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)

We recently identified three cases of primitive rhabdomyosarcoma (PRMS) presenting with diffuse bone marrow infiltration but inconspicuous soft tissue primaries, referred to The Johns Hopkins Hospital (JHH) as acute leukemia. In each case, the diagnosis of rhabdomyosarcoma was established using immunohistochemical staining and electron microscopy. Ultrastructural examination of tumor cells showed a feltwork of thin filaments, discontinuous basal lamina, glycogen, and primitive cell junctions without cell processes or neurosecretory granules. This presentation of PRMS may be more common than recognized, since it can be readily misdiagnosed as a hematopoietic tumor. While positive staining for muscle-specific actin, desmin, myoglobin, or other markers of skeletal muscle differentiation may be diagnostic, negative staining is inconclusive, requiring recognition of the "minimal" ultrastructural findings of primitive rhabdomyosarcomas.
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PMID:Primitive rhabdomyosarcoma presenting with diffuse bone marrow involvement: an immunohistochemical and ultrastructural study. 297 40

Histological localization of myosins of the smooth and skeletal muscles was investigated in comparison with that of myoglobin by immunoperoxidase technique using the antibody against each of them in surgical specimens from spindle cell tumors and tumor-like lesions of the soft tissue. Skeletal muscle myosin was demonstrated in all of the cases of rhabdomyosarcoma, whereas myoglobin was found in 75% of the examined cases. Smooth muscle myosin was widely distributed not only in the tumor cells of smooth muscle origin such as leiomyosarcoma and angioleiomyoma, but also in the tumor cells showing myofibroblastic differentiation such as malignant fibrous histiocytoma and in the epithelial components of synovial sarcoma. The results showed that skeletal muscle myosin can be regarded as an excellent marker in the diagnosis of rhabdomyosarcoma and that smooth muscle myosin is a useful marker of leiomyosarcoma and leiomyomas, and of tumors with myofibroblastic differentiation.
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PMID:Smooth and skeletal muscle myosins in spindle cell tumors of soft tissue. An immunohistochemical study. 298 65

The clonal cell lines designated as HIRS-BMS and HIRS-BMA were established from HIRS-BM (multipotent primitive cells which differentiated into rhabdomyosarcoma and adenocarcinoma cells) by a single cell plating method. The HIRS-BMS was a rhabdomyosarcoma cell line composed of elongated fibrous cells which contained myoglobin. The HIRS-BMA was an adenocarcinoma cell line composed of round cells. The tissue reconstruction of these cell lines was studied in vitro (rotation culture system) and in vivo (transplantation into hamster cheek pouch). The HIRS-BMS cells produced rhabdomyosarcoma, both in vitro and in vivo, and the HIRS-BMA produced poorly differentiated adenocarcinoma, in vitro and in vivo. The mixture of HIRS-BMS and HIRS-BMA produced a mixed mesodermal tumor resembling the original tumor. These results support the theory of a combined tumor as a cause of mixed mesodermal tumor.
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PMID:Histogenesis and tissue reconstruction of mixed mesodermal tumor. 301 54

Pleomorphic rhabdomyosarcoma in adults over 30 years of age was a diagnosis frequently made in the 1960s and 1970s. Since the general acceptance of malignant fibrous histiocytoma (MFH) as a tumor entity at the end of the 1970s, however, it has become a very rare tumor in adults. Therefore, 21 cases originally diagnosed on the basis of histology and clinical data as pleomorphic rhabdomyosarcoma in the 1960s and 1970s were reexamined immunohistochemically. Other types of pleomorphic sarcomas involved in the differential diagnosis were also studied. Specific antibodies against vimentin, desmin, creatine kinase subunit M, skeletal muscle actin and myosin, and myoglobin, and the avidin-biotin-peroxidase complex technique were used. The immunohistochemical findings indicate that rhabdomyosarcoma occurs only rarely in adults over 30 years of age and that the majority of the tumors have to be reclassified as MFH or leiomyosarcoma. On the other hand, several pleomorphic sarcomas were found to be diagnosed incorrectly as MFH or liposarcoma by routine histologic stains and electron microscopy. The revised diagnosis was pleomorphic rhabdomyosarcoma for one case and pleomorphic leiomyosarcoma for the other cases. Thus, this study clearly shows the usefulness of immunohistochemistry as a technique in the diagnosis of pleomorphic sarcomas in adults.
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PMID:Pleomorphic rhabdomyosarcoma in adults: immunohistochemistry as a tool for its diagnosis. 302 31

Esthesioneuroblastoma (EN), a malignant neuroblastic tumor arising in the superior portion of the nasal cavity, shares histologic similarities with a number of primary malignant tumors that arise in this region, including rhabdomyosarcoma, lymphoepithelioma, and lymphoma. To establish an antigenic profile of EN as an aid in the differential diagnosis of these histologically similar nasal tumors, immunostaining was performed for the following intermediate filaments: keratin, neurofilament, glial fibrillary acidic protein, and desmin; neuron-specific enolase (NSE), S-100 protein, chromogranin, human common leukocyte antigen (HLE), epithelial membrane antigen (EMA), myoglobin, and carcinoembryonic antigen (CEA) on 21 primary nasal tumors: eight EN, five lymphoepitheliomas, two small cell carcinomas, three lymphomas, and three rhabdomyosarcomas. Keratin and CEA stained only the carcinomas (6/7+, 4/7+), respectively; desmin and myoglobin only rhabdomyosarcoma (3/3+, 1/3+); and HLE only lymphomas (3/3+). Chromogranin and neurofilament staining occurred exclusively in one case each of EN. S-100 and NSE commonly stained EN (5/8+, 6/8+), but carcinomas (1/7+, 2/7+) and rhabdomyosarcomas (1/3+, 3/3+) were also positive. Despite the apparent nonspecificity of NSE and S-100, an antigenic profile of positive NSE of S-100 staining with negative epithelial, muscle, and lymphoid antigens uniquely identified six of eight EN. Chromogranin and neurofilament positivity was further evidence for EN in two cases. This antigenic profile is a helpful adjunct in the diagnosis of EN and other primary malignant nasal tumors.
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PMID:Esthesioneuroblastoma. Intermediate filaments, neuroendocrine, and tissue-specific antigens. 303 34

The type, differentiation and histogenesis of the tumor cells of alveolar soft part sarcoma (ASPS) have been analyzed in a series of ten cases by a light-microscopic, ultrastructural, immunohistochemical and cytologic investigation and quantitative DNA analysis. Four tumors deviated from ordinary ASPS: three were wholly or partly of the so-called pleomorphic variant of ASPS and a fourth tumor showed calcifications of the psammoma body type. The ultrastructural findings and immunohistochemical demonstration of desmin supported the hypothesis of a rhabdomyomatous differentiation and gave no support to epithelial (negative immunoreactions for cytokeratins, epithelial membrane antigen, HMFG-1 and -2, tissue polypeptide antigen (TPA] or neuroectodermal (negative for S-100 protein, glial fibrillary acidic protein, neurofilaments) differentiation. The negative immunoreactions for vimentin and myoglobin and the positive reaction for neuron specific enolase (NSE) do not exclude a rhabdomyomatous differentiation since in rhabdomyosarcomas the undifferentiated rhabdomyoblasts generally contain vimentin and the differentiated tumor cells contain myoglobin and rhabdomyosarcoma has previously been reported as being positive for NSE. The production of external lamina material peripherally in the tumor cell nests and around vessels in the vascular septa was demonstrated both ultrastructurally and by immunohistochemistry using antibodies against collagen IV and laminin. The cytologic appearance in smears obtained by fine-needle aspiration from a case of the pleomorphic variant showed some resemblance to that of a carcinoma. The seven tumors with an ordinary cell appearance were found to show a diploid DNA-distribution at a quantitative analysis performed on paraffin sections, while the three tumors wholly or partly of the pleomorphic type showed an additional tetraploid peak.
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PMID:Alveolar soft part sarcoma. An immunohistochemical, cytologic and electron-microscopic study and a quantitative DNA analysis. 312 65

Eighteen poorly differentiated, small and dark cell malignancies afflicting young individuals without light-microscopic evidence of a rhabdomyoblastic differentiation or a growth pattern characteristic of rhabdomyosarcoma were analyzed and compared with a series of 30 alveolar rhabdomyosarcomas of varying differentiation, where the diagnosis could be established light-microscopically. The study comprised clinical data, light and electron microscopy and immunohistochemistry, using a battery of mono- and polyclonal antibodies against intermediate filaments, myoglobin, epithelial membrane antigen, neuron-specific enolase, S-100 and leucocyte common antigen. All 30 alveolar rhabdomyosarcomas were positive for desmin, while a minority were positive for myoglobin, using monoclonal antibodies. In 8 of the 18 small and dark cell malignancies, support for a rhabdomyoblastic differentiation was obtained by a positive staining for desmin. In only 3 of these 8 cases was there ultrastructural evidence of rhabdomyosarcoma. The results of the investigation indicate that immunohistochemistry is a more useful tool than electron microscopy in the diagnosis of poorly differentiated rhabdomyosarcoma and that the criteria for the diagnosis of poorly differentiated rhabdomyosarcoma may need to be reformulated.
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PMID:Alveolar and poorly differentiated rhabdomyosarcoma. A clinicopathologic, light-microscopic, ultrastructural and immunohistochemical analysis. 316 9

Primary cardiac rhabdomyosarcoma (PCR) is a highly malignant tumor that is rarely recognized prior to surgery or necropsy. We present the pathologic findings of a primary atrial rhabdomyosarcoma in an 82-year-old woman and an update on PCR as a clinicopathologic entity. In addition to pleomorphic tumor cells with cross-striations and a positive immunoperoxidase reaction for myoglobin, some areas of our patient's tumor had features of sarcoma botryoides. Electron optic studies showed primitive cells with fibroblastic and/or myoblastic differentiation. A comprehensive study of 77 cases in the international literature yielded new information and perspectives on PCR. The incidence of PCR is bimodal among males, being highest in infancy and early childhood with a secondary peak in the sixth and seventh decades. Females, however, have a single peak incidence in the fifth decade. Septal origin is 35.2% among infants and children but only 5.2% among adults. The predilection for the pediatric age group in conjunction with common septal origin among these patients suggests a congenital form of PCR that may arise from embryonic cell tests.
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PMID:Primary cardiac rhabdomyosarcoma: definition of a rare entity. 320 86

Embryonal rhabdomyosarcoma is the most frequent of tissue sarcomas in children. Its location in the subcutaneous tissue makes it a dermatological diagnosis. In childhood, the exact histological type of the tumour, sometimes difficult to determine, is absolutely necessary since prognosis and treatment differ according to the histogenetic form. Rhabdomyosarcoma is rare in adults. One must rule out malignant pleomorphic histiocytoma which has a more favourable prognosis. Among the antisera recently made available, those directed against desmin, foetal skeletal myosin and/or specific skeletal muscle myofilament seem to be most useful when associated with the anti-myoglobin antibody.
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PMID:[Subcutaneous rhabdomyosarcoma in children. Clinical, immunologic and ultrastructural aspects]. 322 87

A 3-year-old girl had a large exophytic mass protruding from the nares, of seven months' duration. Routine microscopic studies demonstrated a tumor composed of solid sheets of small cells, with scattered foci lining alveola-like spaces. Immunohistochemical studies demonstrated the presence of desmin and myoglobin, and the absence of prekeratin, neuron-specific enolase, and leukocyte common antigen. These observations are consistent with the diagnosis of alveolar rhabdomyosarcoma.
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PMID:Alveolar rhabdomyosarcoma arising in the nasal cavity of a 3-year-old child. 323 83


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