A 32-year-old left-handed female patient was referred to our department with an MR scan showing a right fronto-opercular diffuse low-grade glioma and performed as a diagnostic work-up for headache (). Left-handedness was confirmed with Edinburgh’s inventory. She had no preoperative language disorders.\nAfter navigation-guided identification of the tumour boundaries, an “awake” resection was performed and intraoperative identification of Broca’s area (tag 5) with “speech arrest” and inferior fronto-occipital fascicle (IFOF) with transient fonemic paraphasia was reached (tag 8) , and . Resection was then stopped.\nHistology provided evidence for isocitrate dehydrogenase 1(IDH1)-mutated, ATP-Dependent Helicase (ATRX)-wild diffuse low-grade astrocytoma. Postoperative MR showed a subtotal removal with residual disease where IFOF was intraoperatively identified (). No adjuvant therapy was then proposed, and the patient is currently fully working. If evidence of residual disease progression is observed, a new surgery will be proposed, delaying eventual adjuvant therapy as much as possible.\nThis may be considered an example case of the “multi-staged” approach to dealing with diffuse low-grade gliomas, making it possible to get control of the disease as much as possible, postponing chemotherapy or radiotherapy as far as possible and reserving them for when surgery can no longer be performed.
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.