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Considering intracranial tumours, only few indications of protontherapy, such as chordoma, chondrosarcoma or uveal melanoma, are uniformly approved in the world. Other indications, excluding paediatric pathologies, are still debated. The aim of this article is to describe the rationale for the use of protonbeam irradiation for meningioma, pituitary adenoma, craniopharyngioma, paraganglioma, glioma, and schwannoma, and to inform the radiation oncologists if prospective studies or randomized studies are opened for inclusions. This article deals only with indications for adults.

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OBJECTIFS: The diagnosis of a pheochromocytoma or paraganglioma secreting during pregnancy is a rare and serious situation, involving maternal-fetal prognosis. The purpose of this case series is to discuss the management of these patients. METHODS: This is a retrospective study of cases of pheochromocytoma (n=2) or paraganglioma (n=2) managed during pregnancy between 2013 and 2020 in one center (Lille, France). RESULTS: We report four cases of patients with a diagnosis of pheochromocytoma or paraganglioma during pregnancy, at respectively 4, 28, 31 and 34 weeks of amenorrhea (AS). Their pregnancies were affected by a sudden onset of hypertension sometimes associated with headaches, sweating, and palpitations. All patients delivered by Caesarean section after calcium channel blocker impregnation, with a good outcome. Tumor removal took place at a distance from delivery for each patient. CONCLUSIONS: The therapeutic strategy includes antihypertensive treatment with calcium channel blockers or alphablockers and surgical curative treatment linked to gestational age. Multidisciplinary management as well as early diagnosis can improve the maternal-fetal prognosis. The preferred way of delivery is Caesarean section, but vaginal delivery can also be considered. Removal should ideally take place at a distance from the birth. The analysis of these cases has led to the development of a protocol for monitoring and management of parturients with diagnosis of pheochromocytoma or paraganglioma during pregnancy.

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INTRODUCTION: The paraganglioma of the cauda equina is a rare tumor, the diagnosis is morphological and the immunohistochemistry provides a definite diagnosis. The objective of our study is to specify the clinical signs, radiological and associated pathological criteria and to compare our data with those of the literature. PATIENTS AND METHOD: This is a retrospective study of nine cases of paragangliomas of the cauda equina diagnosed in our department from 2003 to 2018. The median age of the patients was 50 years-old with a male predominance (sex ratio: 3,5/1). All patients had preoperative magnetic resonance imaging (MRI) and surgery to remove the tumor. The diagnosis was performed after HES (Hematoxylin Eosin Saffron) and immunohistochemical sections examination. RESULTS: Radiculalgia was the chief symptom of these tumors. MRI showed an oval lesion uniformly enhanced by Gadolinium in the eight patients whose records were available. Histologically, the tumors had a lobular and trabecular pattern with neuroendocrine-like cells and a rich vascularization. By immunohistochemistry, the cells expressed chromogranin, synaptophysin and CD56. DISCUSSION AND CONCLUSION: Paragangliomas of the cauda equina are rare, benign tumors. Except for cases of secreting tumors, the preoperative diagnosis is difficult. MRI is useful and may reveal radiological features suggestive of these tumors. However, it is rare for the diagnosis to be made before surgery. The diagnosis is established by histological examination and immunohistochemical techniques must be used to confirm the diagnosis. The paragangliomas of the cauda equina are well encapsulated tumors whose complete excision is curative. When the excision is incomplete, treatment with radiotherapy is recommended. Long-term clinical and radiological monitoring is recommended because of the slow evolution of the tumor and the potential for recurrence.

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Phaeochromocytomas and paragangliomas (PPGL) are rare neuroendocrine tumors that arise from the adrenal medulla or sympathetic and parasympathetic ganglia. These tumors produce most often catecholamines in excess, causing hypertension and sometimes severe acute cardiovascular complications. The diagnosis is based on plasma or urines metanephrines measurements and on conventional and nuclear medicine imaging. Catecholamines-producing PPGL is very unlikely if levels are normal. The diagnosis of PPGL cannot be made without visualization of a tumor. Therapeutic management consists mostly of surgical excision, after drug preparation, and should be done in referral centers. About 40% of pheochromocytomas and paragangliomas occur in the context of an autosomal inherited syndrome, making genetic testing essential. The follow-up must be prolonged because a metastatic evolution or a recurrence can be observed in about 15% of the cases.

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Metastatic pheochromocytoma/paraganglioma (PPGL) represents a major clinical challenge due to limitations in accurate diagnostic tools and effective treatments. Currently, patients classified at high-risk by means of clinical, biochemical and genetic criteria, require a lifelong monitoring, while it remains difficult to determine the metastatic potential of PPGL only on the basis of histopathological features. Thus, tumor molecular markers that improve the risk stratification of these patients are needed. In the past few years, we have witnessed an unprecedented molecular characterization of PPGL, which led to the emergence of promising candidate biomarkers predictive of metastatic behavior. Here, we briefly discuss these breakthroughs and provide some insights for the prospective implementation of molecular markers of metastatic PPGL in the clinical setting in years to come.

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