Pathology, research and practice
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Pulmonary artery sarcoma is a rare and highly lethal disease whose clinical findings are often indistinguishable from those of chronic thromboembolic pulmonary hypertension. Partial improvement after thrombolytic therapy has suggested that thromboembolic phenomena may be superimposed on the tumor, but, to date, a well-documented statement of these events has not been provided.
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A large intra-abdominal mass was discovered in a 6-month-old boy during a routine well-child examination. Imaging studies revealed a solid mass which appeared to arise from the left lobe of the liver, extending caudally and filling the entire pelvis. At the time of surgical excision, the mass was found to be unassociated with the liver, but was instead localized to the omentum. ⋯ Cancer Genet. Cytogenet. 168 (2006) 150-154]. We report this unique case of an omental lipoblastoma with a focus on its unusual karyotype, as well as its differentiation from myxoid liposarcoma.
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Pathol. Res. Pract. · Jan 2008
Lymphocyte sub-populations and non-Langerhans' cell monocytoid cells in pulmonary Langerhans' cell histiocytosis.
Pulmonary Langerhans' cell histiocytosis (PLCH) is a disease characterized by the occurrence of complex fibro-cellular interstitial lesions dominated by Langerhans' cells (LC), which occurs predominantly in young adult smokers. We undertook this retrospective study to better define the lymphohistiocytic cell populations in PLCH in order to obtain a greater insight into its pathogenesis. Formalin-fixed, paraffin-embedded, surgically excised, archival lung tissue from seven patients (two males, five females; average age 34.9 years) was immunostained with a panel of antibodies for lymphohistiocytic markers: CD1a, CD3, CD4, CD8, CD15, CD20, CD56, TIA-1, CD68-PGM1, Mac387, and mast cell tryptase. ⋯ Our results showed a predominance of CD8+, TIA-1+ cytotoxic T lymphocytes among the lymphocyte subsets which appear to interact with LC and AM in PLCH lesions. A small sub-population of NLMC was also present. Further studies are required to better define and to evaluate the role of cytotoxic T cells and NLMC in the pathogenesis of PLCH.
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Cylooxygenase-2 (Cox-2) inhibitors have increasingly become therapeutic alternatives in some Cox-2-overexpressing neoplasms. As the treatment eligibility for these drugs hinges on Cox-2 expression, Cox-2 immunostaining has recently been widely examined in several malignant neoplasms. However, data on the expression of Cox-2 in Wilms' tumor (WT) are limited. ⋯ The results of this study demonstrated that Cox-2 was ubiquitously expressed in all cases of WT and their neovasculature, independently of the type of neoplasm (tumors with a favorable or unfavorable histology), tissues which constitute the neoplasm (blastemal, mesenchymal and epithelial, heterologous or non-heterologous elements), patient age, sex, or stage of development and survival rate. Thus, Cox-2 inhibitors could be used for treating all cases of WT. Further studies, including molecular investigations, would be useful to confirm our hypotheses.
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Pathol. Res. Pract. · Jan 2007
Multicenter StudyProtein expression of KIT and gene mutation of c-kit and PDGFRs in Ewing sarcomas.
Ewing sarcoma is a highly malignant tumor of bone preferentially arising in children and young adults. Its 5-year survival rate is only 50% despite the use of multimodal therapeutic approaches, requiring a search for new therapeutic targets and the development of novel therapeutic modalities. KIT and PDGFRs are type III receptor tyrosine kinases, and activating mutations in c-kit (which encodes KIT) and PDGFRs have been reported as oncogenic events in many malignancies. ⋯ Our results for KIT protein expression agree with those of previous studies. This is the largest series of c-kit mutational analysis in Ewing sarcoma to date, and the results definitively show that c-kit activating mutations are not coincident with KIT protein expression in Ewing sarcoma in most samples. These findings imply other mechanisms for KIT activity and leave open the question of whether imatinib would be efficacious in the treatment of Ewing sarcoma.