Cases reported "Mediastinal Neoplasms"

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1/18. Extraskeletal osteosarcoma of the mediastinum after treatment of a mediastinal germ-cell tumor.

    Three years after four cycles of bleomycin, etoposide, and cisplatin (BEP) chemotherapy for a nonseminomatous germ-cell tumor of the mediastinum followed by complete resection of residual teratoma in a 21-year-old man, a mediastinal recurrence was diagnosed as an extraskeletal osteosarcoma. After unsuccessful chemotherapy and removal of the tumor, the patient died of cerebral metastases. Histologic transformation of the teratomatous components of nonseminomatous germ-cell tumors is an uncommon phenomenon showing a particular aspect of germ-cell tumor biology. We review the literature and discuss the pathogenesis concerning this subject.
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2/18. Intracranial dural metastasis of mediastinal seminoma--case report.

    A 24-year-old male presented with a rare intracranial dural metastasis from a mediastinal germ cell tumor infiltrating the superior vena cava, pericardium, ascending aorta, and lung. Routine chest radiography incidentally revealed the abnormal mediastinum, which was partially resected after median sternotomy, followed by radiotherapy. One year after the radiotherapy, magnetic resonance imaging revealed a right parasagittal tumor mimicking a meningioma. Partial tumor removal was performed. Two cycles of chemotherapy with carboplatin and etoposide were then performed, and the residual tumor almost completely disappeared. The histological diagnosis of both the mediastinal and intracranial tumors was pure seminoma. Sections of metastatic tumor revealed several thick fibrous septa and numerous epithelioid granulomas infiltrated by plasma cells, intermingled between the clusters of tumor cells. Follow up of patients apparently in remission from mediastinal seminoma without evidence of advanced or recurrent disease at other sites is recommended.
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3/18. Effective therapy of a vascular tumor of infancy with vincristine.

    Vascular tumors are common in infancy, affecting as many as 10% of children. These lesions often follow a benign course, with an initial proliferative phase followed by spontaneous involution, and require no therapy. Others manifest explosive early growth and Kasabach-Merritt phenomenon, requiring therapeutic intervention. Occasionally, some bulky tumors threaten life or vision because of mass effect, also mandating intervention. steroids are the mainstay of therapy, but often are ineffective. Interferon alpha (2a and 2b) has been used as second-line therapy in cases of steroid failure. However, interferon therapy has been associated with a significant incidence of spastic diplegia. The authors present the case of a 3-month-old girl in whom respiratory distress secondary to tracheal compression developed. magnetic resonance imaging and magnetic resonance angiography showed a large cervicothoracic lesion encasing the great vessels and displacing the airway. She did not display associated Kasabach-Merritt phenomenon. The lesion proved refractory to standard steroid therapy, but responded dramatically to 4 cycles of vincristine (0.05 mg/kg). Although this agent has been used in children with life-threatening Kasabach-Merritt phenomenon, this is the first time it has been described in the setting of compromised vital function. vinca alkaloids recently have been shown to have potent antiangiogenic activities in experimental models. Given the low predicted incidence of side effects at this dose, vincristine used as an antiangiogenic agent may prove an attractive alternative therapy for patients with life-threatening vascular tumors of infancy.
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4/18. TIA-1 cytotoxic large T-cell lymphoma of the mediastinum: case report.

    A 52-year-old previously healthy Caucasian woman presented with superior vena cava syndrome, secondary to compression of a bulky anterior mediastinal mass involving the right lung. Fine-needle aspiration biopsy of the mediastinum yielded large epithelioid cells intermingled with small mature lymphocytes. The epithelioid cells are LCA positive, expressing cytoplasmic CD3 diffusely and TIA-1 focally, but negative for EMA, CD4, CD8, CD15, CD20, CD30, and CD56. The TIA-1 cytoplasmic granules correlated to the azurophilic granules in Diff-Quik-stained cells, pink granules in Ultrafast Papanicolaou-stained cells, and dense core granules in electron microscopy. in situ hybridization for Epstein-Barr viral rna was negative. The background small lymphocytes were composed of a majority of CD4 t-lymphocytes and minority of CD8 t-lymphocytes. The patient responded well to six cycles of CHOP chemotherapy, followed by radiation with a total dose of 4140 cGy delivered to the mediastinum in 23 fractions. On the chest X-ray taken 6 mo later, there was minimal apical fibrosis with no evidence of an acute intrathoracic pathology. To the best of our knowledge, this case may be the first report of cytotoxic large T-cell lymphoma of the mediastinum.
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5/18. Mediastinal seminoma in a patient with wiskott-aldrich syndrome.

    Shortness of breath developed in an 18-year-old man with wiskott-aldrich syndrome, and he was found to have a large mediastinal mass. The gallium scan was positive, and biopsy indicated a seminoma. After treatment with four cycles of chemotherapy, the mass completely resolved. Despite severe thrombocytopenia, he required only two platelet transfusions during therapy. Although lymphomas make up the vast majority of mediastinal tumors in patients with wiskott-aldrich syndrome, a positive gallium scan should not preclude the diagnosis of seminoma or the need for confirmatory tissue diagnosis. This report shows the possibility of uneventful and successful treatment of malignancy in a patient with wiskott-aldrich syndrome and severe thrombocytopenia.
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6/18. Unusual cardiac reaction to chemotherapy following mediastinal irradiation in a patient with Hodgkin's disease.

    A 27 year old man with Hodgkin's disease experienced three separate episodes of chest pain, each occurring on the sixth day of a cycle of mustargen, oncovin, procarbazine, prednisone (MOPP) combination chermotherapy. The first episode appeared to represent a myocardial infarction, whereas the next two were less serious. Numerous studies were performed including coronary angiography, cardiac catheterization and open pericardial biopsy. It is suggested that the patient represents an example of a previously undescribed syndrome due to chemotherapy administered after cardiac irradiation.
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7/18. Mediastinal germ cell tumor associated with histiocytic sarcoma of spleen: case report of an unusual association.

    We present an unusual association of mediastinal germ cell tumor containing seminoma and angiosarcoma components and splenic histiocytic sarcoma. A 15-year-old boy presented with chest pain. Histopathologically, an anterior mediastinal mass contained typical seminoma, immature teratoma, embryonal carcinoma, angiosarcoma, yolk sac tumor, and polyembryoma. An abdominal ultrasonogram revealed a huge splenomegaly with multiple ill-defined low echogenic nodules, 1 month after the second cycle of chemotherapy. Histopathologically, large, round-to-oval tumor cells with abundant eosinophilic cytoplasm often contained eccentrically placed nuclei with vesicular chromatin and an irregular nuclear membrane. The tumor cells were immunoreactive for CD68, CD31, and CD4. The cytogenetic results showed deletion of the long arm of chromosome 5 and trisomy 8. This lesion might have been on the pathway of multistep tumorigenesis toward a final leukemia.
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8/18. Pneumomediastinum as a complication to treatment of mediastinal (thymic) large B-cell lymphoma.

    Mediastinal (thymic) large B-cell lymphoma (Med-DLBCL) is a subtype of diffuse large B-cell lymphomas (DLBCL) with a typical radiological appearance of bulky anterior mediastinal mass, often with areas of necrosis. We report a case of Med-DLBCL with unusual radiological findings and clinical development. Computed tomography (CT) obtained at presentation revealed a huge anterior mediastinal tumor with an axial diameter of 180 mm. Nineteen days after the first cycle of chemotherapy, chest radiography and CT revealed large areas of tumor necrosis and pneumomediastinum with air-fluid levels. To our knowledge, air-fluid levels inside Med-DLBCL have not been previously described. This finding, in combination with necrotic sputum, may indicate communication between the tracheobronchial tree and the tumor.
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9/18. Midline carcinoma with t(15;19) and BRD4-NUT fusion oncogene in a 30-year-old female with response to docetaxel and radiotherapy.

    BACKGROUND: Poorly differentiated midline carcinoma with a translocation between chromosomes 15 and 19, i.e. t(15;19), has been recognized as a distinct clinical entity for over a decade. This tumor affects young individuals, shows a rapidly fatal clinical course despite intensive therapy. The t(15;19) results in the fusion oncogene BRD4-NUT. Information concerning treatment of this rare disorder is scarce. CASE PRESENTATION: A 30-year-old woman was admitted with a rapidly progressing tumor in the mediastinum, cervical lymph nodes, vertebral column and the epidural space. Pathological, cytogenetic, FISH and PCR analysis revealed a glycogenated carcinoma rarely expressing cytokeratins and showing t(15;19) and BRD4-NUT gene rearrangement. The patient was initially treated with a Ewing sarcoma chemotherapy regimen, but had rapid progression after two cycles. She then received docetaxel and radiotherapy, which resulted in almost complete disappearance of the tumor. CONCLUSION: Docetaxel may be considered for initial chemotherapy in young patients presenting with a midline carcinoma with bone marrow involvement and cytogenetic and molecular genetic finding of a t(15;19)/BRD4-NUT-rearrangement. We herein describe, in detail, the laboratory methods by which the BRD4-NUT -rearrangement can be detected.
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10/18. Neuron-specific enolase and thymidine kinase as an aid to the diagnosis and treatment monitoring of small cell lung cancer.

    The serum levels of neuron specific enolase (s-NSE) and thymidine kinase (s-TK) were studied in detail in patients with small cell lung cancer (SCLC) to evaluate as to whether their combined use may aid to diagnosis and follow-up of this particular tumor. Only s-NSE could differentiate between SCLC and non-small cell lung cancer (NSCLC) or benign pulmonary diseases (BPD) to some extent, pathologic serum concentrations occurring in 81%, 17%, and 0%, respectively. The comparable figures for s-TK were 62%, 24%, and 28%, respectively. serum NSE decreased and increased paralleling tumor regression and progression, respectively, except when brain metastases were present. Alterations of s-TK, in contrast, did not usually mirror the course of disease. During initial chemotherapy (CT) a transitory increase of serum levels was observed for both NSE and TK. Monitoring, based on daily blood samples, showed comparable peaks only for s-TK during the following CT cycles, whereas s-NSE was within the normal range even when tumor mass was still present. Those subsequent s-TK peaks under CT may be due to tumor cell lysis as a result of CT indicating the efficacy of treatment by this way. Rapidly proliferating tissues such as bone marrow or bowel mucosa, however, have also to be considered as possible sources of s-TK.
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