Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Poorly differentiated rhabdomyosarcomas are traditionally distinguished from lymphomas by their absence of lymphoid markers such as immunoglobulin or CD20 expression. We have encountered three alveolar rhabdomyosarcomas that were initially diagnosed as lymphoid neoplasms because of the expression of a lymphocytic phenotype in morphologically undifferentiated tumor cells. Subsequent cytogenetic analysis revealed a t(2; 13) in two cases. All cases recurred in the chest wall and showed positivity for muscle markers, such as muscle-specific actin, myoglobin, MyoD1, and/or desmin on subsequent immunohistochemistry. The findings in these three cases lead us to conclude that the presence of a lymphoid phenotype does not absolutely exclude the diagnosis of rhabdomyosarcoma.
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PMID:Undifferentiated rhabdomyosarcoma with lymphoid phenotype expression. 902 9

A 15-month-old castrated male dog with a history of intermittent epistaxis and sneezing was admitted for the examination of a maxillofacial mass. An impression smear of a biopsy sample from the cauliflower-shaped gingival mass contained numerous round cells, 5-25 microm in diameter, which contained a moderate amount of clear to pale blue cytoplasm and resembled lymphoid cells. Mitotic figures were frequently observed. The mass was diagnosed as malignant round cell neoplasia. On histologic examination the tumor was composed of diffusely arranged, small, atypical round cells with a small amount of fibrovascular stroma. Immunohistochemically, the cells were negative for CD3, CD18, CD20, CD79alpha, cytokeratin, melan-A, chromogranin A, alpha-smooth muscle actin, and myoglobin but positive for vimentin and desmin. The cells also had strong positive nuclear staining for myogenin and MyoD1. A diagnosis of solid-pattern alveolar rhabdomyosarcoma was made on the basis of morphologic and immunohistochemical results. Alveolar rhabdomyosarcoma should be considered in the differential diagnosis of tumors in juvenile dogs, especially when cytologic findings reveal round, undifferentiated cells.
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PMID:Cytologic, histologic, and immunohistochemical features of maxillofacial alveolar rhabdomyosarcoma in a juvenile dog. 1964 42

The aim of this study was to determine the histological spectrum of operated cardiac tumors, excluding myxoma, at a tertiary center in India. Between 1995 and 2010, we encountered 188 cases of operated cardiac tumors that had been subjected to histopathological examination. Morphological characterization was done by light microscopy along with histochemical stains. Immunohistochemistry using a panel of antibodies, i.e., vimentin, desmin, myogenin, smooth muscle actin (SMA), epithelial membrane antigen (EMA), cytokeratins, factor VIII-related antigen, S100-protein, synaptophysin, chromogranin, Bcl2, MIB-1, leukocyte common antigen (LCA), CD 3, CD20, CD34, and CD 99 (MIC-2) was performed wherever applicable. Out of the 188 cases, 184 were primary cardiac tumors, including 170 cases of benign cardiac tumors. Among the benign tumors, myxomas were the most frequent ones (168 cases), followed by fibroma (2 cases). Primary malignancy was diagnosed in 14 cases, including undifferentiated sarcomas, primitive neuroectodermal tumor, rhabdomyosarcoma non-Hodgkin lymphoma, angiosarcoma, synovial sarcoma, and leiomyosarcoma. Metastatic (secondary) tumors were seen in four cases, including one each of adenocarcinoma, choriocarcinoma, renal cell carcinoma, and alveolar soft part sarcoma. Hence, out of the total of 188 cases, 20 were non-myxoma cardiac tumors (NMCTs), including 2 benign tumors, 14 malignant tumors, and 4 metastatic tumors. In our series, the majority of cardiac tumors were primary in nature. The malignant primary tumors outnumbered benign ones, excluding myxomas, and the most common malignant histology was undifferentiated sarcoma, as opposed to the literature.
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PMID:Spectrum of cardiac tumors excluding myxoma: Experience of a tertiary center with review of the literature. 2207 57

We present the case of a 16-year-old boy admitted with a large mediastinal mass. Both in the mediastinal tru-cut biopsy and the bone marrow biopsy, a malignant small round cell tumor was determined. The clinical diagnosis was leukemia or lymphoma. The cytoplasmic CD20 expression was shown on the tumor cells of the mediastinal tru-cut biopsy. Rhabdomyosarcoma was diagnosed by the flow cytometric analysis and immunohistochemical demonstration of muscle markers. Rhabdomyosarcomas are traditionally distinguished from lymphomas by their absence of lymphoid markers such as CD20. But in this case we have encountered CD20 expression in the tumor cells. This finding lead us to conclude that the presence of a lymphoid phenotype does not absolutely exclude the diagnosis of rhabdomyosarcoma.
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PMID:[A case of CD20 positive mediastinal rhabdomyosarcoma]. 2301 31

Spindle cell rhabdomyosarcoma is a rare subtype of rhabdomyosarcoma mainly seen in children. Occasional aberrant staining with a variety of immunohistochemical markers has been noted. The aberrantly expressed markers include alpha-smooth muscle actin, cytokeratin, S100, neurofilaments, CD20, immunoglobins, and CD117. We report herein two pediatric cases displaying strong CD34 positivity and one with additional focal CD117 positivity, causing considerable difficulty in distinction from solitary fibrous tumor and extra-gastrointestinal stromal tumor. To our knowledge, CD34 staining has been merely reported in rhabdomyosarcoma. Spindle cell rhabdomyosarcoma has to be considered in the differential diagnosis of childhood spindle cell tumors. Post-chemotherapy specimens should be evaluated in caution, since chemotherapy can cause considerable changes in tumor antigen expression. Since CD117 and CD34 are stem cell markers, their positivity in pediatric tumors should be interpreted with caution. Even if the morphology is not supportive, a wide immunohistochemical panel should be applied in childhood malignant solid tumors.
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PMID:Spindle cell rhabdomyosarcoma displaying CD34 positivity: a potential diagnostic pitfall; report of two pediatric cases. 2402 13

A 64-year-old male farmer presented with a rapidly progressive swelling of the left mandible since 6 months. The swelling was firm to hard, diffuse, nontender, obliterating the vestibule with paresthesia of lower lip. The cone beam computed tomography imaging revealed an ill-defined, moth-eaten radiolucency with destruction of the buccal and lingual cortical plates. The rapid growth and aggressive behavior of the lesion coupled with guidance from the patient's previous reports from the incisional biopsy and fine needle aspiration cytology warranted a mandibular resection. Microscopic examination showed an encapsulated lesion situated in the connective tissue containing a mixture of proliferating spindle-shaped cells arranged in fascicles and round cells infiltrating into the connective tissue stroma and bone. The neoplastic cells exhibited atypical features such as pleomorphism, hyperchromatism and increased mitotic figures with noncleaved nuclei. A working diagnosis of a spindle-cell sarcoma was arrived at with various differentials provided such as fibrosarcoma, rhabdomyosarcoma, leiomyosarcoma, malignant peripheral nerve sheath tumor, Langerhans cell histiocytosis and lymphoma and stating the need for immunohistochemistry to subtype the tumor. The neoplastic cells were negative for Van Gieson's stain and Masson's trichrome. Immunohistochemical analysis performed using desmin, smooth muscle actin, S-100 and CD1a in a bid to determine the phenotype of the tumor and rule out the previously stated differentials were all negative for the lesion. Lymphoid markers such as leukocyte common antigen and CD20 (cluster differentiation marker for B-cells) showed positivity in spindle-shaped cells as well as round cells indicating the tumor to be a lymphoproliferative lesion of B-cell type. A final diagnosis of "spindle-cell variant of non-Hodgkin's lymphoma" was rendered based on the immunohistochemical profile.
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PMID:A rare spindle-cell variant of non-Hodgkin's lymphoma of the mandible. 2719 75

Neurofibromatosis type 1 (NF1) is a common, cancer-predisposing disease caused by mutations in the NF1 tumor gene. Patients with NF1 have an increased risk for benign and malignant tumors of the nervous system (e.g., neurofibromas, malignant peripheral nerve sheath tumors, gliomas) and other tissues (e.g., leukemias, rhabdomyosarcoma, etc.) as well as increased susceptibility to learning disabilities, chronic pain/migraines, hypertension, pigmentary changes, and developmental lesions (e.g., tibial pseudoarthrosis). Pigs are an attractive and upcoming animal model for future NF1 studies, but a potential limitation to porcine model research has been the lack of validated reagents for direct translational study to humans. To address that issue, we used formalin-fixed tissues (human and pigs) to evaluate select immunohistochemical markers (activated caspase-3, allograft inflammatory factor-1, beta-tubulin III, calbindin D, CD13, CD20, desmin, epithelial membrane antigen, glial fibrillary acidic protein, glucose transporter-1, laminin, myelin basic protein, myoglobin, proliferating cell nuclear antigen, S100, vimentin, and von Willebrand factor). The markers were validated by comparing known expression and localization in human and pig tissues. Validation of these markers on fixed tissues will facilitate prospective immunohistochemical studies of NF1 pigs, as well as other pig models, in a more efficient, reproducible, and translationally relevant manner.
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PMID:Immunohistochemical Markers for Prospective Studies in Neurofibromatosis-1 Porcine Models. 2884 62