. Central air... (Source: BMC Pulmonary Medicine)
Source: BMC Pulmonary Medicine - March 10, 2023 Category: Respiratory Medicine Authors: Lei Wu, Yan Zhang, Xiaoya Guo, Xing Tang, Keke Xin, Li Yang, Zhe Wang, Wen Jian and Feng Zhao Tags: Case report Source Type: research
Nasal Presentations of Rosai-Dorfman Disease: Clinical Manifestation and Treatment Outcomes
CONCLUSIONS: Diffuse lesions in nasal cavity and sinuses, and even widely affected nasal skull base, laryngopharynx, orbit, and cavernous sinus, should be considered the possibility of Rosai-Dorfman disease. Characteristic immunohistochemical staining is helpful for the diagnosis. Endoscopic surgical therapy remains the mainstream treatment for patients enduring an unbearable course. Oral corticosteroid administration serves as an adjuvant therapy for first-line treatments.PMID:36884341 | DOI:10.1177/01455613231162226 (Source: Ear, Nose and Throat Journal)
Source: Ear, Nose and Throat Journal - March 8, 2023 Category: ENT & OMF Authors: Haoyuan Xu Huankang Zhang Wanpeng Li Chen Zhang Huan Wang Dehui Wang Source Type: research
Nasal Presentations of Rosai-Dorfman Disease: Clinical Manifestation and Treatment Outcomes
CONCLUSIONS: Diffuse lesions in nasal cavity and sinuses, and even widely affected nasal skull base, laryngopharynx, orbit, and cavernous sinus, should be considered the possibility of Rosai-Dorfman disease. Characteristic immunohistochemical staining is helpful for the diagnosis. Endoscopic surgical therapy remains the mainstream treatment for patients enduring an unbearable course. Oral corticosteroid administration serves as an adjuvant therapy for first-line treatments.PMID:36884341 | DOI:10.1177/01455613231162226 (Source: Ear, Nose and Throat Journal)
Source: Ear, Nose and Throat Journal - March 8, 2023 Category: ENT & OMF Authors: Haoyuan Xu Huankang Zhang Wanpeng Li Chen Zhang Huan Wang Dehui Wang Source Type: research
Successful Treatment of Non-Langerhans Cell Histiocytosis with the MEK Inhibitor Trametinib: A Multicenter Analysis
Blood Adv. 2023 Mar 1:bloodadvances.2022009013. doi: 10.1182/bloodadvances.2022009013. Online ahead of print.ABSTRACTErdheim-Chester disease (ECD) and Rosai-Dorfman disease (RDD) are rare non-Langerhans cell histiocytoses (non-LCHs) for which therapeutic options are limited. MAPK pathway activation through BRAFV600E mutation or other genomic alterations is a histiocytosis hallmark and correlates with favorable response to BRAF inhibitors and the MEK inhibitor cobimetinib. However, there has been no systematic evaluation of alternative MEK inhibitors. To assess the efficacy and safety of the MEK inhibitor trametinib, we ret...
Source: Adv Data - March 1, 2023 Category: Epidemiology Authors: Ashley Aaroe Razelle Kurzrock Gaurav Goyal Aaron Michael Goodman Harsh Patel Gordon J Ruan Gary Ulaner Jason R Young Ziyi Li Derek Dustin Ronald S Go Eli L Diamond Filip Janku Source Type: research
Extranodal Rosai-Dorfman disease involving pulmonary artery detected by < sup > 68 < /sup > Ga-FAPI PET/CT and fused < sup > 68 < /sup > Ga-FAPI PET/MRI
Eur J Nucl Med Mol Imaging. 2023 Feb 28. doi: 10.1007/s00259-023-06167-7. Online ahead of print.NO ABSTRACTPMID:36853563 | DOI:10.1007/s00259-023-06167-7 (Source: Molecular Medicine)
Source: Molecular Medicine - February 28, 2023 Category: Molecular Biology Authors: Liang Zhao Yizhen Pang Long Sun Qin Lin Haojun Chen Source Type: research
Recurrent central nervous system Rosai-Dorfman disease with KRAS mutation: a case report
Rosai-Dorfman disease (RDD) is a rare, non-Langerhans cell histiocytosis of unknown etiology. we report a very rare case of recurrent central nervous system RDD with KRAS gene mutation and review the literatur... (Source: Diagnostic Pathology)
Source: Diagnostic Pathology - February 13, 2023 Category: Pathology Authors: Qingyang Wang, Hongxiang Ren, Liyuan Zheng, Juan Wang and Dingrong Zhong Tags: Case Report Source Type: research
OCT2 expression in histiocytoses
AbstractDiagnosis of histiocytosis can be difficult and one of the biggest challenges is to distinguish between reactive and neoplastic histiocytes on histology alone. Recently, OCT2 nuclear expression was reported in Rosai-Dorfman disease (RDD). Our purpose was to expand the testing of OCT2 on a broader variety of sporadic or H syndrome-related histiocytoses. Cases of histiocytoses were retrieved from the files of Ambroise Par é Pathology Department. All slides and molecular analyses were reviewed, and staining was completed with immunohistochemistry for OCT2. A total of 156 samples from different localizations were test...
Source: Virchows Archiv - February 9, 2023 Category: Pathology Source Type: research
