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 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.