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1/595. dermatofibrosarcoma protuberans in an infant with a history of choroid plexus papilloma.

    We describe an infant with a dermatofibrosarcoma protuberans (DFSP) who also had a choroid plexus papilloma. This report underscores the occurrence of DFSP in this age group and may support a neural pathogenesis of this tumor.
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ranking = 1
keywords = sarcoma
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2/595. Multifocal angiosarcoma of the scalp: a case report and review of the literature.

    Multifocal angiosarcoma of the scalp is uncommon. Its risk factors include a history of radiotherapy and possibly sun exposure. Angiosarcoma is an aggressive tumor with a high propensity for both local recurrence and distant metastasis. overall, the prognosis is poor. The size of the lesion is an important prognostic factor. Treatment modalities include surgery with wide margins (with or without adjuvant therapy) and multidrug chemotherapy, followed by radiotherapy. The local failure rate is high.
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ranking = 1.2
keywords = sarcoma
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3/595. A case of synchronous double primary lung cancer with neuroendocrine features.

    We report a case of unique double primary lung cancers with neuroendocrine features in a 63-year-old male smoker. The mass in the left lower lobe (LLL) was a small cell/large cell carcinoma with spindle cell sarcomatous areas and organoid structure. The mass in the left upper lobe (LUL) was a tubular adenocarcinoma with neuroendocrine features including organoid nests showing occasional rosette formation, nuclear palisading in the periphery of the nests and positive immunoreaction for CD56, chromogranin a and synaptophysin. The difference in histological structures between the two masses led us to diagnose double primary lung cancer. The combination of small cell lung carcinoma and spindle cell carcinoma is very uncommon. The relationship between LLL and LUL tumors remains unclear. Multiple lung cancers with neuroendocrine features have only rarely been reported in the literature. The patient in our case died of widespread cancer 2 years and 4 months after the surgery without adjuvant chemotherapy, a longer postoperative survival time than in cases of ordinary extensive small cell lung cancer. Multiple lung cancers with neuroendocrine features are extremely rare and similar cases have not been reported in the literature. Neuroendocrine differentiation has attracted widespread attention and, therefore, examining neuroendocrine features in lung cancers is important.
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ranking = 0.2
keywords = sarcoma
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4/595. osteosarcoma arising in a solitary osteochondroma of the fibula.

    We present a case of osteosarcoma arising in an osteochondroma of the right fibula in a 30-year-old woman. The available radiographic studies of the lesion were not suggestive of malignant transformation. The lesion and underlying bone were excised. Histologic examination showed a conventional high-grade osteoblastic osteosarcoma that focally eroded the fibrocartilaginous cap. The patient received postoperative chemotherapy and shows no evidence of disease 27 months following operation. The occurrence of osteosarcoma in an osteochondroma is an extremely rare event, and only a few cases are on record in the literature.
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ranking = 1.4
keywords = sarcoma
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5/595. Coexistent gastric MALT lymphoma and Kaposi sarcoma in an hiv positive patient.

    A 47 year old hiv positive male presented with haematemesis and epigastric pain. A gastrectomy was performed for intractable bleeding. The cause of the haematemesis proved to be a Kaposi sarcoma of the stomach which had resulted in mucosal ulceration. Several other smaller foci of Kaposi sarcoma were also present. Coexistent with the Kaposi sarcoma was a dense lymphoid infiltrate with lymphoid follicles and reactive germinal centres. Centrocyte-like cells caused marked effacement and destruction of gastric glands with the formation of lymphoepithelial lesions, typical of a MALT lymphoma. These cells were of B cell lineage and some expressed the hiv antigen, p24. Follicular dendritic cells and macrophages within germinal centres were also p24 positive. immunohistochemistry and in situ hybridisation did not detect Epstein-Barr virus. Although helicobacter pylori was not identified by light microscopy in the sections sampled, this does not preclude its possible role, with other cofactors such as hiv, in the causation of the MALT lymphoma.
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ranking = 1.4
keywords = sarcoma
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6/595. Colonic adenomas with extramedullary myeloid tumor (granulocytic sarcoma).

    Extramedullary myeloid tumor (EMT) is an accumulation of malignant immature cells of the granulocytic series that is usually green in appearance due to the presence of myeloperoxidase. These invasive and destructive tumors occur most commonly in the skull and surrounding tissues, lymph nodes, skin and soft tissues. Regardless of the site, EMTs are difficult to recognize and may be easily overlooked or diagnosed as malignant lymphoma. EMTs may precede the diagnosis of a chronic myeloproliferative disorder or acute myeloid leukemia, may present coincident with the hematologic diagnosis, or may herald a relapse after therapy. An accurate diagnosis of EMT is of great clinical importance in the ongoing management of hematologic malignancies. We report here two unusual cases of EMT of the colon, which infiltrated adenomatous polyps. We conclude that increased cellularity within the lamina propria of polyps and mucosal surfaces in general should be carefully examined.
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ranking = 0.8
keywords = sarcoma
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7/595. Collision of uterine rhabdoid tumor and endometrioid adenocarcinoma: a case report and review of the literature.

    Extrarenal malignant rhabdoid tumors have been reported in a variety of anatomic sites but infrequently in the female genital tract. In the uterus, they have been described as a pure tumor, in association with endometrial stromal sarcomas, and as a component of a malignant mullerian mixed tumor. This study reports an unusual uterine neoplasm in a 49-year-old woman, in which a malignant rhabdoid tumor occurred as a collision tumor with a well-differentiated endometrioid adenocarcinoma. The tumor was a 14-cm polypoid mass that filled the endometrial cavity. The two neoplastic components were distinct on microscopic and immunohistochemical examination. Ultrastructural examination confirmed the rhabdoid phenotype of the sarcomatous component. The patient died of disease 4 months after diagnosis with progression of the malignant rhabdoid tumor. The highly aggressive behavior of the rhabdoid (i.e., nonepithelial) component in this collision tumor lends support for a distinction of this neoplasm from a malignant mullerian mixed tumor, with which it may be confused.
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ranking = 0.4
keywords = sarcoma
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8/595. Synchronous Paget's sarcoma of tibiae in which Paget's disease was limited to these bones.

    A 51-year-old native of Rio de Janeiro presented with bilateral synchronous Paget's sarcomas in the tibiae, which developed in the upper right tibia and in the distal third of the left tibia. There were no other areas of Paget's disease. The largest tumor spread to the right inguinal nodes and also soft tissue. The tumor in the left tibia spread dramatically in the soft tissues up the leg and only involved the medullary cavity in its inferior portion. The patient died, but there was no autopsy. Comments are made about the prevalence of Paget's disease in Rio de Janeiro.
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ranking = 1
keywords = sarcoma
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9/595. High grade, synchronous colon cancers after renal transplantation: were immunosuppressive drugs to blame?

    Recipients of renal transplants are known to have an increased incidence of cancer, which is believed to be related to the use of immunosuppressive drugs used to prevent rejection. Although the risks of lymphoma and Kaposi's sarcoma are clearly increased in this setting, the association with colon cancer is controversial. We report a 44-yr-old woman, 20 yr post-renal transplant, and with no family history of colorectal cancer or polyps, who was found to have synchronous, poorly differentiated colon cancers associated with extensive abdominal lymph node, bone marrow, and bone (skull) metastasis. The long term immunosuppressive drugs that she had received may have been an important factor in her tumor development and/or progression. Our case and literature review suggest a possible mild, increased risk of colon cancer development in patients after renal transplantation.
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ranking = 0.2
keywords = sarcoma
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10/595. Fine structure of a radiation-induced osteogenic sarcoma.

    An osteogenic sarcoma arose in the right orbit of a 7-year-old boy some 5 years after the right orbit had been treated by four courses of radiotherapy (total dose approximately 13,000 rads) for a multicentric retinoblastoma. death occurred 6 months after the orbital tumor was first detected. Study of the orbital tumor by electron microscopy revealed a cell population of varied morphology in which two main types were identified. In one group, the cells were large with radiolucent cytoplasm, which contained long branching segments of rough endoplasmic reticulum. In the second group, the cells were smaller with irregular nuclei and an electron-dense cytoplasm, which contained short segments of dilated rough endoplasmic reticulum and numerous mitochondria. The first group of cells closely resembled osteoblasts, while the second group had some features of osteoclasts or their percursors. The branching processes of the tumor cells were separated by an amorphous ground substance, which contained collagen-like fibrils and hydroxyapatite crystals. Crystal deposition was in some instances in close relation to extracellular membrane-bound vesicles.
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ranking = 1
keywords = sarcoma
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