When a Meningioma Isn't: Endoscopic Endonasal Orbital Decompression and Biopsy of Skull Base Rosai-Dorfman Disease Treated Previously with Empiric Radiation Therapy

Shah, Varun; Mohyeldin, Ahmed; London, Nyall R.; Fritz, Joel; Prevedello, Daniel M.; Carrau, Ricardo L.; Hardesty, Douglas A.

doi:10.1016/j.wneu.2019.12.032

Cited by 4 publications

(3 citation statements)

References 15 publications

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“…4). Out of the 53 cases studied, 10 displayed unusual symptoms 24,25,33,34,36,43,44,52,53 . Specifically, the symptoms related to the eyes were observed in 5 studies 24,33,34,43,53 , while ear-related symptoms were observed in 3 studies 24,34,36 and paresthesia was seen only in 1 study 44 .…”

Section: Resultsmentioning

confidence: 99%

Manifestations, Diagnosis and Treatment of Osseous Rosai-Dorfman Disease: A Systematic Review

Agarwal,

Abozeed

2023

Preprint

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BACKGROUND We hypothesize that lymphadenopathy and extraosseous manifestation affect the management protocol and our aim is to aid in development of standardized diagnosis and treatment protocols. METHODS PubMed and Google Scholar were searched from January 2012 through December 2022 using keywords Rosai-Dorfman, Rosai and Dorfman, and bone. Out of 98 researches, meta-analysis of 51 full-text studies involving 53 individuals with osseous RDD was conducted. RESULTS A significant association was found between lymphadenopathy with steroid therapy (OR: 20.125, 95%CI: 3.02, 134.09, P=0.0004). Extraosseous RDD showed significant association with steroid therapy (P=0.0145), T1-isointense MRI (OR: 0.173, 95%CI: 0.0304, 0.986, P=0.035), T1-hyperintense MRI (OR: 0.16, 95%CI: 0.0314, 0.815, P=0.019) and T2-hyperintense MRI (OR: 0.115, 95%CI= 0.0206, 0.6456, P=0.007). CONCLUSION Osseous RDD is rare and lacks standardized diagnosis and treatment protocols. Patients with lymphadenopathy were 9 times more likely to receive steroids, and those with extraosseous manifestations were less likely to undergo MRI. RDD is often not initially considered in differential diagnoses, leading to worsened conditions. We propose that further testing is required in affected individuals to develop standardized protocols.

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Section: Resultsmentioning

confidence: 99%

Manifestations, Diagnosis and Treatment of Osseous Rosai-Dorfman Disease: A Systematic Review

Agarwal,

Abozeed

2023

Preprint

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“…Thus, chemotherapy was an effective treatment for CNS-RDD. Studies showed that radiation therapy was also effective for the local control of CNS-RDD ( 30 , 31 ). However, none of the patients in our study received radiotherapy.…”

Section: Discussionmentioning

confidence: 99%

Rosai–Dorfman disease of the central nervous system: A clinical, radiological, and prognostic study of 12 cases

et al. 2022

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BackgroundRosai–Dorfman disease (RDD) is a rare benign non-Langerhans cell histiocytic proliferative disease. RDD with central nervous system (CNS) involvement (CNS-RDD) is extremely rare. Its etiology is unclear, and there are no consensus recommendations for its treatment. More studies are needed to elucidate the clinical and radiological manifestations and prognosis of CNS-RDD.MethodsFrom January 2012 to June 2022, 12 patients with CNS-RDD (intracranial or spinal) were retrospectively evaluated, including collecting clinical data, imaging data, and pathological findings; summarizing imaging characteristics; and conducting follow-up studies on CND-RDD patient treatment and prognosis.ResultsTwelve CNS-RDD patients (nine male and three female patients, aged 12–67 years) were enrolled in this study. Nine patients represented convex and/or skull base RDD (eight with edema, six with lobulation and/or pseudopodium sign, four with multiple intracranial lesions), two patients had parenchymal RDD, and one patient had spinal cord subdural lesions. Symptoms of patients would vary according to the locations of the lesion, including but not limited to headaches, dizziness, seizures, cranial nerve dysfunction, and visual impairment. The immunohistochemistry of RDD showed positive expression of S100 and CD68 but not CD1a. Total resection (n = 7), subtotal resection (n = 3), partial resection (n = 1), and stereotaxic biopsy (n = 1) were achieved, respectively. A combination of chemotherapy plus steroid therapy was performed on two patients (relapsing case and residual lesion) and showed a remarkable effect.ConclusionCNS-RDD, as a rare disease, presents a significant diagnostic challenge for clinicians. Solitary CNS-RDD are easily misdiagnosed as meningioma. However, when the MRI imaging of the disease represents dura-based masses with significant edema, homogeneous enhancement, lobulation, and/or pseudopodium sign, we should consider it might be the CNS-RDD. Surgery is an important and effective therapy for CNS-RDD. Steroids and chemotherapy are safe and effective for the postoperative treatment of relapsing cases or residual lesions.

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“…El tratamiento generalmente no se inicia hasta que el paciente esté sintomático o curse con progresión de la enfermedad 3 . La resección de la lesión puede ser curativa para la enfermedad unifocal y la reducción del volumen de la lesión puede justificarse en casos de obstrucción de la vía aérea superior, compresión de la médula espinal o lesiones grandes que causen compromiso del órgano afectado 1,3,8 . En la enfermedad recurrente o refractaria puede ser necesario utilizar terapia sistémica o radiación.…”

Section: Discussionunclassified

Rosai-Dorfman extranodal: una enfermedad rara con manifestación clínica común

Fragozo-Ramos

Ortiz-Marin

Corzo-Pacheco

et al. 2020

Rev Esp Casos Clin Med Intern

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La Histiocitosis sinusoidal con linfadenopatía masiva o enfermedad de Rosai-Dorfman (ERD), es un trastorno proliferativo benigno de los histiocitos. Afecta con mayor frecuencia a niños y adultos jóvenes, pero puede ocurrir a cualquier edad. La enfermedad es más común en hombres e individuos de ascendencia africana. Es una entidad infrecuente con gran variedad de manifestaciones clínicas. Se presenta un caso de ERD extranodal de diagnóstico complejo con compromiso óseo y del Sistema Nervioso Central (SNC). La biopsia fue concluyente remarcando los aspectos distintivos de esta patología.

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When a Meningioma Isn't: Endoscopic Endonasal Orbital Decompression and Biopsy of Skull Base Rosai-Dorfman Disease Treated Previously with Empiric Radiation Therapy

Cited by 4 publications

References 15 publications

Manifestations, Diagnosis and Treatment of Osseous Rosai-Dorfman Disease: A Systematic Review

Manifestations, Diagnosis and Treatment of Osseous Rosai-Dorfman Disease: A Systematic Review

Rosai–Dorfman disease of the central nervous system: A clinical, radiological, and prognostic study of 12 cases

Rosai-Dorfman extranodal: una enfermedad rara con manifestación clínica común

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