Nervous System Rosai-Dorfman Disease Research Articles

Recurrent central nervous system Rosai-Dorfman disease with KRAS mutation: a case report

BackgroundRosai-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 literature to improve our understanding of this disease.Case presentationA 19-year-old male patient was admitted to our hospital for headache. Cranial magnetic resonance imaging revealed a mass of abnormal signal shadows in the prepontine cistern. The mass was surgically removed and the patient was consequently diagnosed with intracranial Rosai-Dorfman disease. Seven months later, pathological examination confirmed that the RDD had recurred. Next-generation sequencing found KRAS mutation in exon 4 (C.351A > C. P. K117n).ConclusionRDD of the CNS has no distinct clinical manifestations and imaging characteristics, and the final diagnosis should be based on the results of the pathological examination. Although RDD is not currently classified as a neoplastic disorder, some evidence of clonality has changed our understanding of it. Follow up examinations over a long period are necessary to determine the efficacy of treatment.

Management of central nervous system Rosai-Dorfman disease: A single center treatment experience

Rosai-Dorfman disease (RDD) is an idiopathic histiocytic proliferation disease with various clinical manifestations. A retrospective study of patients with pathological diagnosed RDD primarily involved in the central nervous system was conducted from January 2011 to December 2020 at a tertiary center. The clinical profile, imaging, and treatment data were collected. There were 16 male and 5 female patients with RDD-CNS. The patients were aged from 6 to 68 years with a median of 37 years. Of these 21 patients, 15 presented with intracranial RDD and 6 with spinal RDD. The main symptoms of RDD-CNS included headache, epilepsy, and neurological deficits. 76.19% (16/21) of the patients showed dura-based, homogeneous enhancement lesion on magnetic resonance imaging (MRI). Twenty patients received surgery as first treatment, and one patient received biopsy after steroid therapy. Total lesion resection was achieved in 42.9% (9/21) of the patients, subtotal resection in 47.6% (10/21), and biopsy in 0.9% (2/21). The symptoms were alleviated or stayed stable. Some RDDs (80%, 4/5) in the skull base had some complications. The patients were followed up for 11–108 months with a median duration of 47 months. Lesion progression or recurrence was found in two patients. The various clinical manifestations, as well as the dura-based and homogenous enhancement imaging profiles of RDD-CNS patients pose a great diagnostic challenge for clinicians. Surgery is effective for RDD-CNS requiring treatment. Medical therapy and radiotherapy would be feasible as noninvasive treatments, varying degrees of efficacy. The overall prognosis of RDD-CNS is acceptable. Periodic long-term follow-up is necessary.

Intracranial And Intraorbital Rosai Dorfman Disease

Rosai Dorfman disease is a rare histiocytic disorder. It is also known as Sinus Histiocytosis. It is with massive lymphadenopathy involves an overproduction of a type of white blood cell. The disease is rarely associated with intracranial and intraorbital involvement. Intracranial Rosai-Dorfman can mimic meningioma. Other pathologies also underline its pathologies. Here, we report a nine-year-old boy with a history of proptosis of the right eye and presenting with multiple skull lesions. Histopathological study revealed Sphenopetroclival lesion, which features that of Rosai Dorfman Disease. His MRI scan of the brain was taken, which showed evidence of right optic nerve meningioma with sella and suprasellar extension, causing severe proptosis. The child underwent right frontotemporal craniotomy with petrosectomy and Transylvanian, subtemporal approach to multicompartmental Rosai-Dorfmans lesion. After four months, the patient had a recurrence of the disease on which chemotherapy and steroids were started, which also did not show much response while taking an MRI scan. A corticosteroid is a useful option in the Central Nervous System Rosai Dorfman disease treatment. But this patient showed a negative outcome to the treatment.

FDG-PET/CT image of a cystic central nervous system Rosai-Dorfman disease.

FDG-PET/CT image of a cystic central nervous system Rosai-Dorfman disease.

Diagnosis and management of Rosai–Dorfman disease involving the central nervous system

Introduction: Rosai–Dorfman disease is a benign non-neoplastic proliferative disorder of histiocytes originally described in the cervical lymph nodes. Extranodal sites were later recognized, and by 1990, they were shown to represent over 40% of cases; however, central nervous system involvement is still considered rare. We review the literature, which shows a steady increase in reports of Rosai–Dorfman disease involving the brain and/or spine.Methods: A literature search was performed for the period 1969–2008, using multiple search engines, with keywords Rosai–Dorfman disease, central nervous system Rosai–Dorfman disease and sinus histiocytosis with massive lymphadenopathy.Results: By December 2008, 111 cases of Rosai–Dorfman disease involving the central nervous system had been reported in the literature, including our cases. In the central nervous system, Rosai–Dorfman disease is ubiquitous. Although it is characterized by unique, indeed pathognomonic, histopathological cytoarchitecture, it may be mistaken for many other neoplastic and inflammatory histioproliferative diseases. Surgical resection with post-operative corticosteroids remains the treatment of choice.Conclusions: Rosai–Dorfman disease involving the central nervous system appears to be more common than previously thought and may well assume a more prominent place in the differential diagnoses of dural-based lesions. Expert awareness of the characteristic histopathology and immunohistochemistry of the disease is required for accurate diagnosis.

CT and MRI analysis of central nervous system Rosai-Dorfman disease

Objective To study the CT and MRI imaging features of central nervous system Rosai-Dorfman disease and to enhance knowledge and differential diagnostic ability for central nervous system Rosai-Dorfman disease.Methods The CT and MRI imaging appearances in 4 cases of pathologically proven Rosai-Dorfman disease were retrospectively evaluated and the literature of central nervous system Rosai-Dorfman disease were reviewed.Results Two cases had cranial CT scans,4 cases had cranial MRI scans,On CT scans,cerebral edema was demonstrated in one case and the other case was normaL MRI scans showed the lesions were solitary in saddle area in 3 cases,and multiple in anterior cranial fossa in 1 case.The lesions exhibited iso- to hypointensity on both T1WI and T2WI images.Following intravenous injection of contrast medium,ring-like enhancement was seen in 2 cases and homogeneous enhancement in 1 case.Nodular enhancement was seen in the case of multiple lesions in the anterior cranial fosse.All lesions were dural-based.Conclusions In patients with fever,headache,elevation of the erythrocyte sedimentation rate (ESR) and a polyclonal increase in .γ-globulins,the possibility of central nervous system Rosai-Dorfman disease should be considered when single or multiple dural-based mass lesions,especially in sellar region,were identified by CT and MRI. Key words: Histiocytesis,sinus; Central nervous system; Magnetic resonance imaging; Tomography,X-ray computed