Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018681 (headache)
56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Sphenoorbital meningiomas require extensive bone removal around the superior and lateral orbital walls, superior orbital fissure, and anterior middle fossa floor. Incomplete resection can lead to recurrence or growth into the cavernous sinus (CS). A 46-year-old woman with a history of childhood leukemia treated with chemotherapy and whole-body radiotherapy had presented to an outside institution in 2004 with headache and vision changes and undergone subtotal resection for right sphenoorbital meningioma. Residual tumor growth caused progressive optic neuropathy, and she underwent multiple orbital decompressions and fractionated radiotherapy. In 2017, she underwent another craniotomy for repeat resection. Additional tumor growth causing neuropathic facial pain syndrome and progressive ophthalmoplegia was treated with orbital enucleation. On referral to our institution, magnetic resonance imaging demonstrated right sphenoorbital and CS meningioma extending into the sella and nearly to the medial border of the contralateral CS. Given her complete ophthalmoplegia and recent orbital enucleation, she underwent revision right frontotemporal craniotomy for radical resection of invasive meningioma, including right internal carotid artery occlusion and CS resection (Video 1). The skull-base defect was repaired with autologous fascia and a free muscle flap. Postoperative transient aphasia and left hemiparesis resolved over several days. At the 1-month follow-up examination, she was neurologically intact, with moderate improvement of facial pain syndrome (preoperative pain score, 9 of 10; postoperative pain score, 6 of 10). Magnetic resonance imaging demonstrated gross total resection. Pathological tissue analysis was consistent with grade 1 meningioma with an increased MIB-1 proliferative index, although, clinically, the tumor behaved more malignantly. The patient provided consent.
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PMID:Resection of Invasive Sphenoorbital and Cavernous Sinus Meningioma via Frontotemporal Craniotomy. 3241 38

Skull base chordomas account for less than 0.2% and chondrosarcomas for less than 0.15% of all intracranial tumors. Although their clinical and imaging presentations are similar, they derive from different origins. Chordomas arise from embryonic remnants of the primitive notochord and chondrosarcomas from primitive mesenchymal cells or from the embryonic rest of the cranial cartilaginous matrix. Both entities are characterized by infiltration and destruction of the surrounding bone and soft tissue and a high locoregional recurrence rate. Chondrosarcomas, when treated with similar complex strategies, display a much better prognosis than chordomas. The overall survival is approximately 65% for chordomas and 80% for chondrosarcomas at 5 years and 30 and 50%, respectively, at 10 years. Chordomas are divided into the following 3 histological types: classical (conventional), chondroid, and dedifferentiated. Chondrosarcomas have conventional, mesenchymal, clear cell, and dedifferentiated subgroups. Both tumor entities often present with nonspecific symptoms, and headaches are the most reported initial symptom. Computed tomography and magnetic resonance imaging are required to determine the tumor localization and the extent of tumor growth. The treatment philosophy is to maximize tumor resection, minimize morbidity, and preserve function. Neurosurgical approaches commonly used for the resection of intracranial chordomas and chondrosarcomas are transsphenoidal, transbasal, cranio-orbitozygomatic, transzygomatic extended middle fossa, transcondylar, and transmaxillary approaches. Chordomas and chondrosarcomas are not sensitive to chemotherapy and there are no approved drugs for their treatment. The present treatment concept is a combination of surgical resection with a maximal excision and preserving patients' quality of life by adjuvant radiotherapy for both chordomas and chondrosarcomas.
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PMID:Skull Base Chordomas and Chondrosarcomas. 3254 Nov 36


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