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)

The rhabdomyosarcoma of the spermatic cord is a rare tumour of the genital apparatus, of which 80 cases have been published in world literature. In the present paper, we publish a case of pleomorphic rhabdomyosarcoma of the spermatic cord, pointing out the highly malignant nature of this lesion (a 10% survival rate after 5 years) as well as the delay in its diagnosis on some occasions due to confusion with hydroceles, hernias and cord cysts. As far as the treatment is concerned, all the authors agree on the extensive exeresis of the tumour, with a need for a para-aortic lymphadenectomy when there are greater divergences. In spite of these tumours being only slightly radio-sensitive, its usefulness when used locally to prevent relapses has been proved. The experience with chemiotherapy is very limited.
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PMID:[Rhabdomyosarcoma of the spermatic cord: apropos of a case]. 47 81

This is a case of alveolar rhabdomyosarcoma with a rare clinical evolution. A first metastasis causes paraplegia; a second causes obstructive jaundice; a third subcutaneous metastasis is resected; the primitive tumor is discovered accidentally in the right calf, 8 weeks after the beginning of the disease. The literature is reviewed. Diagnosis of the alveolar rhabdomyosarcoma is often difficult because of confusion with a lymphoma, another type of sarcoma, a melanoma or even an epithelial tumor. The surgeon who removes a superficial node, obviously malignant, in a young subject, should think of this type of tumor. A multidisciplinary treatment associating radical resection, radio- and chemotherapy improves the very poor prognosis of this sarcoma.
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PMID:[A propos a case of alveolar rhabdomyosarcoma (author's transl)]. 98 37

Fifty-six renal neoplasms reviewed by the National Wilms' Tumor Study Pathology Center presented with histologic features that resulted in confusion with rhabdoid tumor of kidney, a usually lethal childhood renal tumor; all were eventually diagnosed as other entities. Conspicuous filamentous cytoplasmic inclusions or large nucleoli, typical findings in rhabdoid renal tumors, were the usual source of diagnostic difficulty. Most, but not all, tumors occurred in pediatric patients. Sixteen were examples of Favorable Histology Wilms' tumor, which invited confusion with rhabdoid tumors either on the basis of filamentous cytoplasmic inclusions (15 cases) or macronucleoli (one case). In most cases, foci of typical Wilms' tumor blastemal aggregation or evidence of definitive nephrogenic differentiation facilitated the correct diagnosis. All 10 patients for whom information about outcome was available were alive at last follow-up. The other 40 renal lesions mimicking rhabdoid tumor of kidney consisted of a clinically and histogenetically diverse group of neoplasms, including anaplastic Wilms' tumor, congenital mesoblastic nephroma, renal cell carcinoma, transitional cell carcinoma, collecting-duct carcinoma, oncocytoma, rhabdomyosarcoma, malignant neuroepithelial tumors, and lymphoma. Most of these lesions could be separated from renal rhabdoid tumors and correctly classified on the basis of careful attention to light microscopic details, but in several cases electron microscopy or immunocytochemical studies were helpful or essential.
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PMID:Renal neoplasms mimicking rhabdoid tumor of kidney. A report from the National Wilms' Tumor Study Pathology Center. 165 2

Sinonasal neoplasms and neoplasm-like proliferations composed of light microscopically poorly differentiated or undifferentiated, small- to medium-sized cells cause considerable diagnostic confusion. Lesions in this category include lymphoepithelioma (undifferentiated carcinoma), olfactory neuroblastoma, small-cell undifferentiated (oat cell) carcinoma, sinonasal undifferentiated carcinoma, malignant melanoma, pituitary adenoma, lymphoid hyperplasia, malignant lymphoma, plasmacytoma, lymphomatoid granulomatosis, rhabdomyosarcoma, mesenchymal chondrosarcoma, small cell osteosarcoma, Ewing's sarcoma, and synovial sarcoma. Many of these lesions can be definitively diagnosed based on light microscopic features alone, but, in some instances, additional techniques such as immunohistochemistry are of value. The authors review the pertinent clinicopathologic features of the above lesions, with emphasis on light microscopic, immunohistochemical, and ultrastructural features of particular utility in differential diagnosis.
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PMID:"Undifferentiated" neoplasms of the sinonasal region: differential diagnosis based on clinical, light microscopic, immunohistochemical, and ultrastructural features. 269 5

Ifosfamide was given to 61 patients with malignant solid tumors diagnosed before the age of 21 years. In this phase II study, all patients received 1.6 g/m2/day X 5 iv over 15 minutes followed by mesna at a dose of 400 mg/m2 iv at 15 minutes and 4 and 6 hours after ifosfamide. Responses were observed in five of 15 patients with osteosarcoma, two of ten with neuroblastoma, two of six with Wilms' tumor, two of five with rhabdomyosarcoma, four of eight with other soft tissue sarcomas, one of one with retinoblastoma, one of two with germ cell tumors, one of one with B-cell lymphoma, and one of one with a primitive neuroectodermal tumor. Fifty-nine of 61 patients had received prior alkylating agent therapy which included cyclophosphamide, cisplatin, mechlorethamine, melphalan, or dacarbazine. Fourteen of 19 responses developed in patients whose tumors were resistant to treatment with cyclophosphamide. A patient with malignant Schwannoma who had received no prior chemotherapy developed a complete response which lasted 12 months. A patient with brain metastases of osteosarcoma has had complete response for greater than 2 years. Complete response was also observed in a patient with B-cell lymphoma. Toxicity consisted of mild to moderate nausea and vomiting, transient reversible myelosuppression, occasional elevation of serum BUN or creatinine, and transient neurotoxicity characterized by somnolence, confusion, weakness, tremor, hallucinations, or seizures. We conclude that ifosfamide is an important alkylating agent without apparent complete cross-resistance with cyclophosphamide, and as such should be further investigated for determination of its activity in patients with pediatric neoplasms and considered for incorporation into phase II-III trials for certain tumors.
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PMID:Phase II trial of ifosfamide in children with malignant solid tumors. 310 34

Paratesticular rhabdomyosarcoma is a rare tumour of childhood and early adult life. We report a case in which the paratesticular location and colour Doppler appearances led to confusion with epididymitis.
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PMID:Case report: paratesticular rhabdomyosarcoma--colour Doppler appearances. 786 66

Sarcoma metastatic to the brain is uncommon and rarely occurs as the initial manifestation of tumor. Alveolar soft-part sarcoma, a rare but well studied subtype of a soft tissue sarcoma with a propensity for central nervous system invasion, presenting with brain metastases, has been reported only once previously. We report the case of a 28-year-old man who presented with partial seizures and who was found to have a homogeneously enhancing frontal lesion on a broad dural base disclosed by computed tomography. preoperatively, the lesion was thought to be a meningioma. The tumor was excised easily and had features typical of an alveolar soft-part sarcoma, which were revealed by light and electron microscopy as well as immunohistochemical analysis. Multiple lung nodules compatible with metastases were found on a chest film. Meningeal dissemination has been reported in a variety of sarcoma types, including rhabdomyosarcoma, fibrosarcoma, and leiomyosarcoma. We add alveolar soft-part sarcoma to this list and suggest that increased recognition of the propensity for these tumors to exhibit metastatic spread to the dura should eliminate diagnostic confusion and provide an earlier diagnosis of these rare lesions. The patterns of spread in metastatic sarcoma deserve further study.
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PMID:Metastatic alveolar soft part sarcoma presenting as a dural-based cerebral mass. 812 54

A lipid rich rhabdomyosarcoma of the paratesticular region was studied by light microscopy, histochemistry, immunohistochemistry and electron microscopy. The tumour was composed of primitive looking, vacuolated, and pleomorphic cells. Lipid was present in varying amounts in all cells but was especially abundant in the vacuolated and pleomorphic cells. Some cells showed eosinophilic fibrillary cytoplasm but cross-striations were not seen. Tumour cells were positive for desmin, muscle specific actin, and vimentin. A few cells were myoglobin positive. At electron microscopy, the presence of lipid was confirmed, while thick and thin filaments, Z disks, lamina and glycogen were observed, thereby confirming striated muscle differentiation. Although moderate amounts of lipid can be expected in almost any tumour, lipid rich rhabdomyosarcomas have received little attention. The present report provides a comprehensively examined case of such a tumour initially presenting diagnostic difficulty because of its possible confusion with liposarcoma.
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PMID:Lipid rich rhabdomyosarcoma. 816 5

Pleomorphic adenoma can show diverse lines of differentiation in the epithelial and myoepithelial elements, such as cartilage, bone, and fat. Myoid differentiation, however, has not been documented. We report an unusual case of nasal pleomorphic adenoma which shows focal skeletal muscle differentiation. The tumor was discovered only after successful radiation therapy for an undifferentiated carcinoma of the nasopharynx. Apart from being of morphological interest, the presence of cells with skeletal muscle differentiation also raises the practical issue of possible confusion with rhabdomyosarcoma in a biopsy. The distinguishing features are lack of cytological atypia and presence of a neoplastic glandular element.
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PMID:Nasal pleomorphic adenoma with skeletal muscle differentiation: potential misdiagnosis as rhabdomyosarcoma. 938 37

The diagnosis of rhabdomyosarcoma (RMS) is usually straight-forward when light microscopy and immunohistochemistry are used. However, tumors that exhibit a low degree of differentiation and small biopsies can lead to confusion. In such patients and for the detection of minimal (residual) disease, a polymerase chain reaction (PCR)-based approach would be a valuable diagnostic adjunct. This type of approach would be highly sensitive and should be free from the risk for contamination of the tumor sample with normal tissue. Because myogenin and the alpha and gamma subunit of the fetal type acetylcholine receptor (AChR) are specific immunohistochemical markers for RMS, their expression on the mRNA level in RMS, other childhood and adult tumors, and normal tissues was studied. Although the sensitivity of both approaches was 100% in embryonal and alveolar RMS, detection of myogenin mRNA was not specific for RMS but occurred in normal muscle and the majority of the other normal tissues and childhood tumors. Conversely, detection of fetal AChR mRNA as defined by an alpha/tau ratio of < 1 was encountered only in RMS and denervated muscle. The authors conclude that mRNA of the fetal type AChR but not myogenin is a highly specific and sensitive target for the PCR-based diagnosis of RMS.
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PMID:Polymerase chain reaction-based diagnosis of rhabdomyosarcomas: comparison of fetal type acetylcholine receptor subunits and myogenin. 983 66


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