Myeloblastic leukemia with massive neoplastic infiltration of the skin and mediastinum in a cow.
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An acute myeloblastic leukemia was found in a 3.5-year-old Holstein cow. The neoplasm was characterized by massive tumor growths, and there were multiple tumor nodules in the dermis or subcutis and a large tumor mass in the mediastinum. This tumor showed negative reactivity for CD3, CD79a, major histocompatibility complex class II and myeloid/histiocyte antigen. Ultrastructural features such as dispersed cytoplasmic granules and poorly developed organelles were compatible with those of early promyelocytes. (+info)
Autoimmunity to gephyrin in Stiff-Man syndrome.
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Stiff-Man syndrome (SMS) is a rare disease of the central nervous system (CNS) characterized by chronic rigidity, spasms, and autoimmunity directed against synaptic antigens, most often the GABA-synthesizing enzyme glutamic acid decarboxylase (GAD). In a subset of cases, SMS has an autoimmune paraneoplastic origin. We report here the identification of high-titer autoantibodies directed against gephyrin in a patient with clinical features of SMS and mediastinal cancer. Gephyrin is a cytosolic protein selectively concentrated at the postsynaptic membrane of inhibitory synapses, where it is associated with GABA(A) and glycine receptors. Our findings provide new evidence for a close link between autoimmunity directed against components of inhibitory synapses and neurological conditions characterized by chronic rigidity and spasms. (+info)
Cystic schwannoma presenting as massive hemoptysis in an adult.
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A 62-year-old man who presented with the chief complaint of hemoptysis is reported. A chest radiograph obtained on admission showed a huge cystic mass located at the posterior mediastinum. Prior to surgery, transarterial embolization was done because of continuous massive hemoptysis. An uneventful removal of the tumor was performed, and the pathological diagnosis was schwannoma. The hemoptysis was thought to have been caused by changes in the cystic formation in combination with inflammation which extended to the lung. (+info)
Primary mediastinal malignancies in children: report of 22 patients and comparison to 197 adults.
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PURPOSE: Examine a contemporary series of patients with primary pediatric malignant mediastinal tumors and determine epidemiology, histology, treatment, and survival. Patients and Methods. All malignancies diagnosed between January 1, 1973 and December 31, 1995 were analyzed. RESULTS: Twenty-two patients, age 18 years or less, with pediatric primary mediastinal malignancies were identified from a database of 110,284 patients with primary malignancies. During the same period, 197 adult patients with primary mediastinal malignancies were identified. Fifty-nine percent of the pediatric patients were male. Median age was 11 years. Lymphoma was present in 55%, neurogenic malignancies in 23%, malignant germ cell tumors in 18%, and sarcoma in 5%. Neurogenic tumors presented in infants and lymphomas and germ cell tumors presented in teens (p = 0.005). In treated children, surgery was used more often in neurogenic tumors and germ cell tumors than in lymphomas (p = 0.002). Five-year survival was 74% for lymphomas, 67% for neurogenic tumors, 25% for germ cell tumors, and 61% overall (p = 0.23). Compared to adults, children had more neurogenic tumors (p < 0.001) and fewer thymomas (p = 0.0499). There were no significant differences in staging or survival between children and adults. CONCLUSIONS: Pediatric mediastinal malignancies occurred with a frequency of 1/5,013 patients with malignant tumors. Lymphoma, neurogenic tumors, and germ cell tumors predominated. Compared to adults, children had more neurogenic tumors and fewer thymomas. Within the pediatric group, differences were found in age of presentation between histologic groups. These differences between adults and children, and between infants and teens, should be considered when evaluating a patient suspected of having mediastinal malignancy. (+info)
Preoperative staging of non-small-cell lung cancer with positron-emission tomography.
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BACKGROUND: Determining the stage of non-small-cell lung cancer often requires multiple preoperative tests and invasive procedures. Whole-body positron-emission tomography (PET) may simplify and improve the evaluation of patients with this tumor. METHODS: We prospectively compared the ability of a standard approach to staging (computed tomography [CT], ultrasonography, bone scanning, and, when indicated, needle biopsies) and one involving PET to detect metastases in mediastinal lymph nodes and at distant sites in 102 patients with resectable non-small-cell lung cancer. The presence of mediastinal metastatic disease was confirmed histopathologically. Distant metastases that were detected by PET were further evaluated by standard imaging tests and biopsies. Patients were followed postoperatively for six months by standard methods to detect occult metastases. Logistic-regression analysis was used to evaluate the ability of PET and CT to identify malignant mediastinal lymph nodes. RESULTS: The sensitivity and specificity of PET for the detection of mediastinal metastases were 91 percent (95 percent confidence interval, 81 to 100 percent) and 86 percent (95 percent confidence interval, 78 to 94 percent), respectively. The corresponding values for CT were 75 percent (95 percent confidence interval, 60 to 90 percent) and 66 percent (95 percent confidence interval, 55 to 77 percent). When the results of PET and CT were adjusted for each other, only PET results were positively correlated with the histopathological findings in mediastinal lymph nodes (P<0.001). PET identified distant metastases that had not been found by standard methods in 11 of 102 patients. The sensitivity and specificity of PET for the detection of both mediastinal and distant metastatic disease were 95 percent (95 percent confidence interval, 88 to 100 percent) and 83 percent (95 percent confidence interval, 74 to 92 percent), respectively. The use of PET to identify the stage of the disease resulted in a different stage from the one determined by standard methods in 62 patients: the stage was lowered in 20 and raised in 42. CONCLUSIONS: PET improves the rate of detection of local and distant metastases in patients with non-small-cell lung cancer. (+info)
Natural course of neuroblastoma detected by mass screening: s 5-year prospective study at a single institution.
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PURPOSE: To describe various favorable courses of neuroblastoma (NBL) detected by mass screening and to present our observation program as a temporary treatment option, to be used until a final decision is made regarding the mass screening program for 6-month-old infants. PATIENTS AND METHODS: Between October 1993 and November 1999, 26 of 51 patients with NBL detected by mass screening were enrolled in our observation program. The criteria for observation included urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels less than 50 microg/mg creatinine, smaller tumor size (< 5.0 cm), preoperative status, and granted informed consent. Patients were divided into four groups according to changes in urinary VMA and HVA values and tumor size. Patients who no longer fulfilled criteria underwent surgery. RESULTS: The observation period ranged from 4 to 73 months. Urinary VMA and HVA levels decreased in 19 of 26 patients, often by age 16 months. Eighteen patients had regressing tumors, and in 10 of these cases, the tumor was undetectable or barely detectable by imaging techniques. Four patients younger than 12 months had increased tumor marker levels and tumor volume, histologically reflecting neuroblastic proliferation. The remaining three patients, all older than 18 months, had varied tumor marker levels but increased tumor volume, histologically reflecting an increase in Schwann cells. No upgrading of tumor stage or unfavorable biologic factor was noted in any patient. CONCLUSION: None of our patients showed evidence of transition from favorable to unfavorable prognosis, a finding that points to a reduction in the significance of screening as a public health measure. Until results of ongoing screening trials involving older patients have been evaluated, the observation program can be used as a temporary measure to avoid, with little risk, unnecessary surgical intervention. (+info)
Intracranial dural metastasis of mediastinal seminoma--case report.
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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. (+info)
Mediastinal plasma cell tumor in a sheep.
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An extramedullary plasmacytoma was found in a 10-year-old sheep. The tumor involved the mediastinum, where a 25 x 15 x 10-cm encapsulated mass was found. The lungs had multiple metastases ranging from 0.5 to 2 cm in diameter, and the portal vein contained a 10-cm-long mass. The cytologic and histopathologic analyses were consistent with a moderately differentiated plasmacytoma. The immunophenotype of the tumor cells was lambda light chain IgG+, CD79a-, and CD3-. Occasional granulomas were observed at the periphery of the mediastinal and pulmonary tumors. Microbiologic culture yielded growth of Corynebacterium from these granulomas. This is the first report of plasmacytoma in sheep. The tumor most likely arose from mediastinal lymph nodes and metastasized to the lungs and portal vein. (+info)