Cases reported "chordoma"

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1/459. Interesting radiologic findings in suprasellar mass lesions. Report of three cases.

    The authors report three quite rare lesions of the sellar/parasellar region. They are namely; pituitary abscess, cystic macroadenoma and osteochondroma. In none of the cases, the preoperative diagnostic priority was not same as the final histopathologic diagnosis. The radiologic findings of these pathologies are discussed with emphasis on differential diagnosis. ( info)

2/459. A new transfacial approach for lesions of the clivus and parapharyngeal space: the partial segmented Le Fort I osteotomy.

    Tumors of the clival and parapharyngeal areas are a challenge because of their location. They used to be considered inaccessible because the aggressive approaches employed caused elevated levels of morbidity. This fact led to more conservative approaches that attempted to preserve the exposure of the lesion. These approaches were a combination of cranial and facial procedures, thus utilizing a combined effort between neurosurgeons and maxillofacial surgeons. We described our experience with a partial segmented Le Fort I osteotomy added to a transmandibular approach to expose a chordoma of the clivus and left parapharyngeal space. A three-dimensional imaging was used as a diagnostic tool and to plan the optimal surgical approach. The operative technique was described in this case study. Some important technical details of the approach are described. The global outcome was favorable. ( info)

3/459. First cytogenetic study of a recurrent familial chordoma of the clivus.

    Two recurrences of a familial clivus chordoma, arisen from a patient who developed the primary tumor at age of 8 years, were investigated by cytogenetic and the fluorescence in situ hybridization (FISH) approach. Of the patient's 3 daughters, 2 developed, respectively, a clivus chordoma and an astrocytoma in infancy, a familial aggregation highly suggestive of a genetic background. After a 31-year hiatus, 2 tumor recurrences, developed over 17 months, were removed surgically. Both were hypo- or nearly diploid, and had a pronounced karyotypic heterogeneity with clonal and non-clonal rearrangements affecting several chromosomes. The same rearrangement, a dic(1;9)(p36.1;p21), was shared in both tumor specimens and, in 90% of the cells, chromosome 1p appeared to be involved in unbalanced translocations with different chromosomes, leading to variable losses of 1p. Previous cytogenetic data concerning chordoma are limited to 10 sporadic tumors with an abnormal karyotype; although no tumor-specific rearrangements have been identified, chromosome 1p appears to be involved frequently. ( info)

4/459. Clear cell meningioma of the lumbo-sacral spine with chordoid features.

    Clear cell meningioma (CCM) is a peculiar variant that differs from conventional meningioma in affecting younger patients, arising more often in spinal or cerebellopontine locations, and showing a higher recurrence rate. Classical meningothelial areas are scarce in these tumors and the differential diagnosis with other neoplasms, particularly metastatic carcinoma, is often difficult. We report a case of clear cell meningioma from the lumbosacral spine in which location, radiologic presentation, light microscopic appearance in initial sampling, and some of the ultrastructural findings were reminiscent of chordoma. The tumor cells were diffusely positive for vimentin and very focally positive for epithelial membrane antigen. Ultrastructural demonstration of interdigitating cell processes joined by numerous desmosomes confirmed the diagnosis of CCM. ( info)

5/459. A case of chordoma in association with rectal carcinoma.

    A 74-year-old male patient presented with anal and sacral pain 18 months after abdomino-perineal resection for rectal cancer. Computerized tomography (CT) of the pelvis demonstrated a well defined mass anterior to the lower sacrum, posteriorly infiltrating and destroying the fourth and fifth sacral nerves and invading the right gluteal fossa. A 7.5 x 15 x 2 cm encapsulated mass was demonstrated during the operation using a posterior approach and the lower sacral segments together with the tumour were removed by amputation at S3 level. Histopathology revealed chordoma. This case is unique because of the rarity of chordoma in association with rectal tumour at the sacrococcygeal region. ( info)

6/459. The surgical management of sacrococcygeal chordoma.

    BACKGROUND: Complete excision of sacrococcygeal chordoma is necessary at initial surgery due to its poor sensitivity to radiotherapy and chemotherapy. However, due to the anatomic characteristics of this tumor, intralesional excision tends to be employed, resulting in local recurrences in many patients. methods: The clinical features and results of surgical treatment of 13 patients with sacrococcygeal chordoma who were treated at the Chiba Cancer Center and Chiba University beginning in 1972 were analyzed. RESULTS: Intralesional excision was performed in eight patients, marginal excision in two patients, and wide excision in three patients. Local recurrence was observed in six patients, with a high proportion occurring in the gluteal muscles attached to the sacrum (the gluteus maximus muscle and piriform muscle). Seven patients died of their disease and six patients were alive with no evidence of disease. The 5-year survival rate was 81.8% and the 10-year survival rate was 29.1%. CONCLUSIONS: It is highly possible that residual chordoma infiltrating the gluteal muscles accounts mainly for the local recurrences. Therefore, a precise preoperative assessment of the tumor infiltration into the gluteal muscles by magnetic resonance imaging is important for the prevention of local recurrence. For complete tumor removal, a radical wide posterior surgical margin of the gluteal muscles should be employed. A less radical anterior surgical margin is sufficient because there is a firm presacral fascia anterior to the sacrum. The appropriate surgical margin for the complete removal of the chordoma differs according to the location of the tumor and tissues involved. ( info)

7/459. Surgical management of intraosseous skull base tumors with aid of Operating arm System.

    Invasion of bone and critical neurovascular structures often impedes complete resection of intraosseous skull base neoplasms, and these lesions tend to recur unless all infiltrated bone is removed. Evolving experience with image guidance over the past few years indicates the potential value of neuronavigation in skull base lesions diffusely infiltrating or fixed to bone structures. We report our early experience with the Radionics Operating arm System (OAS), specifically emphasizing its utility as an adjunct in the treatment of intraosseous skull base tumors, mainly meningiomas. In April 1995 the OAS was introduced into clinical use at the neurosurgical university clinic in Munster, germany. Since then, the system's utility has been explored in 10 patients out of the total neuronavigation series presenting with intraosseous skull base tumors (nine females and one male, mean age 47 years; nine meningiomas, one chordoma). For navigational planning, both 3-mm computed tomography scans and a set of 3-mm fat-suppression magnetic resonance images were chosen. At least four adhesive skin markers were used for system calibration. The system was technically usable in all cases in this small series. Because of the relative immobility of the bone structures and/or the tumor, no significant deviation from the preoperative registration accuracy was noted at the end of the procedures. The main advantages were easier localization and resection of infiltrated bone, which is often not grossly identifiable, even under the microscope. Our preliminary experience with the OAS suggests that image guidance is helpful in this type of lesion, providing better anatomical orientation during surgery and delineating tumor margins and their relation to critical neurovascular structures. The problem of a possible intracranial tumor and brain shift can be neglected in these lesions. The system facilitates resection by volumetric contour information, allowing more aggressive and complete resection. ( info)

8/459. Complex cervical spine neoplastic disease: reconstruction after surgery by using a vascularized fibular strut graft. Case report.

    The authors report a case of an aggressive chordoma in the cervical spine of a 15-year-old girl who underwent radical resection followed by reconstruction using an anterior vascularized fibular strut graft and posterior arthrodesis prior to receiving immediate postoperative radiation therapy. The patient had successful graft incorporation 4 months postoperatively. The authors review the advantages of using vascularized fibular strut grafts for the treatment of multilevel cervical spine neoplastic disease and discuss the theoretical advantages of using vascularized grafts that tolerate therapeutic levels of radiation. ( info)

9/459. Drop metastases in a patient with a chondroid chordoma of the clivus.

    Metastasising chordomas are extremely rare and only four cases with drop metastases have been reported. We report a patient with an intracranial chondroid chordoma, typically involving the clivus, treated by repeated resection, percutaneous transluminal embolisation and radiosurgery. During follow-up with MRI asymptomatic intradural drop metastases were observed throughout the spine, with transgression of the intervertebral foramen, forming a "dumbbell". ( info)

10/459. chordoma in the cervical spine managed with en bloc excision.

    STUDY DESIGN: En bloc resection of a chordoma in the midcervical vertebral spine was performed. OBJECTIVES: To document the surgical technique used for en bloc excision of a chordoma arising in the midcervical spine. SUMMARY OF BACKGROUND DATA: Malignant tumors arising in long bones are excised en bloc. The authors recently designed a technique for en bloc resection of malignant tumors in the thoracolumbar spine using the T-saw. However, this technique is difficult in tumors of the cervical spine, and there are no previous reports of successful en bloc resection of such tumors. methods: Using an anterior approach, the ipsilateral vertebral artery was ligated. This was followed by sharply cutting the pedicle of the cervical vertebra with a specially designed T-saw. RESULTS: En bloc excision of chordomas in the cervical spine was achieved using the T-saw. CONCLUSION: Although the surgical margin was intralesional in a small area, the technique used in this case study indicates that en bloc excision of such tumors can be used with a safety margin even in the cervical spine. ( info)
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