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 52-year-old man complained of nasal stuffiness along with bleeding, headache, and vomiting. He was referred to our department after magnetic resonance imaging (MRI) showed an enormous mass occupying the nasal and paranasal cavities and extending into the bilateral frontal base [Figure and ]. No neurological deficit other than anosmia was identified. The tumor spread beyond the nasal cavity and paranasal sinuses, and was therefore classified as stage C based on the modified Kadish clinical staging system [].[] Total removal of the tumor was achieved surgically, using a transnasal approach in combination with a bilateral front basal craniotomy [Figure and ]. The dura along the anterior skull base was opened partially and the cranial base was reconstructed using the pericranial flap. Histological examination of the tumor demonstrated proliferating tumor cells with large, oval nuclei containing prominent nucleoli. Scattered necrotic changes, nuclear fission, and Homer–Wright rosettes were identified. These findings were consistent with Hyams grade III ONB [].[] No neurological deficit other than olfactory analgesia was observed after surgery, however, the patient suffered from meningitis caused by cerebrospinal fluid rhinorrhea that developed 1 week postoperatively. Because complete eradication of the rhinorrhea and meningitis required 9 weeks of treatment, radiation therapy was started 10 weeks after the surgery. Large-field irradiation of the resected area, from the nasal cavity to the frontal lobe, was administered with a total of 60 Gy in 30 fractions. The patient was discharged 17 weeks after the surgery and was followed-up on an outpatient basis. At the time of discharge, whole-body enhanced computed tomography (CT) revealed no apparent distal metastasis or tumor recurrence.\nAt 24 weeks postoperatively, the patient noted numbness in both upper limbs, which gradually worsened. He was readmitted 25 weeks after the surgery because of rapidly deteriorating symptoms. Neurological findings on readmission revealed tetraparesis, hypesthesia, and hypoalgesia below C6, hyperreflexia of both legs, and urinary incontinence. CT and MRI revealed an intradural lesion compressing the spinal cord along its right ventral aspect at C5 through T2 [Figure –]; MRI of the head showed no evidence of tumor recurrence. In view of the diffuse nature of the disease, it was decided to surgically relieve the compression by the tumor to avoid complete tetraplegia. A laminectomy from C4 to T3 was performed. Intraoperative findings confirmed the absence of tumor in the epidural space. However, under the arachnoid membrane, the tumor compressed the spinal cord along its right ventral aspect. Because the tumor adhered to the spinal surface and was entangled in several nerve roots, it was removed but not completely. A dural plasty was then performed with a Gore-Tex® membrane to avoid restriction of the subdural space. The tumor was histologically diagnosed as ONB, consistent with the first operation [Figure and ]. After surgery, his urinary incontinence promptly improved. Paralysis of the right upper and both lower limbs remained but seemed to be improving. Residual tumor was seen on MRI performed 1 week after the surgery but the spinal cord was well decompressed [Figure –]. Whole-spine irradiation of 58 Gy in 29-Gy fractions was started 1 week postoperatively. MRI after irradiation no longer revealed residual spinal tumor, but shortly after spinal irradiation, the patient became progressively lethargic. Brain MRI showed multiple leptomeningeal enhanced lesions. General malaise and anorexia followed and the patient developed bilateral pneumonia, which caused his death 16 weeks after the surgery for ISM and 41 weeks after the first operation. During the time before his death, there had been no further deterioration in the motor weakness of his four extremities.