A 35-year-old female was admitted to our institution because she had been suffering from progressive headaches for about 10 years – referred as debilitating in the past 6 months – which worsen with emotions like rage or concern, associated with blurred vision and tinnitus. Mini-mental state examination (MMSE) gave a score of 23.\nHer clinical history was positive for acute lymphoblastic leukemia, diagnosed in 1990 and treated with AIEOP 8703 protocol (cobalt radiotherapy [RT] in pediatric age and intrathecal chemotherapy with IV vincristine, IV daunorubicin, prednisone, methotrexate intrathecal, and IM L-asparaginase). In 1995, she was declared in complete remission. A right oophorectomy at the age of 9, caused by ovarian torsion, was also reported. In 2000, she underwent brain magnetic resonance imaging (MRI) that did not find pathological alterations. In 2011, she underwent in vitro fertilization, which unfortunately failed.\nThe patient underwent in-depth investigation that documented cerebral meningiomatosis, characterized by 5 meningiomas, <3 cm each, located at the right cerebral middle fossa, at the border of the right tentorium, at the torcula, at the middle third of the superior sagittal sinus (SSS), and at the left Sylvian fissure; a T2-weighted brain MRI sequence documented a typical bilateral enlargement of optic nerve sheath, related to intracranial hypertension; of notice, ventricles were not enlarged, but they presented slit-like aspect []. Considering that the tentorial meningioma was compressing the Sylvian aqueduct, an MRI with cerebrospinal fluid (CSF) flowmetry was performed revealing an irregular pattern at the level of Sylvian aqueduct []; cerebral MR venography showed normal venous flow at the level of the SSS, due to the fact that the meningiomas were of little size and in T1-weighted gadolinium-enhanced images, there was no invasion of the SSS. Initially, the patient underwent medical therapy based on acetazolamide, without clinical benefit. Then, the patient underwent a 72 h spinal CSF drainage connected to LiquoGuard® system that showed elevated intracranial pressure (ICP) values, ranging between 13 and 21 mmHg/24 h with a mean value of 17 mmHg. Subsequently, she reported a transitory relief of symptoms with a corresponding decrease in ICP equal to a mean value of 13 mmHg. The patient was then addressed to the ophthalmologist, who documented a bilateral papillary stasis. At the beginning, a diagnosis of pseudotumor cerebri was made but it was object of controversies during multidisciplinary oncologic discussions. We repeated brain MRI scan, fundus oculi examination, and we performed PET scan with gallium to confirm the meningiomatosis hypothesis, with the plan to perform Gamma Knife treatment to control at least some of the meningiomas.\nIn our opinion, the patient was not affected by pseudotumor cerebri due to the normal global brain anatomy with good representation of the cisterns and sulci and the very high ICP values. Our hypothesis was that the absence of hydrocephalus was related to previous RT and intrathecal chemotherapy in pediatric age and that the tentorial meningioma causes compression of the Sylvian aqueduct; we elaborated two options for a multimodal treatment strategy: (a) surgical resection of the tentorial meningioma and Gamma Knife treatment to control the other lesions, with ventriculoperitoneal shunt (VPS) to be considered in a second surgical time after clinical postoperative evaluation and (b) VPS to resolve intracranial hypertension and radiosurgery to control mainly the tentorial meningioma and avoid further growth with associated compression of the brainstem. In agreement with the patient, we decided to perform the latter option. First, she underwent Gamma Knife treatment of the tentorial meningioma because the presence of the ventricular catheter could cause artifacts and negatively affect the accuracy of radiosurgery planning. The following day, with the guidance of the neuronavigation, we introduced the ventricular catheter at the right Kocher’s point and connected the distal catheter with a single route, without the typical skin interruption at the cervical level, to the peritoneum in the epigastric-suprahepatic site. A programmable valve with virtual off option was implanted and set at the 5th setting (145 mmH2O).\nThe postoperative period was uneventful, a postoperative brain computed tomography scan [] showed correct catheter positioning and stability of the ventricular diameters. The patient had a fast recovery with resolution of headache, visual disturbance, and tinnitus. A postoperative fundus oculi and optical coherence tomography scan documented an improvement of papillary stasis and a significant improvement in the thickness of the optic nerve fibers.\nAfter a 6-month follow-up, she is doing well, without clinical disturbance. MMSE gave a score of 25. She will continue to be followed for the other meningiomas and the VPS function with brain MRI every year and further Gamma Knife treatment of the lesions.
A 39-years-old man was admitted to the emergency room of our Institution for a 1-month history of gait disturbance, postural instability, speech disturbance, and episodes of incontinence. His history was remarkable for communicating hydrocephalus, which had been surgically treated at the age of 14 months with the placement of a ventriculoperitoneal shunt, followed by a distal shunt revision at the age of 16 years. At the admission, brain computed tomography (CT) scan was performed and it showed the presence of 5 cm mixed-density pseudonodular lesion. His neurological examination revealed temporospatial disorientation, absence of focal motor deficits and sensory alteration, normal and symmetrical osteotendinous reflexes, walk of little steps, and wide-based gait, several body oscillations during the Romberg’s test. A contrast-enhanced brain CT study [] confirmed the presence of an enhancing 57 mm left frontotemporal non-homogeneous neoformation, with several calcifications, hypodense colliquated areas, and perilesional edema. Preoperative magnetic resonance imaging (MRI) with gadolinium contrast medium, even though strongly fouled by motion artifacts due to poorly cooperative patient, showed a lesion with lobulated margins, non-homogeneous post contrast enhancement and heterogeneous signal intensity in relation to the presence of solid component mixed with necrotic-colliquative areas and calcifications. In particular, in the peripheral frontal portion, a small oval-shaped component was visible, surrounded by strongly hypointense peripheral border in FFE sequences, arguably due to hemosiderin deposition. The tumor presented a maximum diameter of 6.2 × 4.1 cm on the axial plane [], 6.3 × 3.5 cm on the sagittal plane [], and 3.6 × 3.3 cm on the coronal plane [], and it extended cranially to the knee of the corpus callosum, displacing the large falx to the right and causing a compressive effect on the frontal horn of the right lateral ventricle.\nSurgical procedure was performed with the aid of optic neuronavigation, intraoperative monitoring and the resection was performed though a left pterional approach.[,] Intraoperatively, the neoplasm appeared of soft and partly fibrous consistency, of reddish-grey color and with a fairly good cleavage plane from the adjacent cerebral parenchyma. With the aid of ultrasonic aspirator and microsurgical technique, macroscopical total resection of the exposed neoplastic lesion was carried out. Autologous fibrin glue was used to seal the dural plane.\nFragments of the lesion were sent for pathological examination, which revealed a population of neuronal lineage (Sinaptophysin+, neuron specific enolase+, glial fibrillary acid protein−) consisting of roundish monomorphic cells mixed with a proliferation of capillary vessels and the presence of widespread calcifications; the Ki-67 index was <5%, there was no evidence of mitosis or areas of necrosis. Histopathological and immunohistochemical pattern, according to radiological findings, was consistent with a diagnosis of EVN, classified by the WHO as a Grade II tumor [-].\nThe postoperative course was uneventful and control CT scan [] showed no complications. A further MRI was performed [-], which showed on the left frontal area, in correspondence of the surgical cavity, a large area of structural alteration, consisting predominantly of cerebrospinal fluid and a tissue portion with calcifications.\nAfter the hospital discharge patient underwent at home neurorehabilitation with consistent improvement of his gait and speech. He also underwent adjuvant radiotherapy and at the present his follow-up is still ongoing.