Cases reported "Leiomyosarcoma"

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1/50. Primary epithelioid leiomyosarcoma of bone. Case report and literature review.

    We describe an epithelioid leiomyosarcoma of bone located in the right knee of a 51-year-old woman. Plain radiograph and CT scan revealed a poorly defined lytic and destructive mass in the upper metaepiphyseal right tibia which involved surrounding soft tissues. The lesion was composed of proliferating monotonous round cells with a high mitotic activity with scanty intersecting spindle cell fascicles. immunohistochemistry of both areas demonstrated a strong positivity for actin (HHF-35 and alpha-SMA) and vimentin, and negative reactions for desmin, keratin (AE1 AE3), epithelial membrane antigen, S-100 protein, factor viii-related antigen, CD 31 and CD 34. Ultrastructural study confirmed a diagnosis of leiomyosarcoma. This is the first detailed description of the microscopic and radiological features of primary epithelioid leiomyosarcoma of bone.
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2/50. US and CT findings of multicentric leiomyosarcomatosis.

    This article presents a case of leiomyosarcomatosis with widespread lesions involving the soft tissues and the most unlikely organs such as thyroid and salivary glands, pancreas, ligamentum teres, bladder wall, and bones without lymph node or distant metastasis. The CT and US findings of this rare phenomenon are discussed with regard to the literature.
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3/50. leiomyosarcoma of the broad ligament: case report and literature review.

    A 56-year-old woman with leiomyosarcoma of the broad ligament discovered in the outpatient clinic in Marmara University Medical School is presented. Treatment consisted of total abdominal hysterectomy and bilateral salpingo-oophorectomy. After a one-year disease-free interval, the patient underwent a repeat laparotomy and recurrence of the previously excised malignant tumor was removed. The patient received external radiotherapy to the pelvis with linear accelerator.
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4/50. Primary leiomyosarcoma of the pancreas: a case report and review of literature.

    Primary leiomyosarcoma of the pancreas is a rare tumor for which only 21 reports appear in the world literature. We describe an additional case of pancreatic leiomyosarcoma in a 76-year-old man, who complained of persistent high fever. Histologic examination revealed a pleomorphic spindle cell tumor. Reactivity for muscle-specific actin, alpha-smooth muscle actin, and basement membrane components, along with negative staining for epithelial and neural markers, were consistent with a smooth muscle sarcoma. The patient died of disease 1 year after complete surgical excision. This report highlights the need to use a complete antibody panel in order to accurately immunophenotype pleomorphic malignant tumors of the pancreas. A review of the cases compiled in the literature indicates that pancreatic leiomyosarcoma, like its counterpart arising in deep soft tissues, is an aggressive neoplasm characterized by short survival and a high rate of metastases.
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5/50. Extraperitoneal pelvic leiomyosarcoma. MR findings in a case.

    The magnetic resonance imaging (MRI) of a patient with nongynecologic pelvic leiomyosarcoma is presented. A retroperitoneal mass appeared under the broad ligaments, in the right paravesical and parametrial, lateral pararectal site. The mass leaned on the uterus and vagina. On MRI, the mass had solid structure, isointense on T1-weighted images, inhomogeneously iperintense on T2-weighted images with central areas of increased intensity. Late after contrast agent administration, the mass appeared inhomogeneously ipointense with areas of fair late contrast enhancement. The morphology of the tumor, the retroperitoneal site, and MRI sequences make the differential diagnosis. These evidences were useful in treatment planning.
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6/50. Epithelioid leiomyosarcoma of the uterus with oncocytic change.

    We report on a rare case of epithelioid leiomyosarcoma of the uterus with oncocytic change and aggressive clinical behavior. The tumor arose in the left lateral wall of the uterus and measured 22 cm in greatest dimension. Histologically, it consisted of solid sheets of round and polygonal cells displaying a homogeneously eosinophilic cytoplasm and pleomorphic nuclei with prominent nucleoli. Six mitotic figures were found per 10 high-power fields in the most mitotically active areas, and focal tumor cell necrosis was present elsewhere. Under electron microscopy, numerous normal-appearing mitochondria and occasional bundles of microfilaments with focal densities were noted in the tumor cell cytoplasm, and poorly-formed external basal lamina and occasional pinocytic vesicles were found on the cell membrane of some tumor cells.
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7/50. Epithelioid leiomyosarcoma of the external deep soft tissue.

    Epithelioid leiomyosarcoma in the external deep soft tissue is extremely rare. Most epithelioid leiomyosarcomas occur in the uterus. We present a case of epithelioid leiomyosarcoma occurring in the muscle of the thigh of a 78-year-old man. Histologically, the tumor predominantly consisted of round or polygonal cells arranged in sheets with a focal spindle cell component. Immunohistochemical analysis revealed that the tumor cells expressed vimentin, alpha-smooth muscle actin, and alpha-sarcomeric actin. The tumor was negative for desmin, S100 protein, glial fibrillary acidic protein, pan-keratin, epithelial membrane antigen, CAM 5.2, HMB-45, leukocyte common antigen, factor viii-associated antigen, and CD34. Electron microscopically, some tumor cells contained abundant actin-type filaments in their cytoplasm.
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8/50. leiomyosarcoma of the uterus with a florid intravascular component ("intravenous leiomyosarcomatosis").

    A leiomyosarcoma of the uterus in a 54-year-old woman exhibited striking involvement of large vessels of the myometrium and broad ligament on both gross and microscopic examination. The pattern of vascular involvement resembled that seen in intravenous leiomyomatosis. Imaging studies showed recurrent tumor within the inferior vena cava 3 months after hysterectomy. To our knowledge, this is the first reported such case in the literature, for which we propose the designation intravenous leiomyosarcomatosis of the uterus.
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9/50. Peritoneal washing cytology findings of disseminated myxoid leiomyosarcoma of uterus: report of a case with emphasis on possible differential diagnosis.

    We report the cytology findings of a rare case of myxoid leiomyosarcoma of the uterus with intraabdominal dissemination. The cytology showed uniformly dispersed spindly to polygonal "epithelioid" tumor cells focally linked by background matrix. Spindly tumor cells attaching to and radiating from branching capillary structures were also present. Occasionally, scattered "signet-ring" cells were found, mimicking metastatic poorly differentiated adenocarcinoma. The background mucoid substance was more conspicuous in the cell block sections. Gross and histologic examination of the surgical specimen revealed a large uterine leiomyosarcoma with prominent myxoid change. Ultrastructural study showed that the "signet-ring" appearance was mainly due to degenerative cytoplasmic change with ballooning of mitochondria, dilatation of endoplasmic reticulum, and elevation of outer nuclear membrane. In contrast to other spindle cell malignancies such as sarcomatoid mesothelioma, sarcomatoid carcinoma, or malignant melanoma, true sarcoma cells in general carry a distinctive cytologic appearance in washing/effusion fluid. In a correct clinical setting, the peculiar association with mucoid matrix and absence of classic lipoblasts should also raise the suspicion of metastatic myxoid leiomyosarcoma.
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10/50. Pleomorphic leiomyosarcoma of the adrenal gland.

    Primary leiomyosarcomas arising in the adrenal gland are exceedingly rare, with only 3 cases reported in the literature. We present the clinical, morphologic, and immunohistochemical features of a pleomorphic leiomyosarcoma, a variant of leiomyosarcoma that has not been described in the adrenal gland. A 63-year-old man presented with a 1-year history of enlarging right upper quadrant mass and pulmonary nodule. A diagnosis of metastatic pulmonary carcinoma to the adrenal gland was rendered on a needle biopsy specimen. Preoperative chemotherapy reduced only the pulmonary mass but not the adrenal mass, which continued to enlarge. Documented by computed tomography and confirmed at surgery, the tumor had completely replaced the right adrenal gland, invading into both the posterior aspect of the right liver and the superior pole of the right kidney. Histologic sections showed a diffuse proliferation of pleomorphic, large, and polygonal neoplastic cells with prominent nucleoli. Many bizarre mitotic figures were present. The neoplastic cells were strongly positive for desmin, calponin, and vimentin. Approximately 80% of the neoplastic cells were positive for the proliferation marker Ki-67. They were negative for smooth muscle actin, muscle-specific actin, myoglobin, myogenin, CD117, cytokeratins, carcinoembryonic antigen, epithelial membrane antigen, chromogranin, CD34, CD31, S100 protein, and HMB-45.
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