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 54-year-old male physician presented to the emergency department with a 4-day duration complaint of ataxia and bilateral lower extremities numbness (right greater than left) which had progressed. Additionally, he had experienced an episode of fecal incontinence without any bladder issues. The ataxia had been progressively worsening without significant focal weakness.\nHis medical history was significant for ENB of the anterior cranial fossa treated with resection and radiation therapy 13 years prior to this admission. He then underwent left cervical lymph node resection and local radiation of his ENB metastasis 6 years after the initial craniotomy due to the finding of an anterior neck mass. Two years later, during a posttrauma evaluation for neck pain, it was noted on his bone scan that he had multiple metastatic lesions to the cervical spine without any cord compression. At that time, he started on chemotherapy with Cytoxan, adriamycin infusion, and DTIC. After some response in his cervical lymph nodes, he was taken off chemotherapy for a period. Due to progressive adenopathy, the patient started on cisplatin and VP-16 for several months. Once again, his symptoms stabilized.\nThree years prior to this admission, due to a diagnosis of leptomeningeal spread, the patient underwent a repeat craniotomy for resection of his anterior fossa recurrence, followed by whole brain radiation. Following a complaint of mid-lower back pain, he was diagnosed with a T11 lesion and had local radiation. To treat progression of his leptomeningeal disease, he completed three cycles of temodar in the year prior to this admission. His medical history also included scoliosis surgery with Harrington rods 25 years prior to his last admission.\nOn physical examination, the patient had slight weakness of his left lower extremity, especially his left extensor hallucis longus. He had decreased light touch and pinprick sensation of his right T11 dermatome and diminished proprioception of his left lower extremity. He had decreased rectal tone and hyperreflexia of his left knee with trace right ankle reflexes. There was a positive Babinski sign on the left. Due to artifacts from Harrington rod scoliosis correction of his thoracic spine, we obtained a CT myelogram in addition to the MRI of his thoracic spine. The CT myelogram revealed a near-complete block of contrast at T8-9 (). His cervical spine MRI showed stable metastasis to his C5-6 level without any cord compression. Multiple bony lesions throughout the spinal vertebras were noted.\nGiven the patient's medical history and symptoms, surgical resection was recommended. He underwent a posterior laminectomy from T7–9. Epidural and intradural extension of the tumor to the ventral left side of the cord with significant cord compression was noted. With a finding of arachnoid plane between the tumor and spinal cord, microsurgical technique was used to remove 80–90% of the tumor and duraplasty was performed from T7–9 to create space in case of tumor recurrence.\nMicroscopic examination of T8 lesion revealed a small cell tumor lacking necrosis (). Immunohistochemical analysis showed diffuse immunoreactivity for neuron-specific enolase and synaptophysin (Figures and ), with scattered cell positive for chromogranin A (). S100 stain showed perilobular disposition of flattened cells, indicative of sustentacular differentiation (). The findings were of a metastatic neuroendocrine carcinoma, compatible with an esthesioneuroblastoma.\nPostoperatively, the patient did well with a stable neurological exam followed by completion of 2 cycles of chemotherapy with Cytoxan, adriamycin, and DTIC. At his follow-up visits every 3 months, his tumor remained stable on CT and MRI studies for a period of one year.\nShortly thereafter, the patient presented with worsening ataxia and bilateral lower extremity numbness and paresthesias. His motor exam was worse indicating bilateral 4/5 strength diffusely in his lower legs. The MRI and CT myelograms of his thoracic spine revealed recurrent tumor extending above the resection cavity from T7-8 with significant cord compression ().\nThe patient underwent a microsurgical resection of his intradural tumor with extension of his laminectomy. Ventral-lateral extension of his tumor to the neural foramens was noted. The canal was widened and once again, duraplasty from T6–9 was performed. The histology was of a persistent esthesioneuroblastoma. Postoperatively, his exam remained stable and he was discharged to a rehab facility. The patient subsequently had local radiation to his thoracic surgical cavity and his lower cervical spine (C5-6), which showed progression of his tumor without significant clinical symptoms.\nA year later, despite radiotherapy and aggressive treatment, the patient again presented with worsening lower extremities weakness. His MRI and myelograms illustrated recurrence of his tumor in his upper thoracic spine and contrast block at the T8 level (). Consequently, the patient underwent staged resection and exploration of his tumor from T4–T10 with decompression of his neoplasm. Onlay duraplasty with subarachnoid drain placement above the resection cavity was performed. He continued treatment with physical therapy and was followed by the pain service for his neurogenic pain and spasm.\nEight months after his last resection, the patient's MRI studies showed progression of his tumor with soft tissue masses throughout his thoracic spine and diffuse gliosis. No further significant canal stenosis was noted (). He was dependent on a wheelchair, but physical therapy felt that he might get some improvement in strength with intense rehabiltiation. Thus, he was transferred to inpatient rehabiltiation. Three years later, he passed away from pneumonia.