Cases reported "Chordoma"

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1/26. 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.
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ranking = 1
keywords = sacrum
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2/26. 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.
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ranking = 2
keywords = sacrum
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3/26. Tumor-like tuberculosis of the sacrum.

    Isolated tuberculosis of the sacrum in a 43-year-old woman manifested as functional impairment of the right lower limb. Sacral tuberculosis is rare in patients with no history of tuberculosis. Another unusual feature was the tumor-like aspect of the lesion, with diffuse, ill-defined osteolysis of a large part of the sacrum and extension to the presacral soft tissues responsible for rectal displacement.
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ranking = 6
keywords = sacrum
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4/26. Crush preparation findings of "sarcomatoid" chordoma of the sacrum: report of a case with histologic, immunohistochemical, and ultrastructural correlation.

    We report on the crush preparation findings of a case of "sarcomatoid" chordoma occurring in the sacral region of a 78-yr-old Chinese male. The smears showed clumps and small cords of polygonal tumor cells containing bubbly cytoplasm and round to oval nuclei. Focally, there were also aggregates of long filamentous spindle cells and stellate bizarre cells with marked nuclear pleomorphism. Occasional tumor cells were seen in association with dense amorphous material. Histologic examination of the excised specimen showed features of the so-called "sarcomatoid" chordoma which consisted of prominent foci of mitotically inactive spindle and pleomorphic cells, in addition to the conventional chordoma areas. An osteosarcoma-like pattern of probably metaplastic nature was also seen within the tumor. Immunohistochemical study showed that most tumor cells expressed cytokeratins. Ultrastructural examination revealed the characteristic rough endoplasmic reticulum-mitochondria complexes. While there are many spindle and pleomorphic cells seen in crush preparations, the distinction from other true high-grade malignancies is important. Recognition of these "pseudoanaplastic" cytologic features also helps to expand the morphologic spectrum of chordoma.
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ranking = 4
keywords = sacrum
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5/26. Investigation of the added value of high-energy electrons in intensity-modulated radiotherapy: four clinical cases.

    PURPOSE: Intensity-modulated radiotherapy (IMRT) with photon beams is currently pursued in many clinics. Theoretically, inclusion of intensity- and energy-modulated high-energy electron beams (15-50 MeV) offers additional possibilities to improve radiotherapy treatments of deep-seated tumors. In this study the added value of high-energy electron beams in IMRT treatments was investigated. methods AND MATERIALS: In a comparative treatment planning study, conventional treatment plans and various types of IMRT plans were constructed for four clinical cases (cancer of the bladder, pancreas, chordoma of the sacrum, and breast). The conventional plans were used for the actual treatment of the patients. The IMRT plans were optimized using the orbit optimization code (Lof et al., 2000) with a radiobiologic objective function. The IMRT plans were either photon or combined electron and photon beam plans, with or without dose homogeneity constraints assuming standard or increased radiosensitivities of organs at risk. RESULTS: Large improvements in expected treatment outcome are found using IMRT plans compared to conventional plans, but differences in tumor control probability (TCP) and normal tissue complication probabilities (NTCP) values between IMRT plans with and without electrons are small. However, the use of electrons improves the dose-volume histograms for organs at risk, especially at lower dose levels (e.g., 0-40 Gy). CONCLUSIONS: This preliminary study indicates that addition of higher energy electrons to IMRT can only marginally improve treatment outcome for the selected cases. The dose-volume histograms of organs at risk show improvements for IMRT with higher energy electrons, which may reduce tumor induction but does not substantially reduce NTCP.
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ranking = 1
keywords = sacrum
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6/26. First histologically confirmed case of a classic chordoma arising in a precursor benign notochordal lesion: differential diagnosis of benign and malignant notochordal lesions.

    The first histologically confirmed case of a classic chordoma arising in a precursor benign notochordal lesion is presented and the differential diagnosis between benign and malignant notochordal lesions is discussed. A 57-year-old man presented with a classic chordoma in the coccyx. The resected specimen demonstrated a small intraosseous benign notochordal lesion in the coccyx, which was adjacent to the classic chordoma. Also seen were two separate, similar benign lesions in the sacrum. The classic chordoma consisted of multiple lobules that were separated by thin fibrous septa and that showed cords or strands of atypical physaliphorous cells set within an abundant myxoid matrix. In contrast, the benign lesions consisted of intraosseous sheets of bland physaliphorous cells without any extracellular matrix. The affected bone trabeculae showed sclerotic reactions. It was concluded that benign and malignant notochordal lesions can be distinguished microscopically.
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ranking = 1
keywords = sacrum
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7/26. Local recurrence after S2-3 sacrectomy in sacral chordoma. Report of four cases.

    Excision is the treatment of choice in cases of sacral chordoma. Local recurrences, however, have often been observed even after total en bloc resection. The authors assessed outcomes in four cases of tumor recurrence in patients who underwent total en bloc S2-3 resection for sacral chordomas that were located below S-3. The primary recurrences were located at either side of the lateral portion of the remaining sacrum in all patients. In two patients in whom preoperative magnetic resonance imaging indicated no invasion of the tumor into surrounding soft tissues, recurrence in the resected end of the gluteus maximus or piriformis muscle was also observed. The authors therefore recommend that the S2-3 sacrectomy should be performed over an adequate margin, including a part of sacroiliac joints at the bilateral portions of the sacrum and soft tissues such as the gluteus maximus or piriformis muscle.
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ranking = 2
keywords = sacrum
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8/26. A unique presentation of retroclival chordoma.

    Chordomas are rare tumours which arise from remnants of the primitive notochord. They occur primarily in the sacrum, clivus and cervical regions. We report a case of retroclival chordoma which presented as an extradural haemorrhage following minor trauma. The underlying tumour was not apparent on imaging performed immediately following the event, and chordoma presenting in this manner has not previously been described in the literature. The tumour became apparent on subsequent imaging, and progressed despite surgical debulking and radiotherapy.
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ranking = 1
keywords = sacrum
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9/26. Total sacrectomy and reconstruction for sacral tumors.

    STUDY DESIGN: Report of three patients in whom the lumbosacral junctions were successfully restored by spinal instrumentations after total sacrectomies. OBJECTIVES: To describe the surgical technique of the reconstruction of the continuity between the pelvic ring and spinal column by using a transpedicular and iliac screw system. SUMMARY OF BACKGROUND DATA: Although there have been case reports about reconstruction methods after total sacrectomy, biomechanical, and technical problems still remain unresolved. methods: Total sacrectomy was carried out in three cases: two with chordomas and one with a recurrent giant cell tumor. In the first case, reconstruction was achieved with Zielke transpedicular screw and rod system and a sacral rod. The other two patients were reconstructed using a transpedicular and iliac screw system and a sacral rod for bilateral fixation of the iliac wings. In the third patient, the vertical rods were connected to transverse rod with rod connectors. RESULTS: No instrumentation failure was observed, and the continuity between the pelvic wing and spinal column was established with the instrumentation and bone grafting. Although one patient died of metastatic chordoma, the lumbosacral junction was successfully reconstructed with the instrumentation. The other two patients could stand with double crutches 13 and 2 years after surgery, respectively. CONCLUSIONS: Total sacrectomy is a feasible operation for primary malignant tumors involving the entire sacrum. Reconstruction of the union between the lumbar spine and the ilia with spinal instrumentation achieves stabilization suitable for ambulation.
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ranking = 1
keywords = sacrum
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10/26. Computer aided tumor resection in the pelvis.

    Surgical treatment of malignant tumors within the pelvis is a complex problem due to the anatomy and biomechanics. There are standardized preoperative diagnostic tools like computed tomography (CT) or magnetic resonance imaging (MRI) that provide multidimensional information. However, this information cannot be transferred intraoperatively. Computer aided orthopedic surgery (CAOS) may be a solution for precise intraoperative accuracy for these indications. We report on two patients with tumors within the pelvis. In one patient, an infiltrating recurrent chordoma within the sacrum was resected with CAOS. The other patient presented with a periacetabular chondrosarcoma. Resection was done with navigation so precise that a custom-made hemipelvis prosthesis with a special coating fit. In both patients, a complete resection was achieved with tumor-free resection margins. Navigation may be helpful in tumor surgery within the pelvis.
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ranking = 1
keywords = sacrum
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