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 48-year-old woman presented with a large sinonasal mass with extensive intracranial extension through the cribriform plate into the anterior cranial fossa []. Tumor was noted in the right orbit and maxillary and sphenoid sinuses and there was lymphatic spread to a retropharyngeal lymph node. She underwent radical resection of the Kadish stage D esthesioneuroblastoma with a right orbital exenteration and upper neck dissection followed by adjuvant radiation therapy. Yearly surveillance imaging was stable until a 6-year follow-up MRI revealed 1 cm of patchy enhancement in the right frontal lobe and the dura overlying the resection cavity. Follow-up imaging 6 months later showed mildly increased dural enhancement and interval development of a new dural-based lesion in the posterior fossa.\nSpinal imaging revealed multiple dural-based enhancing lesions in the cervical, thoracic, and lumbar spine with possible osseous involvement, consistent with drop metastasis []. The patient underwent a suboccipital craniectomy for resection of the posterior fossa lesion. Intraoperatively, the neoplasm was deep to the dura but above the arachnoid membrane; it was carefully dissected and removed en bloc. Two days later, the patient underwent T8–T10 laminectomies, left T9 costotransversectomy, and ligation of the nerve root for resection of a large dural-based mass displacing the thoracic cord and effacing the neural foramen. Pathological analysis of both lesions affirmed a diagnosis of recurrent esthesioneuroblastoma with leptomeningeal spread [].\nThe patient initially began a course of systemic carboplatin, vincristine, and lomustine, as well as intrathecal methotrexate. She ultimately completed the intrathecal methotrexate with carboplatin, etoposide, and cyclophosphamide. Follow-up imaging 16 months after the initiation of chemotherapy revealed resolution of the metastatic lesions and no radiographic appearance of residual disease, 9 years after her original diagnosis. She continues to receive surveillance for the recurrent disease by evaluation of her cerebrospinal fluid (CSF) every 3 months.