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.
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.