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.
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.