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)

During the 5-year period between 1988 and 1989, five patients with primary sarcomas of the liver underwent surgery. Since the patients presented in an early stage of the tumor, all the sarcomas were resectable, in three cases with wide margins (R-0 resection). Five histological types were detected: malignant hemangiopericytoma, malignant fibrous histiocytoma, hemangiosarcoma, rhabdomyosarcoma, and embryonal sarcoma. Two patients with high-grade sarcomas received adjuvant chemotherapy. The follow-up was favorable in three patients with R-0 resections (two had adjuvant chemotherapy). They were still alive, with no evidence of disease 30, 46, and 63 months after the diagnosis. The two other patients had to be reoperated on for local recurrences. Both died of their tumor disease, 30 and 35 months after the initial diagnosis. Extensive chemotherapy in one of these cases failed to arrest tumor progression. Hence, liver resection with wide margins is a very important measure in such cases.
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PMID:Primary sarcoma of the liver in the adult. Report of five surgically treated patients. 142 75

The spectrum of non-meningothelial mesenchymal tumors that may arise within the central nervous system is presented, based on the current classification of soft tissue tumors. Among malignant types, hemangiopericytoma, rhabdomyosarcoma, mesenchymal chondrosarcoma, and malignant fibrous histiocytoma are the most frequent ones. Rare tumor entities are mentioned. As in soft tissue sarcomas, diagnosis is mainly based on light and electron microscopy, while immunohistochemistry can improve accuracy of diagnosis.
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PMID:Mesenchymal, non-meningothelial tumors of the central nervous system. 166 99

We reviewed patient records of 99 consecutive orbital exenterations performed between 1969 and 1988. Patients ranged in age from 2 to 86 years (mean, 55.9 years). Classification of cases on histopathologic criteria showed 32 exenterations were performed for squamous cell carcinoma originating in the paranasal sinus (13), skin (12), conjunctiva (six), and lacrimal sac (one). Orbital exenteration was performed for treatment of other epithelial malignancy in basal cell carcinoma (eight), sebaceous carcinoma (six), adenoid cystic carcinoma (five), undifferentiated carcinoma (four), adenocarcinoma (two), intraepithelial carcinoma of the conjunctiva (two), benign mixed tumor (one), and transitional cell carcinoma (one). Exenterations were performed for melanoma of the conjunctiva (ten), nasosinus (three), skin (two), orbit (two), and choroid (one). Exenterations were also performed as treatment for mucormycosis (five), meningioma (three), fibrosarcoma (two), rhabdomyosarcoma (two), hemangiopericytoma (two), orbital cellulitis (one), fibrous histiocytoma (one), schwannoma (one), lymphangioma (one), benign lymphoepithelial lesion (one), and undifferentiated malignancy (one).
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PMID:A 20-year series of orbital exenteration. 195 84

Increased MR signal intensity was observed on T2-weighted, STIR, and Gadolinium-DTPA-enhanced T1-weighted images of subcutaneous and muscular soft tissue in 9 of 10 children treated with combination chemotherapy and radiation therapy (RT) for malignancy in the pelvis or an extremity. Total radiation doses ranged from 59.5 to 65 Gy. Eight of the patients with these changes received hyperfractionated RT (seven for Ewing sarcoma and one for perineal rhabdomyosarcoma); one was treated for pelvic hemangiopericytoma with once-daily fractions. Evidence of soft tissue damage became apparent as early as the sixth week of RT and was seen for up to 69 wk post-RT. There was no clear MR evidence of RT-induced soft tissue damage in one patient, who underwent hyperfractionated RT for pelvic rhabdomyosarcoma. Other MR findings in this group included evidence of bladder wall thickening in three of the seven patients given pelvic RT and increased T1-weighted signal of irradiated marrow in nine patients. All patients had clinical evidence of skin, soft tissue, or epithelial radiation effects. Increased MR signal intensity secondary to RT-induced damage can be differentiated from widespread tumor by geometric borders that conform to the margins of the radiation field.
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PMID:Changes in MR signal intensity and contrast enhancement of therapeutically irradiated soft tissue. 226 4

Thirty-four cases of soft tissue tumors (STT) other than rhabdomyosarcoma in infants under 1 year of age were seen in our institution between 1955 and 1985. All were diagnosed initially as malignant tumors except for three cases of fibromatosis, and, thus, they received therapy appropriate at that time. During a recent pathologic review, four were seen to have had hemangioma, six hemangiopericytoma, one hamartoma, seven fibromatosis, eight fibrosarcoma, and eight unclassified sarcomas. Of these 34 cases, the initial histological diagnosis was confirmed in only 17. Sixteen patients were believed to have received inappropriately aggressive therapy; indeed, four of these cases diagnosed prior to 1975 had been benign. Major long-term side effects were seen in 7/16 treated patients (six radiotherapy, one surgery), who, in retrospect, should have received less aggressive treatment. The reasons for these initial errors in diagnosis included doubtful histologic features associated in some cases with alarming clinical presentation. We believe that difficulties still remain in distinguishing benign from malignant STT in this age group by histological examination. Therefore, we urge extreme caution in using aggressive therapy before the diagnosis is certain.
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PMID:Diagnostic and therapeutic problems of soft tissue tumors other than rhabdomyosarcoma in infants under 1 year of age: a clinicopathological study of 34 cases treated at the Institut Gustave-Roussy. 229 90

In view of the personal observation that malignant peripheral neuroectodermal tumours (MPNT) can present different histological growth patterns, 41 cases of MPNT were histologically and immunohistochemically studied. The median age of the 41 patients was 15 years (range: 9 months - 23 years). There were 27 males and 14 females. Most tumours (23/41) were located in the thoracopulmonary region. In 31/41 cases there was bone as well as soft tissue involvement. The following histopathological patterns were found: Ewing's sarcoma-like (n = 7), atypical Ewing's sarcoma-like (n = 4), neuroblastoma-like (n = 8), rhabdomyosarcoma-like (n = 8), and hemangiopericytoma-like (n = 1). In 2 cases combined patterns were noted, one tumour being characterized by neuroblastoma-like and Burkitt's lymphoma-like features. Most cases of MPNT differed from the cytological features of typical Ewing's sarcoma in that they contained hyperchromatic nuclei with distinct nucleoli. Some reticulin fibrils were found in between the cells of some cases. Immunohistochemically, 19/23 cases reacted positively to vimentin, 29/32 to neuron specific enolase (NSE), 16/28 to protein S-100, and 1/9 to glial fibrillary acidic protein. 12/24 cases reacted positively to NSE and protein S-100. Neurofilaments and desmin were not found in the formalin fixed material of the present study. The results show that most cases of MPNT can be distinguished from typical Ewing's sarcoma by cytological and histological findings. Differential diagnosis from atypical Ewing's sarcoma, neuroblastoma, and rhabdomyosarcoma is possible by immunohistochemistry.
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PMID:[Malignant peripheral neuroectodermal tumors. Histological and immunohistological conditions in 41 cases]. 267 76

Most of the retroperitoneal tumors (not originating from organs) are extremely rare (0.1% of all tumors) and are malignant. Clinical signs occur only late, are non-specific and are related to extreme tumor size. The most frequent tumors are malignant fibrous histiocytoma, liposarcoma, leiomyosarcoma, rhabdomyosarcoma, hemangiopericytoma and neurogenic tumors. The relevant clinical and morphological findings of these tumors as well as prognostic features are discussed.
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PMID:[Tumors of the retroperitoneum]. 268 Oct 85

A total of 488 tumors entered in the Eastern Cooperative Oncology Group (ECOG) Study EST 3377 were evaluated histologically by a panel of pathologists from member institutions for quality control purposes. The overall agreement rate between the eligible submitting diagnosis and the pathology review panel's diagnosis was 74% (312/424). In 10% (44/424), the case was excluded because it was deemed to be nonsarcoma. In the other 16%, the disagreement concerned the type of sarcoma. The histologic type with the lowest agreement rate was rhabdomyosarcoma (17%), followed by sarcoma not otherwise specified (NOS) (27%), angiosarcoma (33%), and fibrosarcoma (48%). These figures reflect the significant degree of difficulty in the diagnosis of these tumor types. The treatment response rate of soft tissue sarcomas in the randomized study of Adriamycin (Adria Laboratories, Columbus, OH) regimens was slightly higher for those with lower grade sarcomas, i.e., 25% (four of 16) response rate for Grade 1 lesions; 22% (17/77) for Grade 2, and 21% (35/170) for Grade 3. When adjusted for type of sarcoma, there was no noticeable difference between Grade 1-2 versus 3 in response rate. A statistically significant difference in the percentage of complete responders was noted between Group A tumors (synovial sarcoma, hemangiopericytoma, sarcoma NOS, and Ewing's; 12.2%) versus Group B tumors (all other types--mostly spindle cell sarcomas; 3.5%) (P = 0.02).
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PMID:Pathologic analysis of advanced adult soft tissue sarcomas, bone sarcomas, and mesotheliomas. The Eastern Cooperative Oncology Group (ECOG) experience. 273 94

Small, round, blue-cell tumors (SRCT), including rhabdomyosarcoma, Ewing's sarcoma of bone and soft tissue, mesenchymal chondrosarcoma, small cell osteosarcoma, hemangiopericytoma, neuroblastoma, peripheral neurectodermal tumor (peripheral neuroepithelioma of bone and soft tissue), and the malignant small cell tumor of the thoracopulmonary region described by Askin (Askin's tumor), are often difficult to distinguish by light microscopy. We have evaluated the cytogenetics of these tumors by studying 24 tumor explants in short-term culture and 22 tumor cell lines. In Ewing's sarcoma (a tumor of unknown histogenesis), and in peripheral neuroepithelioma and Askin's tumor (tumors with evidence of neural origin), we have observed an indistinguishable t(11;22) translocation.
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PMID:Cytogenetic characterization of selected small round cell tumors of childhood. 300 99

A consecutive series of 100 patients operated on for lesions that were assumed to be soft tissue tumors, all of whom had been the subject of fine-needle aspiration in the preoperative investigation, is described. A correlative study of smears and the light- and electron-microscopic findings of embedded fine-needle aspirates and the histopathology of the surgical specimens was performed. Eighty of the lesions were found to be genuine soft tissue tumors, of which 51 were sarcomas. The other 20 cases were either metastatic carcinoma, malignant melanoma, or malignant lymphoma. The embedding technique produced additional light-microscopic information about tissue structure and growth pattern, and electron-microscopic information about tissue and cell differentiation of importance to the diagnosis. In the case of certain types of soft tissue tumor, such as lipoma, neurilemmoma, liposarcoma, and malignant fibrous histiocytoma, and for well-differentiated metastatic carcinoma and pigmented malignant melanoma, the diagnosis may be strongly suggested by the appearance of the smears; the embedding technique serves to further support the diagnosis. In the case of small round-cell malignancies, the ultrastructural examination proved to be of special value, ie, in the distinction of rhabdomyosarcoma, poorly differentiated metastatic small cell carcinoma and malignant melanoma, and occasional cases of malignant lymphoma. Spindle cell sarcomas, such as leiomyosarcoma when well differentiated, biphasic synovial sarcoma when it includes glandular structures, and malignant hemangiopericytoma, could be recognized ultrastructurally, although electron-microscopy generally failed to reach a definite diagnosis as to the subtype in most cases of poorly differentiated spindle-cell sarcoma.
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PMID:Ultrastructural studies in the preoperative cytologic diagnosis of soft tissue tumors. 330 37


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