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)

Embryonal rhabdomyosarcoma is a common tumor of children, rarely appearing in the scrotum from tunica vaginalis or paratesticular tissues. In adults it is rare and carries a particularly bad prognosis. A case of paratesticular rhabdomyosarcoma occurring in a seventy-three-year-old man who survived a total of five and one-half years from the time of his original operation, is presented.
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PMID:Paratesticular rhabdomyosarcoma in an adult. 744 13

Pseudosarcomatous myofibroblastic tumor (PMT) is the result of reactive proliferation of myofibroblasts. In children, PMT of the urinary bladder can be mistaken for embryonal rhabdomyosarcoma clinically, radiologically, and by light microscopy. We are reporting the clinical, histological, and immunohistological features of 11 patients with childhood PMT of urinary bladder that were diagnosed initially as a sarcoma, usually rhabdomyosarcoma. The morphologic spectrum of PMT is broad, with mixtures of myxoid, leiomyomatous, and sclerosing matrix patterns, the myxoid type being the most common. The proliferating cells consist of three forms of myofibroblastic cells: long spindle cells (type I), intermediate spindle cells (type II), and ganglion-like cells (type III), together with various types of inflammatory cells. The immunohistologic profile of the proliferating cells was characterized by positive reactions to vimentin, muscle-specific actin, alpha-smooth-muscle actin, polyclonal desmin, and keratin. Ultrastructural studies showed myofibroblastic differentiation of the tumor cells. No patients have had metastases or local recurrence. Histologic, immunohistochemical, and clinical data from 71 cases of PMT, including the 11 cases in this report, confirm the benign behavior of these lesions. The etiology of these lesions is unclear, including the absence of surgical or other trauma in all of the children.
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PMID:Pseudosarcomatous myofibroblastic tumor of the urinary bladder in children: a study of 11 cases with review of the literature. An Intergroup Rhabdomyosarcoma Study. 757 84

Unilateral proptosis, dislocation of the globe and impairment of motility in childhood are known to be typical presenting signs of rhabdomyosarcoma of the orbit. In a retrospective analysis of 17 patients with rhabdomyosarcoma (1974-1993) only 8 patients presented with typical signs. 9 patients did never show the typical signs which initially lead to a wrong diagnosis in many cases. A 5-year-old boy complained of a conjunctival cyst in the lower fornix of the left eye. Two weeks later the cyst was replaced by a solid tumor. The tumor was not completely resectable, histology revealed an embryonal rhabdomyosarcoma. Presenting signs and symptoms of orbital rhabdomyosarcoma show a sometimes misleading variety including conjunctival cyst formation. In any recent onset of unilateral rapidly progressing alterations of the lid, the conjunctiva or the caruncle in childhood the physician should consider a rhabsomyosarcoma of the orbit.
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PMID:[The initial clinical manifestations of rhabdomyosarcoma]. 765 15

Rhabdomyosarcoma is a rare soft tissue neoplasm most commonly encountered in childhood and adolescence which has a predilection for the head and neck area, the genito-urinary tract and the extremities. Primary cutaneous presentation is extremely unusual and has been rarely reported in the literature. Herein, we describe two cases of rhabdomyosarcoma arising in the dermis of a 9-year-old girl and an 86-year-old man. Clinically, the tumours presented as solitary plaque-like or nodular lesions confined to the skin of the nose and chest wall, respectively. Histologically, the tumour in the first patient corresponded to an embryonal rhabdomyosarcoma. The tumour recurred locally four times, and in the last recurrence, showed features resembling those of malignant 'triton' tumour with fascicles of S-100 protein-positive spindle cells admixed with the rhabdomyoblastic components. The tumour in the second patient corresponded to the solid variant of alveolar rhabdomyosarcoma. Immunohistochemical studies in both tumours showed positive labelling for muscle-specific actin, desmin and vimentin. Ultrastructural examination in one case showed clusters of intermediate filaments in the cytoplasm recapitulating abortive sarcomeric structures consistent with rhabdomyoblastic differentiation. Both patients developed repeated recurrences over a period of 2-4 years despite adequate surgical excision, and the second patient had an axillary lymph node metastasis. Primary cutaneous rhabdomyosarcoma should be considered in the evaluation of small 'blue cell' tumours or undifferentiated malignant neoplasms of the skin, and appropriate immunohistochemical studies in conjunction with electron microscopy should be employed for proper evaluation of such lesions.
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PMID:Primary cutaneous sarcomas showing rhabdomyoblastic differentiation. 771 81

Primary rhabdomyosarcoma of the meninges, a very rare brain tumor, is reported. Cytologic findings by squash preparation were useful as an adjunct to frozen section diagnosis during surgery. The cytologic features of rhabdomyosarcoma without cross-striation have some similarities to those of gemistocytic astrocytomas and anaplastic meningiomas, but the cytoplasmic filaments of rhabdomyosarcoma are different from those of gemistocytic astrocytoma and anaplastic meningioma. Histologically the tumor was embryonal rhabdomyosarcoma with a partially botryoid pattern. The tumor cells have no cross-striations but react positively to antimyoglobin serum on immunoperoxidase staining. In this case, intracytoplasmic filaments resembling poorly formed myofibrils were found on electron microscopic study. The histologic and immunohistologic findings suggest that this rare mesenchymal malignancy might arise from primitive totipotential cells.
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PMID:Meningeal rhabdomyosarcoma. Report of a case with cytologic, immunohistologic and ultrastructural studies. 776 28

Rhabdomyosarcoma is an uncommon tumour that may present at a wide variety of different sites. We report a 4-year-old girl who developed an embryonal rhabdomyosarcoma arising in the left pinna which was clinically indistinguishable from a lymphangioma. The case illustrates that this neoplasm can be easily misdiagnosed because of its variable morphology. Early recognition is important as successful treatment is now possible with a combination of chemotherapy, surgery and/or radiotherapy.
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PMID:Auricular embryonal rhabdomyosarcoma. 794 90

Insulin-like growth factor II (IGF-II) acts as autocrine growth and motility factor in human rhabdomyosarcoma cell lines, and Northern blot analysis of tumor biopsy specimens from both alveolar and embryonal rhabdomyosarcoma demonstrates high levels of IGF-II mRNA expression. To determine the frequency and site of expression of IGF-II in these tumors, the authors performed in situ hybridization. All tumor specimens examined expressed the gene for IGF-II, and this expression was localized to the tumor cells themselves and not to the surrounding stroma. These data suggest that the IGF-II autocrine loop may be operating not only in vitro but also in vivo.
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PMID:Specific expression of insulin-like growth factor-II in rhabdomyosarcoma tumor cells. 811 75

Rhabdomyosarcoma is one of the most common soft-tissue neoplasms in children. We report a case of a 9-year-old female with embryonal rhabdomyosarcoma arising in the buccal region with immunohistochemical and electronmicroscopic findings. Under a light microscope, it was observed that the tumor was composed of small round or spindle-shaped cells with pleomorphic and hyperchromatic nuclei. They were immunoreactive for actin, myoglobin and desmin. With an electron microscope, it was found that most tumor cells contained filamentous structures and free ribosomes. Some of them showed typical myofilaments, M-bands and Z-lines. These findings were specific evidence of rhabdomyosarcoma. Twenty courses of VAC (vincristine, D-actinomycin and cyclophosphamide) chemotherapy were administered, followed by surgical resection of the tumor.
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PMID:Embryonal rhabdomyosarcoma arising in the buccal mucosa: a case report with immunohistochemical and electronmicroscopic findings. 815 26

Sixty-seven childhood tumors were studied immunohistochemically for the extracellular matrix element type IV collagen, laminin, and fibronectin. Tumors included Ewing's sarcoma, primitive neuroectodermal tumor, small cell osteosarcoma, neuroblastoma or ganglioneuroblastoma, rhabdomyosarcoma, and lymphoma. It was found that small cell osteosarcoma was often positive for fibronectin but not type IV collagen or laminin, a new observation. In the lymphomas, matrix proteins were rarely found. Ewing's sarcoma was variably positive for type IV collagen and laminin, but fibronectin was absent. Extracellular laminin and fibronectin were found in one of two cases of primitive neuroectodermal tumor. In neuroblastoma and ganglioneuroblastoma, the matrix components were rarely found. These results, discrepant with findings in cultured cells, may reflect the altered capacity of tumors to produce these proteins in vitro, which suggests that caution should be exercised in drawing conclusions regarding the nature or histogenesis of tumors from data obtained with cultured tumor cells. Embryonal rhabdomyosarcoma frequently contained all matrix elements in the extracellular space and in a dotlike pattern in the cytoplasm; alveolar rhabdomyosarcoma rarely contained these proteins and never exhibited the dotlike pattern. The frequent finding of matrix proteins in embryonal rhabdomyosarcoma but only rarely in alveolar rhabdomyosarcoma and the unique immunostaining pattern in embryonal rhabdomyosarcoma may prove to be a useful adjunct in the diagnosis of childhood tumors.
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PMID:Extracellular matrix of small round cell tumors of childhood: an immunohistochemical study of 67 cases. 815 9

Four cases are presented of primary anterior mediastinal tumors in young adults that were characterized by solid, infiltrative lesions showing histologic and immunohistochemical features of rhabdomyoblastic differentiation. The patients were three men and one woman between 19 and 27 years of age (mean age, 23 years). All patients presented with symptoms referable to their tumors, including cough, chest pain, dyspnea, and left-sided pleural effusion. Grossly and radiographically, the lesions were characterized by their solid, infiltrative appearance. Histologically, two cases corresponded to the solid variant of alveolar rhabdomyosarcoma, one case was an embryonal rhabdomyosarcoma with a predominant spindle cell component, and the remaining case showed the features of a pleomorphic rhabdomyosarcoma. No glandular, epithelial, or other component could be identified in any of the tumors on extensive sampling. Immunohistochemical studies showed positive staining of the tumor cells with actin, desmin, and vimentin antibodies, with focal positivity for myoglobin in three cases and focal positive staining with S-100 protein in one case. Stains for low and high molecular weight keratin, carcinoembryonic antigen, alpha-fetoprotein, human chorionic gonadotropin, placental alkaline phosphatase, leukocyte-common antigen, and neuron-specific enolase were negative. All patients experienced rapid recurrence and metastases within the first 6 months after diagnosis. Three patients died within this period due to their tumors; the fourth patient has been lost to follow-up. Pure primary rhabdomyosarcomas of the anterior mediastinum are highly aggressive neoplasms that should be distinguished from germ cell, teratomatous, or carcinosarcomatous tumors with a focal rhabdomyoblastic component.
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PMID:Rhabdomyosarcomas of the anterior mediastinum: report of four cases unassociated with germ cell, teratomatous, or thymic carcinomatous components. 816 67


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