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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Extensive and aggressive surgical removal is treatment of choice for patients who have chordomas of the cranial base. Well-developed microsurgical techniques, as well as good surgical judgment learned from experience are essential to avoid major morbidity. A 14-year-old female presented with progressive headaches and diplopia for three months. Cranial magnetic resonance imaging demonstrated a well-circumscribed mass in the clival region. The mass was totally excised via right combined pterional transsylvian and anterior temporal approach (+ orbitozygomatic osteotomy). The tumor was located extradurally. The resected tumor had the typical histological and immunohistochemical characteristics of chordoma. No radiation therapy or chemotherapy was administered.
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PMID:Removal of clival chordoma in an adolescent thorough combned pterional transsylvian and anterior temporal approach. 1791 81

We report a 3-year-6-month-old boy with chordoid meningioma in the foramen magnum. Chordoid meningioma represents between 0.5 and 1.0% of all meningiomas, and it is frequently located in the supratentorial region. The patient started with an episode of instability, falls, and headache; after which, he developed cuadriparesis, cervical pain, and neck stiffness, which slightly improved after medical treatment, so he was referred to our hospital. Physical examination revealed left hemiparesis and cervical muscle spasm with left torticollis. Magnetic resonance imaging (MRI) demonstrated an intradural-extramedullary well-circumscribed, homogeneous enhancing mass, in the anterior part of the foramen magnum with cervical extension. The operative technique was the extreme-lateral craniocervical retrocondylar approach with total removal and dural coagulation. Histopathological examination demonstrated a chordoid meningioma, with meningotelial basophilic mucoid chordoma-like component in 80% of the tissue. The immunohistochemical stains were positive to epithelial membrane antigen, vimentin, and S-100 protein. At 10 months follow-up, the patient showed improvement in his neurological deficit with physical rehabilitation, and motor response in his extremities are now normal; neck stiffness with cervical spasm disappeared postoperatively. MRI showed no residual tumor. To our knowledge, this is the first report of a chordoid meningioma on the foramen magnum in a child.
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PMID:Chordoid meningioma of the foramen magnum in a child: a case report and review of the literature. 1821 97

Chordomas are rare, slow growing tumors of the axial skeleton, which derive from the remnants of the fetal notochord. They can be encountered anywhere along the axial skeleton, most commonly in the sacral area, skull base and less commonly in the spine. Chordomas have a benign histopathology but exhibit malignant clinical behavior with invasive, destructive and metastatic potential. Genetic and molecular pathology studies on oncogenesis of chordomas are very limited and there is little known on mechanisms governing the disease. Chordomas most commonly present with headaches and diplopia and can be readily diagnosed by current neuroradiological methods. There are 3 pathological subtypes of chordomas: classic, chondroid and dedifferentiated chordomas. Differential diagnosis from chondrosarcomas by radiology or pathology may at times be difficult. Skull base chordomas are very challenging to treat. Clinically there are at least two subsets of chordoma patients with distinct behaviors: some with a benign course and another group with an aggressive and rapidly progressive disease. There is no standard treatment for chordomas. Surgical resection and high dose radiation treatment are the mainstays of current treatment. Nevertheless, a significant percentage of skull base chordomas recur despite treatment. The outcome is dictated primarily by the intrinsic biology of the tumor and treatment seems only to have a secondary impact. To date we only have a limited understanding this biology; however better understanding is likely to improve treatment outcome. Hereby we present a review of the current knowledge and experience on the tumor biology, diagnosis and treatment of chordomas.
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PMID:Tumor-biology and current treatment of skull-base chordomas. 1838 12

Chordomas are uncommon tumours occuring from remnants of the notochord. They are mainly localized in the sacrococcygium, the spine and the skull base. We focused our study on skull base chordomas and reviewed 531 published cases and 6 cases diagnosed in our institution. The six cases diagnosed at our hospital are described with a special mention of a unique case presenting with meningitis. Clinical features, radiology, histopathology and survival with different treatments are reviewed. Skull base chordomas can occur at any age and are slightly more frequent in males. Patients complain most often of diplopia and headaches. Abducens nerve palsy is the commonest neurological finding. Radiological examination should include both CT-scan which shows extensive osteolysis, calcifications and contrast enhancement and MRI for topographical definition. Metastases rarely occur. Treatment remains controversial but combined surgery and radiotherapy is the best treatment with a 5 year survival rate of 75%.
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PMID:Skull base chordomas: presentation of six cases and review of the literature. 1863 20

Meningiomas are rare in children. Chordoid meningioma is a very rare variant, as only 16 cases in children have been reported. The authors report the first case of a chordoid meningioma in the third ventricle. A 12-year-old boy presented with headache, abnormal behaviors, and ataxia. Brain MR imaging revealed a 2-cm, well-enhanced mass in the third ventricle and hydrocephalus. Positron emission tomography with [18F]fluorodeoxyglucose showed that the mass was hypermetabolic. Gross-total removal of the mass was performed using a left frontal transcortical and transventricular approach. The mass originated from the left caudate head and was connected to the choroid plexus. A chordoid meningioma was diagnosed on the basis of the histological characteristics of the tumor, which was composed of cords and nests of eosinophilic vacuolated cells with an abundant myxoid matrix, similar to the features of a chordoma. A typical focal meningiomatous pattern was observed. The tumor cells were immunoreactive for vimentin and epithelial membrane antigen. The patient's headache and gait disturbance improved after the tumor was removed. The tumor showed no signs of recurrence during 12 months of follow-up.
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PMID:Third ventricular chordoid meningioma in a child. 1883 62

A 29-year-old man presented with a primary sellar turcica osteochondroma manifesting as intratumoral hemorrhage mimicking pituitary apoplexy. The patient suffered sudden onset of headache concomitant with vision loss in the left eye. Radiography and computed tomography detected destruction and calcification of the sellar turcica. Magnetic resonance imaging revealed a heterogeneously enhanced suprasellar mass that had elevated and compressed the optic chiasm. The preoperative diagnosis was hemorrhagic pituitary adenoma, craniopharyngioma, meningioma, or chordoma based on the signal heterogeneity of the lesion. To relieve the symptoms and make a definitive diagnosis, surgical removal via a basal interhemispheric approach was carried out. The tumor was not totally removed because of tight adhesion to the pituitary stalk, but postoperative ophthalmological examination revealed improvement of the visual disturbance. The histological diagnosis was osteochondroma based on the presence of mature chondrocytes and osteomatous tissue. Osteochondroma should be included in the differential diagnosis of tumors with acute hemorrhage in the sella turcica.
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PMID:Osteochondroma of the sella turcica presenting with intratumoral hemorrhage. 1916 2

We report a 51-year-old man with an unusual intradural suprasellar chondroid chordoma. He presented with headache and diminution of vision in both eyes. MRI demonstrated the suprasellar tumor as an isointense mass with heterogeneous enhancement after intravenous administration of contrast agent. There was neither bony nor dural association, and gross-total removal of the mass was performed using a left extended pterional approach. Based on the histological characteristics of the tumor, which was composed of typical chordoma cells and islands of chondroid elements, we diagnosed a chondroid chordoma. We believe this is the first report of an entirely intradural chondroid chordoma on the suprasellar region. Clinical, radiological, and pathological features of the tumor are described.
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PMID:Intradural suprasellar chondroid chordoma. 2007 56

Intracranial chondromas are rare benign tumors. Little data are available on the diagnosis and treatment of these tumors. Thus, we aimed to describe a large series of patients with intracranial chondroma and to analyze and discuss the clinical features and microsurgical treatment of these patients. We retrospectively analyzed the clinical data of 30 patients (12 males and 18 females; mean age 35.4 years; age range 16-60 years) who had pathologically confirmed intracranial chondroma treated at our hospital from September 1996 to June 2008. Surgery was performed on all 30 patients: five patients underwent postoperative radiotherapy; 26 patients were followed up postoperatively for a mean duration of 45.8 months. The surgical approach was selected according to tumor location. Total resection was achieved in 11 patients, subtotal resection in 13, and partial resection in nine (three patients had recurrent chondroma). Follow-up showed that 21 patients recovered without recurrences, three had recurrence, and two patients died. The clinical manifestations included headache and multiple cranial nerve lesions. Imaging usually showed a well-demarcated extramedullary tumor, centrally located, without surrounding brain edema, partially calcified (73.3%) and with minimal vascularity, often accompanied by erosion and destruction of surrounding bone (56.7%). It is difficult to totally remove an intracranial chondroma, and it is not possible to differentiate a chondroma from a myxoma or chordoma at the cranial base on the basis of clinical manifestations and neuroradiological findings. Selection of the appropriate surgical approach is important for resection of the tumor.
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PMID:Microsurgical treatment of intracranial chondroma. 2171 89

We describe a case of extraosseous chordoma disseminated in the subarachnoid space with favorable long-term follow-up. During work-up of headaches in a 13-year-old girl, MRI revealed multiple cystic subarachnoid masses in the posterior fossa and spinal canal. She underwent posterior fossa craniectomy and was found to have multicentric subarachnoid chordomas with positive CSF cytology. Six years after the operation and radiotherapy, the girl is without neurological deficits despite persistent multiple subarachnoid cystic masses.
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PMID:Disseminated subarachnoid chordoma: long-term favorable follow-up of a pediatric patient. 2198 17

Primary cranial chondrosarcoma in an uncommon cartilaginous tumours of which the myxoid variant is the least reported in the literature. This tumour accounts for 0.15% of all primary intracranial lesions and 6% of all skull base tumours. Chondrosarcomas are frequently misdiagnosed as chordomas, which have a different prognosis. Differential diagnosis is very important because, when treated with similar aggressive treatment strategies, chondrosarcoma has a much better prognosis than chordoma. We describe a 54-year-old female with a 9-month history of left ophtalmoplegia and increasing headache. MR imaging of the head showed a sellar and left parasellar mass. We performed a gross total removal of the mass via a left pterional approach. The histopathologic diagnosis was of a myxoid chondrosarcoma. A post-operative contrast-enhanced computed tomography (CT) scan of the head showed a total removal of the neoplasm. After surgery, the patient showed a transitory dysphasia with right hemiparesis, but they both considerably improved before discharge. Review of the literature was identified using the Medline database: only 10 cases in the worldwide literature were identified to report on this kind of tumour. We present a case report of myxoid chondrosarcoma, a rare variant of chondrosarcoma, with sellar and left parasellar localization. This histopathological type is a low-grade variant and its total removal is effective.
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PMID:Parasellar myxoid chondrosarcoma: a rare variant of cranial chondrosarcoma. 2219 91


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