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 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.