Rosai-Dorfman Disease involves histiocytic proliferation of the lymphatic system. Extranodal disease involving the central nervous system is uncommon. Furthermore, the combination of this disease entity with Langerhans cell histiocytosis is an even rarer phenomenon that has only recently been highlighted.A young male, who had previously undergone surgical excision of a skull lesion that was reported as Langerhans histiocytosis presented with an intracranial lesion mimicking a meningioma. Histopathology of the lesion was reported as being consistent with Rosai-Dorfman disease and the patient is currently undergoing chemotherapy. This is only the second report of the co-occurrence of Langerhans histiocytosis and Rosai-Dorfman disease in the cranium and intracranial cavity. The possibility that the diseases represent different spectra of the same underlying pathology is one that merits more detailed analysis, especially at the genomic level.KEywords: Rosai-Dorfman disease, Intracranial, Langerhans cell histiocytosis, Co-Occurrence, Skull, Eosinophilic granuloma ÖZ Rosai-Dorfman Hastalığında lenfatik sistemde histiyositik proliferasyon görülür. Merkezi sinir sistemini tutan ekstranodal hastalık nadirdir. Ayrıca bu hastalığın Langerhans hücreli histiyositoz ile kombinasyonu ancak yakın zamanlarda vurgulanmış olan daha da nadir bir fenomendir.Daha önce Langerhans hücreli histiyositoz olarak bildirilen bir kafatası lezyonunun cerrahi eksizyonu yapılmış genç bir erkek, menenjiyoma benzeyen intrakraniyal bir lezyonla geldi. Lezyon histopatolojisi Rosai-Dorfman hastalığı ile uyumlu olarak bildirildi ve hasta halen kemoterapi almaktadır.Bu olgu, kraniyum ve intrakraniyal boşlukta Rosai-Dorfman hastalığı ve Langerhans histiyositozunun birlikte görüldüğü sadece ikinci olgu raporudur. Hastalıkların aynı patolojinin farklı spektrumlarını temsil etme olasılığı özellikle genomik seviyede daha ayrıntılı incelenmelidir.
Intracerebral hematoma occurs in about 35/100,000 population and the incidence is likely increase over the next few decades as the population ages. The most common causes are hypertension and amyloid angiopathy. Bleeds due to these two causes are classified as primary while all other causes, such as AVM bleeds, coagulopathies, and so on, are classified as secondary. Primary tissue damage due to the intracerebral hematoma is followed by edema, neuronal damage, and secondary damage due to cellular breakdown. Basal ganglia are the most common site of intracerebral hemorrhage, accounting for nearly 50% of cases. CT scan, CT angiogram, DSA, and MRI are the investigations of choice. The initial management is medical, with control of blood pressure and antiedema measures forming the mainstay of treatment. Surgical option includes external ventricular drainage, endoscopic evacuation of hematoma, craniotomy and evacuation of hematoma, and decompressive craniectomy and is usually reserved for patients who deteriorate while on treatment.
We present the case of a 22-year-old who developed severe neck pain within two weeks after undergoing posterior fossa decompression for a symptomatic Chiari I malformation. A diagnosis of cerebellar ptosis was made after magnetic resonance imaging (MRI) and he underwent a partial cranioplasty, following which his symptoms resolved. The pathology, diagnostic criteria, and management options are discussed.
Background?Phyllodes tumor or cystosarcoma phyllodes is a rare tumor that accounts for less than 1% of breast lesions. Incidence of these lesions metastasizing to distant sites is very low and only a few cases of metastases to central nervous system have been reported. The management of a case with cervical spine metastasis is described and current literature has been reviewed. Case Description?A 32-year-old woman who underwent excision of cystosarcoma phyllodes of the right breast presented with neck pain. Evaluation showed destruction of the C3?C4 vertebrae. Anterior decompression with anterior and posterior fusion was done, followed by radiotherapy. Conclusion?Spinal metastasis from cystosarcoma phyllodes is extremely rare, but should be kept in mind when a patient presents with symptoms suggestive of a spinal problem. Surgical management of these tumors may enhance the quality of life of these patients.
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