Rosai-Dorfman disease (RDD) is an idiopathic nonneoplastic lymphadenopathy disorder which is characterized by lymph node enlargement, but it may also present

primarily involving a variety of extranodal sites, including central nerves system and craniospinal axis. This study reports five cases of craniospinal RDD, with review of epidemiology, clinical presentation, imaging, and histopathological features with current management strategies.

Five cases of RDD are diagnosed at Hamad General Hospital, Qatar, during 2013-2018. Two cases had dural-based cranial lesions with overlying cranial involvement while three cases were having extradural thoracic spine lesions. All cases underwent surgical intervention and confirmed by histopathology.

Craniospinal RDD is a rare clinical presentation and poses significant diagnostic challenges preoperatively due to its similarity with other neoplastic or inflammatory diseases. learn more Surgical option to remove compressive neural pathology provides a good clinical outcome with no recurrence in long-term follow-up.

Craniospinal RDD is a rare clinical presentation and poses significant diagnostic challenges preoperatively due to its similarity with other neoplastic or inflammatory diseases. Surgical option to remove compressive neural pathology provides a good clinical outcome with no recurrence in long-term follow-up.

Myxopapillary ependymoma occurs more frequently in adults, but is found in the first two decades of life in around 8-20% of patients. Tumors are usually benign with low likelihood for dissemination.

We describe a case of a 13-year-old boy who presented with progressive kyphosis and bilateral weakness of the lower limbs. MRI shows a thoracolumbosacral intradural tumor with invasion of sacral neural foramina and dissemination to the cervicothoracic region. The patient received T10-L5 laminectomy with subtotal tumor resection. Pathological examination revealed myxopapillary ependymoma. After surgical resection, the patient underwent physical therapy with whole spinal radiotherapy for disease control.

Spinal myxopapillary ependymomas are usually benign and slow-growing tumors. This case illustrates an extensive and disseminated myxopapillary ependymoma.

Spinal myxopapillary ependymomas are usually benign and slow-growing tumors. This case illustrates an extensive and disseminated myxopapillary ependymoma.

Osteochondromas are commonly occurring benign bone tumors which may be either a solitary lesion or occur due to association with hereditary multiple exostoses (HMEs). There have been several reported cases of spinal osteochondromas, but intracranial lesions are rare.

A 51-year-old male with a history of multiple osteochondromas presented with myelopathy. He had an exostosis arising from the foramen magnum causing compression of the cervical spinal cord that was successfully removed. Genetic testing revealed that he had HMEs.

Osteochondromas of the skull are extremely rare. However, parts of the foramen magnum ossify in cartilage and can give rise to an osteochondroma. Here, we present a patient with HMEs who developed cervical myelopathy due to an osteochondroma arising from the foramen magnum. Due to the cartilaginous ossification of the foramen magnum, clinicians should be aware that osteochondromas can occur in this location and potentially give rise to cervical myelopathy.

Osteochondromas of the skull are extremely rare. However, parts of the foramen magnum ossify in cartilage and can give rise to an osteochondroma. Here, we present a patient with HMEs who developed cervical myelopathy due to an osteochondroma arising from the foramen magnum. Due to the cartilaginous ossification of the foramen magnum, clinicians should be aware that osteochondromas can occur in this location and potentially give rise to cervical myelopathy.

Angiomatoid fibrous histiocytoma (AFH) is a rare low-grade soft-tissue tumor that typically arises from the deep dermal and subcutaneous tissue of the extremities in children and young adults. Intracranial AFH is exceedingly rare, and only four cases of primary AFH tumors have been reported to date.

A 43-year-old male presented to our hospital with headaches, vision changes, and a known brain tumor suspected to be an atypical meningioma. After undergoing craniotomy for resection of the mass, the immunomorphologic features of the resected tumor showed typical features of AFH with ESWR1 (exon7) – ATF1 (exon 5) fusion.

AFH is a difficult tumor to diagnose with imaging and histologic studies. Thus, further knowledge is necessary – particularly of intracranial cases – to aid clinicians in its diagnosis and management.

AFH is a difficult tumor to diagnose with imaging and histologic studies. Thus, further knowledge is necessary – particularly of intracranial cases – to aid clinicians in its diagnosis and management.

Cavernous malformations prevalence ranges from 0.4 to 0.6% and accounts for 5-15% of all central nervous system vascular malformations. Pineal cavernomas constitute <1% of all locations published in the literature, with a total of 26 cases reported, only 5 regarding the pediatric population until 2020. Overall annual hemorrhage rate is 2.4%. Symptoms are often due to hydrocephalus and intracranial hypertension.

We report a case of a 5-year-old child with visual disturbances, headache, and progressive neurologic deterioration. MR showed a lesion in the pineal region and triventricular hydrocephalus. She was submitted to endoscopic third ventriculostomy and total excision of the lesion by the infratentorial supracerebellar approach a few days later. Histopathological examination confirmed a pineal cavernous malformation. The patient returned to her normal life without any neurologic deficit and a normal development.

The ideal treatment is primary lesion removal; however, due to the infrequency and because it is a curable lesion, studies seeking to deepen the knowledge of this disease are considered relevant.

The ideal treatment is primary lesion removal; however, due to the infrequency and because it is a curable lesion, studies seeking to deepen the knowledge of this disease are considered relevant.