Cases reported "Leiomyosarcoma"

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11/63. Unusual response to second-line single-agent gemcitabine in locally advanced primary leiomyosarcoma of the lung: a case report.

    Primary leiomyosarcomas (LMSs) of the lung are extremely rare malignancies that have been the subject of single or small series of case reports. Today, the gold standard of treatment in patients with locally advanced and metastatic disease includes one of the many possible regimens containing an anthracycline and/or ifosfamide. Few chemotherapy agents are active in the second-line setting. In particular, gemcitabine is considered quite ineffective in the treatment of first- as well as second-line chemotherapy of soft tissue sarcoma and responses to this agent are seldom reported. In this paper, we report a single patient with primary LMS of the lung previously treated with a combination of epirubicin and ifosfamide. The patient responded to second-line chemotherapy with gemcitabine 1250 mg/m2 given as a 30-minute infusion on days 1, 8, and 15 of a 28-day cycle and showed an 8-month response duration and negligible toxicity. Gemcitabine may represent an alternative to the best supportive care in patients affected with soft tissue sarcoma who fail first-line chemotherapy.
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12/63. Unicentric Castleman's disease with leiomyosarcoma: a rare association.

    A 54-year-old asymptomatic woman was first diagnosed with unicentric Castleman's disease at 51 years of age. The disease was identified incidentally behind the inferior vena cava after a computed tomography (CT) scan. She was followed conservatively and no enlargement of the mass was observed. However, a follow-up CT scan revealed another solid mass in the left retroperitoneal area. Both tumors were surgically removed in the same session. The retrocaval mass was histologically confirmed to be Castleman's disease of the hyaline vascular type. Histological examination of the left peritoneal mass revealed low-grade leiomyosarcoma. We present here a case of leiomyosarcoma associated with unicentric Castleman's disease, both of which were localized in the retroperitoneum.
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13/63. Dumbbell-shaped leiomyosarcoma of the inferior vena cava with foci of rhabdoid changes and osteoclast-type giant cells.

    An inferior vena cava (IVC) tumor was incidentally found in a 67-year-old Japanese man. The resected tumor was lobulated and multinodular, measuring 14.0 x 6.5 x 7.0 cm, showing a dumbbell-shaped appearance with a central constriction. The tumor showed both intra- and extra-luminal growth. The tumor was primarily composed of well-differentiated leiomyosarcoma. Spindle tumor cells in the well-differentiated area were positive for vimentin, muscle actin, alpha-smooth muscle actin, and desmin. Foci of rhabdoid cells and osteoclast-type multinucleated giant cells were also found. Rhabdoid cells ultrastructurally had paranuclear aggregates or whorls of intermediate filaments that were positive for vimentin, low molecular weight cytokeratin, and desmin. Osteoclast-type multinucleated giant cells were positive for only CD68 antigen, suggesting a reactive histiocytic lineage. To the best of our knowledge, this is the first case of IVC leiomyosarcoma accompanied by both rhabdoid tumor cells and osteoclast-type reactive multinucleated giant cells. These unusual features should be kept in mind in the diagnosis of dumbbell-shaped retroperitoneal tumors that involve the IVC.
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14/63. leiomyosarcoma of the rectum. A report of two cases and review of the literature.

    Rectal leiomyosarcoma is rare. It is frequently treated by abdominoperineal resection. The role of adjuvant therapy is unclear. Two cases of rectal leiomyosarcoma are presented. Pre-operative endorectal ultrasound predicted submucosal invasion in one case treated by low restorative resection and muscularis propria infiltration in the other managed with abdominoperineal resection. In both cases, ultrasound suggested malignant characteristics as evident by heterogeneous cystic spaces, irregular outline, large size and echogenic foci. histology was CD34 negative and desmin, alpha-smooth muscle actin and HHF-35 positive, distinguishing these tumours from gastrointestinal stromal tumours. Rectal leiomyosarcoma may be successfully treated by restorative resection and pre-operative ultrasound is useful in assisting this surgical decision by defining malignant features of the tumour. Histopathological characteristics predictive of poor prognosis include high mitotic activity, intratumoural necrosis and tumour size.
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15/63. MR imaging of malignant mesenchymal tumors of the liver.

    Magnetic resonance (MR) features of five primary malignant mesenchymal neoplasms (plasmocytoma, leiomyosarcoma, undifferentiated sarcoma, epithelioid hemangioendothelioma, and angiosarcoma) of the liver were reported. All tumors were hypointense on T1-weighted images and hyperintense on T2-weighted images. No halo and intravenous extension were noted. A target appearance was revealed in epithelioid hemangioendothelioma. MR findings of angiosarcoma were essentially the same as those of cavernous hemangiomas (markedly hyperintense with hypointense linear septa on T2-weighted images). MR findings of these rare hepatic malignancies were nonspecific, although they were quite different from those of typical hepatocellular carcinomas. This study suggested that MR differentiation of primary hepatic mesenchymal tumors from other common benign and malignant neoplasms was difficult; however, the number of studied cases was limited.
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16/63. Histogenesis of carcinosarcoma and establishment of leiomyosarcoma cell line (HTMMT) derived from human uterine carcinosarcoma.

    A cell line designated HTMMT was established from the human uterine carcinosarcoma (composed of leiomyosarcoma and adenocarcinoma) of a 66-year-old Japanese woman. The cell line grew well and 83 serial passages were successively done within 24 months. The cell line contained spindle- or fibrous-shaped cells that revealed neoplastic and pleomorphic features, and multipled rapidly without contact inhibition. These cells were characterized as possessing myofibrils. The karyotype exhibited hyperploidy and the chromosome number was ranged from 87 to 100. The cells were transplanted into an immune-depressed hamster cheek pouch or into nude mouse skin, but produced no tumors. We supported the combination theory for the histogenesis of the carcinosarcoma.
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17/63. leiomyosarcoma of the submandibular gland. Report of a case and review of the literature.

    This report describes the first case of primary leiomyosarcoma of the submandibular salivary glands and emphasizes the role of immunohistochemical study for a correct diagnosis of this tumour. In line with results of international literature, we associated surgery with radiotherapy and 2 years postoperatively there was no sign of recurrence.
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18/63. Primary extradural epithelioid leiomyosarcoma of the cervical spine: case report and literature review.

    OBJECTIVE AND IMPORTANCE: No case of primary epithelioid leiomyosarcoma involving the spine has been reported previously. CLINICAL PRESENTATION: A 61-year-old Nigerian woman presented with progressive spastic quadriparesis and acute urinary retention. Her only medical history included a total abdominal hysterectomy for fibroids 10 years earlier in nigeria. Results of the general examination were normal. Pyramidal spastic quadriparesis (3/5) with a sensory level at C5-C6 was found neurologically. magnetic resonance imaging scans of the brain and spine revealed extradural cord compression at C3-C5 as the sole abnormality. This was caused by a large, soft tissue mass arising from the erector spinae muscles, which was predominantly isointense on T1-weighted images, of mixed intensity on T2-weighted images, and homogenously enhanced after gadolinium contrast agent administration. There was an associated signal change in the cord at C3-C4. Computed tomography confirmed the predominantly soft tissue involvement, but with bone erosion and infiltration within the posterior elements of C4. Systemic screening for cancer was negative. INTERVENTION: At decompressive laminectomy, urgently undertaken under corticosteroid cover, an excessively vascularized, soft tissue tumor was subtotally excised, after which independent walking and normal sphincter function were regained within 1 week. Four weeks later, a complete macroscopic tumor excision was undertaken, incorporating lateral mass (C3-C6) and C2 pedicle screw stabilization, along with iliac crest bone grafting. The patient's neurological status continued to improve. However, while awaiting radical radiotherapy, the patient declined further treatment and returned to her native nigeria. Histopathological findings were consistent with an epithelioid leiomyosarcoma. CONCLUSION: This is the first reported case of a primary craniospinal epithelioid leiomyosarcoma.
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19/63. Stereotactic intensity-modulation radiation therapy for vertebral body and paraspinal tumors.

    Therapeutic doses of radiation for paraspinal tumors are often limited by the dose-related tolerance of the spinal cord. Intensity-modulated radiation therapy (IMRT) is an advanced form of three-dimensional conformal radiation therapy that provides improved coverage of tumor volumes while reducing the radiation dose to the spinal cord. Computer-controlled multileaf collimation provides high conformality, which makes it feasible to treat tumors of any shape, even those that are wrapped around the spinal cord. The use of a newly developed, noninvasive body frame, the capability of fusing computerized tomography and magnetic resonance images, and on-line portal films provide precise target immobilization and target identification. In this paper the authors discuss their preliminary experience in six cases in which IMRT was used to treat paraspinal lesions in patients who harbored locally recurrent tumors and/or tumors that previously received the maximum doses of radiation that could be tolerated by the spinal cord.
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20/63. Primary leiomyosarcoma of the great saphenous vein: case report.

    OBJECTIVE: To describe a case of primary leiomyosarcoma of the great saphenous vein. PATIENT RECORD: A 59-year-old Chinese lady presented with two painful lumps in the right thigh in the line of the great saphenous vein. At surgery, in September 2004, two tumors in the right great saphenous vein above the knee were excised with the intervening normal vein. Histopathological studies confirmed both masses as leiomyosarcoma. radiotherapy was given postoperatively. The patient has been followed-up for 300 days after surgery with no evidence of local or distant metastasis. CONCLUSION: leiomyosarcoma of the great saphenous vein can exist at more than one site. Tumor resection and radiotherapy was associated with good patient prognosis.
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