Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Nine patients with mesenchymal tumors of the skull base have undergone radical tumor excision by the use of the Infratemporal fossa approach. Three cases of chondrosarcoma, two of leiomyosarcoma, and one case each of synovial sarcoma, chondroid chordoma, myxoma, and fibromatosis were managed. In the mean postoperative review time of 6.6 years (range 1.7 to 13) there are no clinical or radiologic signs of recurrence at the primary site in any patient, although two have developed distant metastases. The purpose of this article is to demonstrate that, with aggressive and preferably early surgical management using this type of access to the skull base, complete removal of these tumors can be undertaken, and it is now possible to achieve permanent ablation of this type of pathologic condition at the skull base.
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PMID:Mesenchymal tumors of the skull base with particular reference to surgical management and outcome. 1717 Aug 51

The authors report the case of a chordoma with malignant cytologic features, presenting as a mass lesion in the clival and infratemporal region at the level of the craniocervical junction in an 8-year-old female. Following gross resection of the mass, the patient subsequently developed distant subcutaneous and peritoneal metastases from the lesion. The rare histologic features, the surgical approach to the lesion, and the follow-up management of this unique case are discussed.
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PMID:Malignant clival chordoma with postoperative cutaneous metastases. 1717 Sep 54

Chordoma is a rare malignant tumor derived from remnants of the primitive notochord. It can occur anywhere along the scull base and spine. The most commonly involved area is the sacrococcygeal region. Although chordomas invade adjacent structures, they metastasize less often. We present a case of a sacrococcygeal chordoma with liver metastasis. The nature and origin of the liver lesion was proved with fine-needle aspiration (FNA) under computed tomographic (CT) guidance.
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PMID:Isolated liver metastasis from sacral chordoma. Case report and review of the literature. 1747 81

Metastasis to bone is a common event in the natural history of nearly all neoplasms, which often greatly affects the patient's quality of life. Bone metastases can cause pain and pathological fractures, or even a cord compression syndrome with severe neurological symptoms. The treatment of metastatic disease requires a multidisciplinary approach that addresses systemic and local disease. On a basis of available literature as well as own research current opinions on this subject has been presented in these paper.Where the treatment objective is pain relief, a single 8 Gy treatment is recommended as the standard dose-fractionation treatment of symptomatic but uncomplicated bone metastases. External beam irradiation achieves pain palliation in more than 75% of patients. Radiotherapy with doses of 40-50 Gy results in remineralization in 60-80% of patients 4-8 weeks after irradiation. The role of radiotherapy in the treatment of primary bone cancer is limited. The choice of the best local treatment of Ewing's sarcoma of bone remains a controversial issue. Surgery should always be considered in the local treatment of Ewing's sarcoma. Postoperative radiation therapy must be added when surgical margins are inadequate. Radiotherapy is used in the treatment of plasmacytoma, chordoma and chondrosarcoma.
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PMID:Role of radiotherapy in the treatment of osteolytic damage due to bone tumors. 1761 44

Parachordoma, or myoepithelioma, is a very rare tumor histologically resembling chordoma but occurring in the nonaxial soft tissues. It typically has an indolent nature, with occasional late recurrence and even rare metastases. Review of existing literature reveals a male predilection, with the tumor typically occurring in the fourth decade of life in the lower extremity. It typically is managed with wide resection. We report the case of a 60-year-old woman with a right distal upper arm parachordoma treated with wide resection of the tumor.
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PMID:Case report: parachordoma of soft tissues of the arm. 1821 45

According to the WHO grading system, myxopapillary ependymomas are assigned to WHO Grade I. However, the clinico-pathological spectrum might be very heterogenous. Herein, we report 4 cases exhibiting lumbar tumor masses, 1 causing muscular atrophy over a 30-year period, 3 displaying clinical history of persisting lumbar pain for only several weeks. All tumors were crooked with dura and spinal roots resulting in incomplete resection in three cases. On histological examination, two tumors were almost acellular and showed polycyclic hyaline and fibrotic extracellular matrix leading to differential diagnoses of chordoma, meningioma, fibrolipoma and ependymoma. Finally, together with the immunohistochemical investigations, electron microscopy led to the diagnosis of myxopapillary ependymoma, WHO Grade I, with massive degenerative changes. The other 2 cases presented with the typical neuropathology of myxopapillary ependymomas but showed local recurrence within 1 and 13 years throughout the whole neuraxis, and in 1 case additional metastases of the 3rd ventricle. Although the morphological feature of these myxopapillary ependymomas was benign, the presented cases showed that the biological behavior of myxopapillary tumors might differ greatly and that these tumors present a serious operative and diagnostic challenge. Myxopapillary ependymomas occur most often in the lumbosacral region. Due to the anatomic complexity of the cauda equina, a complete resection can be technically challenging in this region. However, a gross total resection at the primary surgery is the most predictive factor for the outcome.
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PMID:The clinico-surgico-pathological spectrum of myxopapillary ependymomas--report of four unusal cases and review of the literature. 1825 71

Axial chordoma represents approximately 1% of malignant bone tumors. This tumor expresses cytokeratins, specifically cytokeratin 19, and commonly S100. More recently brachyury, a transcription factor important in mesodermal differentiation, including notochord development, has been detected by immunohistochemistry in axial chordomas and hemangioblastomas but not chondrosarcomas or other neoplasms. In this report, we describe 10 cases (6 men, 4 women: age 18 to 68 y; mean 44.6) of extra-axial tumors, 8 in bone and 2 in soft tissue, with morphologic and immunohistochemical features identical to those of axial chordoma. Imaging excluded metastases from axial chordoma. Three tumors occurred in the tibia, the others in the rib, metatarsal, ulna, femur, pubis: 2 intracortical, 6 intramedullary. Both soft tissue brachyury-positive tumors, one involving the thumb the other the wrist, were sited in the juxta-articular region. Seven of the tumors were widely excised and these patients are disease-free but of the 3 tumors that recurred, 1 was curetted, 1 was marginally excised, and 1 had a pathologic fracture on presentation. Metastases have not occurred in any of the patients. We also confirm the expression of brachyury in hemangioblastomas, and for the first time demonstrates its expression in spermatogonia and testicular germ cell tumors by immunohistochemistry. Brachyury was not detected in a wide range of tumors including carcinomas, lymphomas, and sarcomas. In conclusion, we describe the first series of extra-axial skeletal chordomas bringing the total number of such cases reported in the literature to 11, and present the first report of 2 soft tissue chordomas as defined by brachyury expression.
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PMID:Brachyury expression in extra-axial skeletal and soft tissue chordomas: a marker that distinguishes chordoma from mixed tumor/myoepithelioma/parachordoma in soft tissue. 1830 Oct 55

Purpose. There are, in general, few published series on chordoma. It is a rare disease and further data are still needed.Patients/methods. The data of 37 patients with chordoma were retrospectively analyzed. Treatment was surgical excision in 11, radical radiotherapy in 9 and a combination of the two in 16 cases. The male to female ratio was 2.7 : 1. Median age was 59 years (range 1-89 years).Results. The most common symptoms at diagnosis were pain (98%), neurological disturbances (42%) and incontinence (33%). The tumours were located in the sacro-coccygeal region in 68%, the spheno-occipital region in 16% and the vertebrae in 16% of the patients. Median tumour.size was 7 cm (range 1-30 cm). Local recurrence occurred in 21/36 treated cases and distant metastases developed in eight patients (23%). The median time to recurrence/progression after primary treatment was 2 years (range 1-10 years). The actuarial 5-year rates of overall, progression-free and symptom-free survival were 40%, 31% and 20%, respectively. The corresponding 10-year rates were 26%, 21% and 14%, respectively. At the time of analysis, seven patients were alive, six without evidence of disease. Four of the six patients without active disease were symptom free. A univariate analysis showed that age, sex, tumour size, histopathology, surgical safety margin, treatment modality and radiation dose did not significantly affect overall, progression-free or symptom-free survival. Only turnout site had a prognostic value with turnouts in the spheno-occipital region carrying the worst prognosis.Discussion. We conclude that effective treatment against chordomas is still lacking and a prospective multi-institutional registration study may provide more information on the optimal work-up and treatment of this rare disease.
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PMID:A retrospective clinicopathological study of 37 patients with chordoma: a danish national series. 1852 Dec 19

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

Chordomas are tumors with a bad prognosis, because of their location, local aggressiveness and high rate of local relapse. Despite of be benign tumors, they have certain capacity of metastasize and a clinical evolution that results interesting. When we analyzed our series with 35 chordomas studied and treated between 1975 and 2002, we found three patients that experienced a systemic dissemination.
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PMID:Metastatic disease from chordoma. 1866 85


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