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 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.