Spontaneous subdural hematoma associated with microcystic meningioma: first case report in the literature.

Background: Non traumatic subdural hematomas are rare, especially those associated with intracranial meningiomas. Among the most common meningiomas associated with spontaneous bleeding are angioblastic and malignant meningioma variants. The pathophysiological mechanisms of this association are not yet fully understood. The association of chronic subdural hematoma with microcystic meningioma histological subtype has not yet been described in the literature. Case report: The authors present a case report of a patient with a spontaneous non traumatic chronic subdural hematoma associated with a microcystic subtype grade I meningioma of the parietal convexity. Epidemiological, etiology, natural history, pathophysiology, risk factors of bleeding and treatment options are reviewed. Conclusion: Spontaneous subdural hematomas associated with meningiomas are rare, specially related to the microcystic variant of meningioma. Careful pre-operative consideration of specific anatomy and pathophysiological features are paramount to their full treatment.

Olfactory groove monophasic sinovial sarcoma and von Recklinghausen's disease: A case report and literature review.

Background: Soft-tissue sarcomas are a rare and diverse group of neoplastic lesions. They represent only 1% of malignant tumors in adults and 15% in children. Synovial sarcoma (SS) is a type of soft-tissue sarcoma, accounting for 5-10% of cases, and commonly affecting extremities. Diagnosis, treatment, and prognosis remain challenging especially when localized in uncommon areas, such as intracranial lesions. Case Description: A 13-year-old male patient with a clinical history of neurofibromatosis Type I (NF1) presenting holocranial headache with jet vomiting and apathy 2 days before admission, without neurological deficits and/or focal findings. On magnetic resonance imaging: an extra-axial infiltrative lesion with contrast uptake at the base of the skull in the olfactory groove topography. After total tumor resection, the anatomopathological examination showed monophasic SS. The patient returned after 6 months with similar symptoms, and the lesion recurred and was reoperated. Unfortunately, 7 months after the second surgery, the patient died. Conclusion: SS can occur extraarticulously and with a variable clinical presentation and poor prognosis despite adjuvant therapies with radiotherapy and chemotherapy. In individuals with clinical history of NF1, there is still no direct correlation between the two manifestations, although current descriptions are suggestive of a possible interaction.

Neurocytoma mimicking macroadenoma.

BACKGROUND: Intraventricular and extraventricular central neurocytomas (CN) are equally frequent among 20-40-year-old men and women. However, sellar and suprasellar extraventricular CN are extremely rare, with only 12 reported cases. CASE DESCRIPTION: The authors report the case of a Brazilian 27-year-old man who presented with progressive vision loss during the last 4 years and serious bilateral keratoconus. We also review the epidemiological, clinical, radiological, pathological, and treatment features of the 12 reported cases. The patient developed left amaurosis and right temporal hemianopsia after undergoing bilateral corneal transplantation, which was detected during campimetry testing, and subsequently underwent magnetic resonance imaging, which revealed a huge hypophyseal tumor. Endocrinological evaluation revealed complete loss of pituitary function. The patient was referred to our department and underwent a two-step surgery (using transsphenoidal approach and cranio-orbital zygomatic approach) based on the diagnosis of an extraventricular central nervous system neurocytoma. Tumor removal was successful, and the patient was discharged at 3 weeks after admission to our department. CONCLUSION: Although extraventricular neurocytomas of the brain are rare, careful preoperative consideration of its anatomy, pathophysiological features, and radiological features can enhance the treatment outcomes.

Cervicomedullary primitive neuroectodermal tumor of the spine: Case report.

BACKGROUND: Intramedullary primitive neuroectodermal tumors (PNETs) are tumors found rarely in the cervical region, with only five such cases described in the literature. The available literature contains only one report regarding cervicomedullary junction PNET. CASE DESCRIPTION: The authors present a case report of a 45-year-old male patient who had undergone urgent hospitalization owing to progressive tetraparesis and subtle impairment of respiratory function. He underwent magnetic resonance imaging, which showed an extensive enhancing cervical intramedullary tumor extending from C5 to the bulbar region. Since he developed severe impairment of respiratory function, he required tracheostomy. He then underwent microsurgery 2 days after his admission, and a partial tumor resection was performed. The pathological diagnosis of PNET of the cervicomedullary junction (CMJ) was made. He had slight worsening of strength after surgery with subsequent deterioration over the next 3 weeks. The tumor displayed aggressive growth; thus, radiotherapy was indicated. Unfortunately, he developed severe febrile neutropenia and died after 2 weeks of radiotherapy. Given the rarity of the condition, we wish to review the epidemiology, pathophysiology, and treatment options of his population. CONCLUSION: Intramedullary PNETs of the cervical spine and CMJ are exceedingly rare in adults; treatment of such patients remains a challenge, despite the modern neurosurgical armamentarium that is available.