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Purpose: Central nervous system (CNS) metastasis from cholangiocarcinoma (CCA) are extremely rare and associated prognosis is poor. The involvement of the CNS by metastatic CCA may discourage any further treatment; however, data from the literature are discordant, due to recent reports of exceptionally long follow-up after surgical resection of a brain metastasis. Material and Methods: Electronic databases, such as PubMed/MEDLINE and Google Scholar, were analyzed for studies published up to October 2018 using the search term "cholangiocarcinoma and central nervous system metastasis or brain metastasis". Results: We found a total of 18 studies cited in the literature of the 30 year span analyzed, and we added a new case we treated at our Institution, reaching a series of 32 patients. Among these, 7 patients had leptomeningeal dissemination and 25 presented solid CNS metastasis. We analyzed the treatment options and the outcomes, addressing also histopathological insights on tumoral markers possibly involved in the mechanism of metastases of cholangiocarcinomas Conclusions: According to the literature data, the outcome remains poor, particularly for those with leptomeningeal diffusion. Nevertheless, long term follow-up is reported in case of surgical resection of CNS metastasis, when there is a good control of the primary tumor. Actually, the majority of patients are often in advanced state of disease at diagnosis and not suitable for initial resective procedure; in these cases neo adjuvant and adjuvant therapies have provided a slight improvement of the outcome. [ABSTRACT FROM AUTHOR]

Background Acute hemorrhagic occurrence of pilocytic astrocytomas is extremely rare but has become increasingly recognized and often presents in clinically emergent situations. The precise significance of hemorrhage in these tumors and its pathogenetic mechanisms have not yet been defined. Nothing is known about the natural history of bleeding pilocytic astrocytomas. Case Description A 21-year-old man presented with acute biventricular hydrocephalus for repeat bleeding of a midbrain lesion that had been diagnosed 18 years earlier after a similar acute hemorrhagic episode. The patient had been initially treated conservatively, considering the high risk of surgery. He underwent urgent biventricular external shunt surgery and, subsequently, total removal of the lesion. Histopathological analysis revealed pilocytic astrocytoma. Including the present case, 59 cases were analyzed and reviewed, including 5 cases with a pilomyxoid variant. Different pathogenetic mechanisms have been proposed, including histological characteristics, rupture of the bridging veins torn by the neoplastic mass, and vascular instability determined by overexpression of vascular endothelial growth factor. Conclusions To the best of our knowledge, the presented case is completely unique owing to the long-term observational follow-up data available. The hemorrhagic potential did not decrease over time, representing a specific feature of this tumor. The occurrence of bleeding might not reflect either malignancy or aggressiveness. However, the worse prognosis is related to the clinical behavior, with life-threatening complications often associated. Surgery remains the standard treatment and will influence the final prognosis. [ABSTRACT FROM AUTHOR]

Background context Cauda equina paragangliomas (CEPs) are rare neuroendocrine tumors. The difficulty in differential diagnosis with other tumors of this region may be misleading for surgical planning and prognostic expectations. Purpose To report on a rare case of CEP and review the most current information regarding the diagnosis, treatment options, and outcomes. Study design Case report and literature review. Patient sample One patient affected by CEP. Methods We report on a 33-year-old woman with a 2-month history of worsening low back pain, aggravated by sitting, bending, and coughing. Neurological examination revealed normal power and muscular tone, no sensory or sphincter abnormality, and normal reflex. Magnetic resonance imaging of the lumbar spine demonstrated an intradural extramedullary lesion at L3, with homogeneous contrast enhancement and hypointense punctate foci. The patient underwent an L3 laminectomy and tumor removal. Relevant articles covering CEPs from 1970 to the present were reviewed. Results The histopathological examinations described paraganglioma features. The postoperative course was uneventful, and all the symptoms resolved, with no tumor recurrence after 3 years' follow-up. Conclusions Cauda equina paragangliomas are rare, benign, and slow-growing tumors. Except for its secreting tumor characteristics, preoperative CEP diagnosis is very difficult. Magnetic resonance imaging is important and may suggest specific radiological features for these tumors; however, these are only relative, and it is rare that diagnosis is made before surgery. Diagnosis is established by histological examination and electron microscopy, and immunohistochemical techniques must be used to achieve a correct diagnosis. Cauda equina paragangliomas are well-encapsulated tumors that may be cured by surgery alone, whereas radiotherapy is reserved for incompletely resected tumors. Overall, prolonged postoperative observation is mandatory because of the slow tumor evolution and the possibility of tumor relapse even up to 30 years after surgery. [ABSTRACT FROM AUTHOR]