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Nasopharyngeal angiofibroma refers to a locally aggressive benign vascular tumor composed of vasogenic and myofibroblastic elements, representing between 0.05% and 0.5% of all head and neck tumors. Here, we report a case of a 21-year-old patient with no particular personal or familial pathological history, who presented with recurrent epistaxis and anterior rhinorrhea and headaches for 1 year. Nasofibroscopy revealed a mass filling the entire left nasal cavity, streaked with blood. A face MRI revealed a highly vascularized and lytic nasopharyngeal tumor infiltrating the deep left lateral facial spaces with the beginning of endocranial infiltration, supplied by the homolateral maxillary artery, initially suggestive of a nasopharyngeal fibroma. Preoperative arteriography showed a voluminous vascular blush at the arterial time to the nasopharyngeal projection with branches arising from the left external and internal carotid arteries and multiple arterial shunts (between the left internal and maxillary carotid arteries), thus contradicting embolization. Early diagnosis is important as it is associated with a high risk of morbidity, but advances in imaging and surgical treatment methods have modified the sites associated with high morbidity risk.

Hirayama disease is a rare non-progressive spinal muscular atrophy related to flexion movements of the neck. It is considered a benign motor neuron disorder with a stationary stage after a progressive course. We report a case of an 18-years old male with a history of asymmetric weakness and amyotrophy of the distal right upper extremity, suggestive of Hirayama disease. Magnetic resonance imaging (MRI) of the cervical spine was obtained both in flexion and neutral position. MRI showed loss of cervical lordosis with focal areas of lower cervical cord atrophy with increased T2 signal intensity of the spinal cervical cord in a neutral position and anterior displacement of the detached posterior dura from the underlying flexion position.

Rosai-Dorfman disease (RDD) is a rare histiocytic disorder characterized by the proliferation of non-Langerhans cells within the lymph nodes, and occasionally in extranodal sites. We report a case of RDD presenting as a left jugal swelling in a 43-year-old male. Imaging studies revealed a poorly defined lesion infiltrating the subcutaneous and muscular planes, as well as the maxillary bone. Biopsy confirmed the diagnosis of RDD. Treatment included surgical excision, followed by low-dose radiation therapy. The patient remained asymptomatic during a one-year follow-up.

Cutis Verticis Gyrata-Intellectual Disability (CVG-ID) syndrome is a rare neurocutaneous syndrome characterized by intellectual disability and scalp folds, furrows that are typically absent at birth and are first noticed after puberty. First reported in 1893, the syndrome was mainly identified in subjects living in psychiatric institutions. Most patients were reported in the literature during the first half of the 20th century. CVG-ID is now a less reported and possibly under-recognized syndrome. Here, we report a patient with CVG-ID that was diagnosed using the novel approach of magnetic resonance imaging.

Intracranial epidermoid cysts are rare, congenital, benign tumors, usually have no clinical implications and remain asymptomatic for years. The diagnosis of pineal cyst is usually established by MRI with defined radiological criteria to distinguish benign pineal cyst from tumors of this area. In most cases no treatment and no follow up is necessary.

Neurofibromatosis (NF) is a neurocutaneous disorder, which involves many organs in the body. There are two types: NF-1 and NF-2. Orbital manifestation is a rarity in NF-1, and it involves dysplasia of the sphenoid bone resulting in herniation of the temporal lobe and subarachnoid space into the orbit culminating in exophthalmos. In this presentation, we report the case of 18 years old boy, admitted for exophthalmosis dating back to the age of 5 years. we performed a cerebral and oculo-orbital MRI which has objectified a sphenoid agenesis (confirmed by CT scan) associated with a neurofibroma of the external canthus of the right eye, we have also objectified multiple signal abnormalities of cerebral white matter corresponding to FASI (focal areas of signal intensity), a more detailed clinical examination was carried out a posteriori which revealed multiple café-au-lait spots, all of these lesions suggestive of neurofibromatosis with cerebral and spheno-ocular involvement.

Langerhans cells histiocytosis (LCH) is a disease caused by the proliferation and abnormal accumulation of Langerhans cells in different tissues and organs. It is a rare disease that still hides many of its mysteries as to its etiology and pathophysiology. The cerebral localization remains exceptional and dominated by hypothalamic-pituitary axis involvement. We report a case of a 20-year-old patient followed for acquired central diabetes insipidus with Panhypopituitarism and multiple dermatological lesions, who recently had visual acuity decline with progressive cerebellar syndrome in a state of apyrexia. Brain MRI showed multiples scattered plaques and nodular lesions involving the periventricular white matter, the basal ganglia, cerebellum, brainstem and cervical medulla. The two diagnoses suggested in this clinical presentation sarcoidosis with pituitary localization and cerebral Langerhans cells histiocytosis. A skin biopsy confirmed the diagnosis of langherans cell histiocytosis.

Resume Les tumeurs intraventriculaires sont des tumeurs rares, d’etiologies variables et le plus souvent benignes. L’approche etiologique se fait en fonction de l’âge du patient, de la localisation et des caracteristiques semiologiques de la tumeur. L’imagerie, notamment l’IRM, est l’examen de choix pour le diagnostic positif et le bilan d’extension. L’objectif de ce travail est de preciser l’apport de l’imagerie dans la caracterisation semiologique des tumeurs intraventriculaires et la gamme diagnostique a evoquer en fonction du siege et de l’aspect tumoral.